Manual of Vascular Medicine [1st ed.] 9783030447144, 9783030447151

Citation preview

Manual of Vascular Medicine Marie Gerhard-Herman Aaron Aday

123

Manual of Vascular Medicine

Marie Gerhard-Herman Aaron Aday

Manual of Vascular Medicine

Marie Gerhard-Herman Brigham and Women’s Hospital Harvard University Boston MA USA

Aaron Aday Vanderbilt University Medical Center Nashville TN USA

ISBN 978-3-030-44714-4    ISBN 978-3-030-44715-1 (eBook) https://doi.org/10.1007/978-3-030-44715-1 © Springer Nature Switzerland AG 2020 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

Vascular Medicine for All: Introduction Vascular medicine is an approach in medicine that addresses concerns about blood vessels anywhere in the body. Manifestations of compromised blood flow are seen in all vascular beds. Abnormalities are seen in vessels of all sizes and locations including not only the extremities but also the lungs, viscera, kidneys, and nervous system. The vascular system consists of a vast number of tubes that leave the heart (pump) and carry fluid that includes blood cells, hormones, and nutrients via the arteries and then capillaries to the tissues. Venules and then veins (85%) and lymphatics (15%) collect and return the fluid to the heart. Vessels differ by region and function with pressure and flow for example directing the differentiation of arterial and venous endothelial cells. Embryologic origin may differ in different regions of a single vessel such as the aorta. Vascular smooth muscle cells populate the media and regulate vascular tone. Signals to the media come from both the endothelial and the adventitial layer. A unifying approach in the arteries is to recognize that arterial blood flow is decreased for a finite number of reasons. It is decreased in the setting of fixed changes like discrete blockage or tubular narrowing as well as episodically decreased with changes like spasm. Clinical manifestations can range from discomfort to life-threatening emergencies. Why change in flow has happened involves many possible etiologies such as atherosclerosis, thrombosis, inflammation, drugs, or toxins. Venous and lymphatic flow are similarly impacted by a variety of pathologic states. Given the wide range of vascular beds and etiologies involved, the care of v

vi

Vascular Medicine for All: Introduction

patients with vascular disease is fragmented and shared by many different disciplines. As a result, vascular cases often present enormous challenges to physicians of all types. The discipline of vascular medicine tries to address the full breadth of these diseases and stresses a multidisciplinary approach to patient care. The majority of vascular medicine specialists are found in the cardiovascular division of medicine. However, training in the discipline of vascular medicine has been often cobbled together from many areas without a clear description of the fundamentals. This has been addressed by the Core Cardiovascular Training Statement of the American College of Cardiology, which provides a clear description of the competencies needed to be able to successfully diagnose and treat vascular disease. Vascular medicine has subsequently been incorporated into board examinations, and vascular topics are now an integral component of the cardiovascular examination of the American Board of Internal Medicine. The aim of this text is to present essential vascular medicine knowledge that addresses these topics in a straightforward manner for the practitioner using clinical scenarios, complete and concise information, and questions allowing for self-assessment. By providing these key fundamentals in an approachable format, we hope to improve clinicians’ confidence in diagnosing and treating the full spectrum of vascular diseases and, ultimately, create a tool to be shared by providers across all specialties.

Contents

1 Noninvasive Vascular Testing���������������������������������������   1 References  13 2 Evaluation of Leg Pain �������������������������������������������������  15 References  19 3 Peripheral Artery Disease���������������������������������������������  21 References  28 4 Aortic Disease�����������������������������������������������������������������  31 References  40 5 Renal and Mesenteric Disease�������������������������������������  43 References  49 6 Vasospastic Disease�������������������������������������������������������  51 References  60 7 Cerebrovascular Disease�����������������������������������������������  63 Clinical Presentation  63 References  70 8 Evaluation of Limb Swelling�����������������������������������������  73 References  77 9 Venous Disease���������������������������������������������������������������  79 References  89 10 Lymphedema�������������������������������������������������������������������  91 References  94

vii

viii

Contents

11 Vascular Compressive Syndromes�������������������������������  97 References 102 12 Special Populations ������������������������������������������������������� 105 References 111 Index����������������������������������������������������������������������������������������� 113

Chapter 1 Noninvasive Vascular Testing

Objective  Understand what type of testing is most likely to answer the vascular question. Vignette  A 50 year old female yoga instructor with history of polymyalgia rheumatica presents with bilateral thigh and calf ache with exertion that goes away with rest. Physiologic Testing Techniques of acquisition in physiological testing include segmental pressure measurements, pulse volume recordings (PVRs), continuous wave (CW) Doppler, plethysmography, exercise testing, transcutaneous oximetry, laser Doppler and skin perfusion pressure. One of the most common tests is the ankle-brachial index (ABI), which is used to detect peripheral artery disease (PAD). This test uses sphygmomanometric cuffs, Doppler instruments, and plethysmographic recording devices [1]. After the patient rests for 10 minutes in the supine position, the systolic blood pressure is first measured using a continuous wave Doppler probe and pneumatic cuff in both brachial arteries at rest. The ankle pressure is then measured in both dorsalis pedis and posterior tibial arteries above the medial malleo-

© Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_1

1

2

Chapter 1.  Noninvasive Vascular Testing

lusafterinflating the cuff to 30 mmHg above the brachial pressure or until the pulse is no longer detectable by Doppler. The ABIs for each extremity are calculated by dividing each of the ankle pressures by the higher of the brachial artery pressures [2]. A normal ABI is between 1.0 and 1.4, whereas an ABI > 0.9 to 1.0 is borderline abnormal. An ABI > 0.90 is considered diagnostic of PAD. An ABI ≥ 1.4 suggests vessels could not be reliably compressed which is often due to vascular calcification artifact and makes interpretation of the pressure measurement unreliable. The shape of the arterial waveform is evaluated the same way throughout the arterial system (Fig. 1.1). Waveforms are obtained using plethysmography with the cuff inflated to venous occlusive pressure, typically no more than 65 mm Hg. The change in volume in the limb segment throughout the cardiac cycle causes a corresponding change in pressure in the cuff. Pulse waveforms can also be obtained using photoplethysmography, which records Normal

Mildly abnormal

Moderately abnormal

Sharp upstroke Flat interval between peaks Possible dicrotic notch

Sharp upstroke Broadened with no flat interval between peaks

Upstroke

Dicrotic notch

Wide and broad peak

Equal upslope and downslope time Flattened peak Same angle up and down slope

Severely abnormal

Very low amplitude Equal upslope and downslope time Flattened peak

Flat, low amplitude

Figure 1.1  Criteria for normal to abnormal waveform shape

Chapter 1.  Noninvasive Vascular Testing

3

reflected infrared light. Waveforms are unaffected by incompressible arteries, and this technique can be useful in individuals with advanced calcific lower extremity arterial disease. Transcutaneous oximetry uses the variations in color absorbance of oxygenated and deoxygenated hemoglobin to determine the blood oxygenation [3]. Normal limb oximetry should be the same in the limb and in the chest. Laser Doppler is another option depends on the light that is scattered from moving blood cells for single point perfusion monitoring. Because the ABI uses pressure measurements at the ankle, it integrates flow disturbances in all segments of the limb arterial bed. However, in many cases it is also useful to detect the anatomic level at which these disturbances occur, particularly in individuals with a history of lower extremity arterial intervention. This can be achieved in a non-invasive manner with segmental Doppler pressure (SDP) measurements, which use the same principles of the ABI to compare limb pressures at the proximal thigh, distal thigh, calf, and ankle levels to the higher of the two brachial artery pressures (Fig. 1.2). Pressure decrements between levels indicate disease between cuff levels. Exercise testing can be used to clarify whether leg symptoms are related to PAD [4]. Ankle pressures are taken before and after exercise. The ankle pressures are obtained starting with the symptomatic leg, followed by the highest brachial pressure. A decrease in ABI to < 0.90 or decrease in ABI of more than 20% immediately following exercise is diagnostic for PAD. Ultrasound Images are acquired through sending and receiving sound wave bundles known as pulses. The ultrasound transducer frequency determines the ideal depth of imaging [5]. Higher frequencies are superior for more superficial structure and low frequencies are ideal for deep structure. The structure of interest should be perpendicular to the ultrasound beam to obtain the brightest image. This is readily achieved in vascular imaging because the neck, extremity, and visceral vessels generally lie parallel to the surface of the skin. Unfortunately,

4

Chapter 1.  Noninvasive Vascular Testing SEGMENTAL PRESSURE AND PVR STUDY Brachial RIGHT LEFT 141 148

0

0.90 PVR Gain:

RIGHT Above Knee 67mmHg 926cc Spd: 25 .75 mmHg/20mm Amp: 13

0.45

RIGHT Below Knee 66mmHg 318cc Spd: 25 .75 mmHg/20mm Amp: 29

PVR Gain:

PVR Gain:

>220

133

125

0.84 PVR

RIGHT 0.63

PVR Gain:

>220

LEFT Gain: 93 DP 66 PT

70mmHg 999cc LEFT Above Knee Spd: 25 .75 mmHg/20mm Amp: 11

90 0.61 74 0.50

PVR

ABI: 0.61 Gain:

LEFT Below Knee 65mmHg 265cc Spd: 25 .75 mmHg/20mm Amp: 34

RIGHT Ankle 62mmHg 161cc Spd: 25 .75 mmHg/20mm Amp: 11

PVR Gain:

LEFT Ankle 62mmHg 151cc Spd: 25 .75 mmHg/20mm Amp: 14

RIGHT 56mmHg 75cc Metatarsal Spd: 25 Amp: 02 .75 mmHg/20mm

PVR Gain:

LEFT Metatarsal 53mmHg 88cc Spd: 25 .75 mmHg/20mm Amp: 03

ABI: 0.63

Figure 1.2  Segmental Doppler pressure and pulse volume recording. In this physiologic study the pressure at each level of the legs is seen in the box. Thigh systolic pressure cannot be accurately determined due to compressibility artifact, seen as pressure > 220 mmHg. There is a drop in pressure between the upper and lower calves; this indicates infrapopliteal stenoses are present. The bilateral ankle brachial index (ABI) less than 0.90 is diagnostic of peripheral artery disease. The pulse volume waveforms are seen at each level in the right and left column. The waveforms widen and amplitude decreases in the infrapopliteal region, consistent with obstructive disease

Chapter 1.  Noninvasive Vascular Testing

5

dense objects, such as plaque with calcium deposits, do not permit sound waves to penetrate and cause acoustic shadowing [6]. Vascular ultrasonography evaluates flow velocity using Doppler shift frequencies by insonating the vessel at an angle that is 30–60° to flow (Fig. 1.3). The returning frequency will shift, either positively or negatively, depending on the direction of blood flow. The concentric or laminar blood flow is disturbed at branching points or in the presence of abnormal walls. The spectral waveform provides information about flow at the site of interrogation as well as proximal and distal to that site (Fig.  1.4). The frequency shift data can also be displayed as color Doppler, and aliasing occurs at the site of stenosis when the flow velocity exceeds the Nyquist limit (i.e., when the Doppler frequency shift exceeds one half the pulse 90° No frequency shift 60°

50% of maximal shift

θ Angle between insonation beam and flow

Blood flow Fd = (2 . Fo. V . cos θ) / c is the Doppler equation used in determining velocity of blood flow

Figure 1.3  Doppler angle used to determine flow velocity. B mode gray scale imaging is obtained with transducer perpendicular to the blood vessel. The insonation beam angles away from 90 degrees in order to obtain velocity. The standard is an angle of 60° between the insonation beam and the blood flow channel. Components of the Doppler equation given in the picture are: Fd = Doppler shift, Fo = Carrier frequency, V = Blood flow velocity, c = Speed of sound

Chapter 1.  Noninvasive Vascular Testing

6

b1

c

a

b2

Figure 1.4 Spectral Waveform Proximal and Distal to Arterial Stenosis. Spectral waveform (A) is first obtained proximal to a stenosis. There is a mild delay in upstroke suggesting more proximal disease. Peak systolic velocity is normal at 50  cm/s (B1). Peak systolic velocity increases at the site of stenosis to 350 cm/s. this indicates high grade stenosis at this site. The image (B2) shows the sample volume in the vessel at the site of great color Doppler signal. Gray scale image shows the artery (arrow) as a tube crossing the image from left to right with material encroaching upon the lumen (∗). The spectral waveform in (C) is obtained distal to the stenosis and shows the delay in upstroke and diminished amplitude expected after a high grade stenosis, i.e. tardus et parvus waveform

repetition frequency). Color aliasing occurs at the site of stenosis when the flow velocity exceeds the Nyquist limit (i.e., when the Doppler frequency shift exceeds one half the pulse repetition frequency). Ultrasound is cost effective, portable and free of ionizing radiation. It has become the gold standard for evaluation of lower extremity deep vein thrombosis [7]. It is also recommended in abdominal aortic aneurysm screening. The other benefit of vascular ultrasound is the characterization of flow velocity and direction. It is also used for carotid stenosis evaluation, pseudoaneurysm detection, assessment of arteriovenous fistulae and venous reflux examination.

Chapter 1.  Noninvasive Vascular Testing

7

Magnetic Resonance (MR) Contrast enhanced magnetic resonance angiography (CE MRA) provides images with high quality spatial resolution. The acquisition of MR images starts with detection of spinning hydrogen protons and the response of tipping those magnetic fields with a radiofrequency pulse. The MR signal happens when the spins are brought back into phase [8]. Tissue resolution with MR is excellent. T1 measures signal recovery; tissues with short T1 are bright, whereas tissues with long T1 are dark. For example, fat has a very short T1. T1 is unique among tissues and is used for image contrast along with the intrinsic T2 of tissue. Magnetic resonance angiography (MRA) imaging relies on selective imaging of moving blood, since blood flowing into the imaging field has not experienced the RF pulse [9]. The signals in the blood vessels are then maximized, in contrast to the signals from the adjacent tissues (Fig. 1.5). Methods can depict blood as either black or white. Overestimating the degree of stenosis is often due to signal loss in the areas of complex flow. Contrast enhanced imaging can take place in one breath hold. Visualization of the arterial system without contrast is limited by long acquisition times (more than one breath-­hold), thus increasing the risk of motion artifact. Timing [10] with respect to gadolinium bolus is used to image arteries and veins when they are at their peak filling time. Peak filling is clearly affected by flow issues like decreased cardiac output and local obstructive disease. Data can be reformatted in many ways with post processing. The potential complication of nephrogenic systemic fibrosis that was rarely seen with gadolinium is no longer seen with the latest generation of contrast agent. MR images tissue very well and is therefore good at detecting vessel wall edema and characterizing atherosclerotic plaque composition [11, 12]. Imaging over a long distance like the length of the leg is dependent on appropriate timing of contrast infusion and image acquisition and can be contaminated by venous opacification distally [13].

8

Chapter 1.  Noninvasive Vascular Testing

Figure 1.5  MRA. Gadolinium MRA of the chest in a young man with acute left arm swelling demonstrates (arrow) a short, segmental thrombosis of the left subclavian vein at the thoracic outlet near the left costoclavicular space. No mass is seen adjacent to the thoracic outlet. No arterial compression is seen

Computed Tomography (CT) Computed tomography angiography (CTA) uses volumetric data acquisition with multidetector CT. Submillimeter spatial resolution is now possible. X-rays are necessary for imaging, and attention is given to limiting the dose as much as is possible. Detector improvements have focused on increasing volume and resolution of imaging [14]. CT value is normalized to the attenuation properties of water and is reported as “Hounsfield units” (HU). The HU value is specific for the type of tissue [15]. The scanning mode can be helical or axial, with less radiation with axial. Electrocardiogram gating is needed for structures prone to cardiac motion artifact such as the ascending aorta. Renal function must be evaluated prior

Chapter 1.  Noninvasive Vascular Testing

9

Figure 1.6  CTA. CT angiogram of the chest in a man with acute chest and back pain showing dissection flap (arrow) in the aortic arch. The remaining images were rapidly acquired and identify that the dissection extends to both iliac arteries

to contrast administration. Contrast use is optimized by using test bolus or multiple small boluses. There are many options for post processing to create the images [16]. Artifacts include beam hardening, which is loss of information where there is calcium, pacemaker wires and other hard object. Partial volume effects occur when there is complex tissue being evaluated. The rapid acquisition in CTA makes it ideal for detecting dissection flaps [17]. Speed and availability make CT excellent for detection of pulmonary embolism [18] and acute aortic syndromes (Fig. 1.6). The spatial resolution is excellent for characterizing wall abnormalities such as fibromuscular

10

Chapter 1.  Noninvasive Vascular Testing

Table 1.1  Noninvasive vascular testing methods Method Advantages Disadvantages Physiologic Provides total Non imaging, individual testing volume of flow in the vessels not seen entire limb segment Ultrasound

No ionizing radiation Portable Provides local flow in a vessel Identify wall characteristics

Artifacts from bone, air, interfaces Best resolution with superficial structures

Computed tomography

Rapid, high quality images Good spatial and temporal resolution

Iodine nephrotoxicity Ionizing radiation

Magnetic resonance

High quality images Excellent tissue resolution Gadolinium less nephrotoxic than iodine

Rare nephrogenic fibrosis with gadolinium contrast Longer acquisition times, particularly if no gadolinium is used

All are operator dependent. Local expertise in individual techniques can vary widely

dysplasia. Rapid acquisition that is readily available makes CT the diagnostic test of choice for pulmonary embolism (Table 1.1). Role of Imaging in Vascular Diagnosis Nonatherosclerotic arterial disease can range from an incidental finding to severe illness in the presence of end organ ischemia [19, 20]. This schema (Fig. 1.7) for evaluation of nonatherosclerotic vascular disease depicts imaging and physical exam findings on the y axis and artery size on the x axis. Patient evaluation should always begin with a detailed history and physical examination with a careful evaluation of peripheral pulses, auscultation of potential bruits, and a thorough skin exam. Blood testing can include complete blood count (CBC), electrolytes, renal function, anti-nuclear antibody (ANA), anti-

AORTA

EXAM HENOCH SCHONLEIN PURPURA arthralgia High IgA CRYOGLOBULIN Raynaud’s phenomenon cryoglobulin, Hepatitis C antibody ANTI GLOMERULAR BASEMENT MEMBRANE antiGBM antibody LEUKOCYTOCLASTIC VASCULITIS

FIBROMUSCULAR DYPLASIA carotid, coronary, renal involvement THROMBOANGIITIS OBILERANS superficial thrombophlebitis, cigarette use

BEHCET’S ulcers, uveitis RHEUMATOID ARTHRITIS rheumatoid deformity, Raynaud’s phenomenon rheumatoid factor LUPUS systemic symptoms, Raynaud’s phenomenon ANA SYSTEMIC SCLEROSIS Raynaud’s phenomenon anti Scl70, anti-centromere DRUG INDUCED ANCA hydralazine, PTU, ANCA

POLYARTERTIS NODOSA (PAN) renal ANCA, hepatitis B antibody KAWASAKI childhood high fever, coronary artery aneurysm EOSINIOPHILIC GRANULOMATOSIS pulmonary and renal involvement eosinophils, ANCA GRANULOMATOSIS WITH POLYANGIITIS pulmonary and renal involvement ANCA MICROSCOPIC ANGIITIS pulmonary involvement ANCA

CAPILLARIES

Figure 1.7  Role of imaging in vascular diagnosis. The x and y axes identify that arterial size and imaging findings are key at arriving at a diagnosis. Each diagnosis is listed under artery size and is followed by clinical clues in orange and lab tests in pink. It provides an excellent starting point for cases of nonatherosclerotic vascular disease

PALPABLE PURPURA

ARTERY SIZE FROM LARGE TO SMALL

GIANT CELL ARTERITIS polymyalgia rheumatica, atherosclerosis, temporal arteritis TAKAYASU ARTERITIS age less than 50 IGG4 superficial thrombophlebitis eosinophils, IgG4 > 86 mg/dL INFECTION septic emboli

“STRING OF BEADS” SEGMENTAL STENOSIS, CORKSCREW COLLATERALS

“HALO” AROUND ARTERY CIRCUMFERENTIAL MEDIAL THICKENING TUBULAR STENOSIS AND ANEURYSM WITH HEALING

IMAGING

Chapter 1.  Noninvasive Vascular Testing 11

12

Chapter 1.  Noninvasive Vascular Testing

neutrophil cytoplasmic antibodies (ANCA), and syphilis screening (RPR-VDRL) [21, 22]. This schema is extensive, but not exhaustive. Nonetheless, it provides an excellent starting point for cases of nonatherosclerotic arterial disease. Clinical Vignette  On evaluation each ankle brachial index measurement is 0.80. Segmental pressure measurements do not identify discrete stenosis. CTA shows diffuse tubular narrowing throughout the arteries of both legs. Duplex ultrasound demonstrates circumferential echolucence around the arteries, consistent with arteritis (Fig. 1.8). The patient is

Figure 1.8  Duplex ultrasound of the femoral arteries. Color demonstrates flow in the arteries in this cross sectional image. Surrounding the artery is a “halo” (*) that is echolucent and is diagnostic for arteritis

References

13

treated with a long course of prednisone and has complete resolution of symptoms [23].

References 1. Gerhard-Herman MD, Gornik HL, Barrett C, Barshes NR, Corriere MA, Drachman DE, et  al. 2016 AHA/ACC guideline on the management of patients with lower extremity peripheral artery disease: executive summary: a report of the American college of cardiology/American Heart Association task force on clinical practice guidelines. Circulation. 2017;135(12):e686–725. 2. Aboyans V, Ricco JB, Bartelink MLEL, Björck M, Brodmann M, Cohnert T, et  al. 2017 ESC guidelines on the diagnosis and treatment of peripheral arterial diseases, in collaboration with the European Society for Vascular Surgery (ESVS). Eur J Vasc Endovasc Surg. 2017;39:763–816. 3. Blake DF, Young DA, Brown LH.  Transcutaneous oximetry: normal values for the lower limb. Diving Hyperb Med. 2014;44:146–53. 4. Kovacs D, Csiszar B, Biro K, Koltai K, Endrei D, Juricskay I, et al. Toe-brachial index and exercise test can improve the exploration of peripheral artery disease. Atherosclerosis. 2018;269:151–8. 5. Kremkau FW. Principles of spectral Doppler. J Vasc Ultrasound [Internet]. 2011;35(4):15. http://www.ingentaconnect.com/ content/svu/jvu/2011/00000035/00000004/art00005 6. Abreu I, Roriz D, Barros M, Moreira A, Caseiro AF.  B-mode ultrasound artifacts. Eur Soc Radiol [Internet]. 2015;1–48. www. myESR.org 7. Bounameaux H, Perrier A, Righini M.  Diagnosis of venous thromboembolism: an update. Vasc Med. 2010;15:399–406. 8. Nitz WR, Balzer T, Grosu DS, Allkemper T. Principles of magnetic resonance. In: Clinical MR imaging (3rd Edition): a practical approach; 2010. 9. Xin Liu, Zhaoyang Fan, Na Zhang, Qi Yang, Fei Feng, Pengcheng Liu, Hairong Zheng, Debiao Li. Unenhanced MR Angiography of the Foot: Initial Experience of Using Flow-Sensitive Dephasing–prepared Steady-State Free Precession in Patients with Diabetes. Radiology. 2014;272(3):885–94. 10. Christensen S, Calamante F, Hjort N, Wu O, Blankholm AD, Desmond P, et al. Inferring origin of vascular supply from tracer arrival timing patterns using bolus tracking MRI. J Magn Reson Imaging. 2008;27(6):1371–81.

14

Chapter 1.  Noninvasive Vascular Testing

11. Wedeen VJ, Hagmann P, Tseng WYI, Reese TG, Weisskoff RM.  Mapping complex tissue architecture with diffusion spectrum magnetic resonance imaging. Magn Reson Med. 2005;54(6):1377–86. 12. Yuan C, Kerwin WS, Ferguson MS, Polissar N, Zhang S, Cai J, et al. Contrast-enhanced high resolution MRI for atherosclerotic carotid artery tissue characterization. J Magn Reson Imaging. 2002;15(1):62–7. 13. Czum JM, Corse WR, Ho VB. MR angiography of the thoracic aorta. Magn Reson Imaging Clin N Am. 2005;13(1):41–64. 14. Kalender WA.  X-ray computed tomography. Phys Med Biol. 2006;51(13):R29–43. 15. Kapoor BS, Esparaz A, Levitin A, McLennan G, Moon E, Sands M.  Nonvascular and portal vein applications of cone-beam computed tomography: current status. Tech Vasc Interv Radiol. 2013;16(3):150–60. 16. Rajiah P. CT and MRI in the evaluation of thoracic aortic diseases. Int J Vasc Med. 2013; 17. Chiles C, Carr JJ. Vascular diseases of the thorax: evaluation with multidetector CT. Radiol Clin N Am. 2005;43(3):543–69. 18. Albrecht MH, Bickford MW, Nance JW, Zhang L, De Cecco CN, Wichmann JL, et  al. State-of-the-art pulmonary CT angiography for acute pulmonary embolism. Am J Roentgenol. 2017;208:495–504. 19. Wu W, Chaer RA.  Nonarteriosclerotic vascular disease. Surg Clin N Am. 2013;93(4):833–75. 20. Southerland AM, Meschia JF, Worrall BB. Shared associations of nonatherosclerotic, large-vessel, cerebrovascular arteriopathies: considering intracranial aneurysms, cervical artery dissection, moyamoya disease and fibromuscular dysplasia. Curr Opin Neurol. 2013;26(1):13–28. 21. Bossuyt X, Cohen Tervaert JW, Arimura Y, Blockmans D, Flores-Suáez LF, Guillevin L, et  al. Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis. Nat Rev Rheumatol. 2017;13(11):683–92. 22. Luqmani RA. Disease assessment in systemic vasculitis. Nephrol Dial Transpl. 2015;30:i76–82. 23. Germanò G, Monti S, Ponte C, Possemato N, Caporali R, Salvarani C, et al. The role of ultrasound in the diagnosis and follow-­up of large-vessel vasculitis: an update. Clin Exp Rheumatol. 2017;35:194–8.

Chapter 2 Evaluation of Leg Pain

Objective  Utilize history and physical examination to determine the etiology of leg pain. Vignette  An 80 year old man presents to the office for evaluation of leg pain. His pain radiates down both lower legs and progresses over the course of the day. He particularly notices it when shopping for groceries, and he typically leans over onto his grocery cart to help alleviate the pain. He never smoked and has no history of diabetes or hypertension. Causes and features of leg pain are given in Table 2.1. This list is not exhaustive; rather it aims to cover the various categories of leg pain. Etiologies involve abnormalities of arteries, veins, nerves and joints, and primary muscular abnormalities should be considered along with arteries in this framework. It provides a simple schema to use in evaluating individuals with leg discomfort. The location and clinical characteristics of the pain are described. Pain radiating down the lateral leg suggests nerve compression, while pain starting distally in the large muscles is consistent with peripheral artery disease. More con-

© Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_2

15

Aching on waking

Arthritis [6] Joint primarily involved

Worse with Slow relief Can improve with exercise rest

Pain moving joint

Knee arthritis

Worse with Present at None exercise rest

Tenderness, swelling is constant

Area of pain corresponds to specific nerve root

Baker’s cyst Behind knee [5] and down calf

No reliable No reliable Improved when effect effect sitting or supine

Normal pulses. Pain changes with lumbar flexion and extension

Brawny skin, distended peripheral veins and venules

Sharp, stabbing pain

Exam Pulses may be decreased and ankle pressure may be less than arm pressure

Calf, but can be Tight, exploding pain Worse with Decreases Not present at rest Venous significant slowly claudication entire leg exertion [4]

Nerve root Radiates down compression the leg [3]

Posterior leg, lateral thigh, bilateral buttocks

Pain moves proximal Sometimes Sitting or Upright worsens flexing the to distal with spine is associated weakness required Can be heavy and for relief tired Numb and tingling

Leg elevation can cause pallor, dependent position may cause rubor

Position

Spinal stenosis [2]

Often improves

Rest

Heavy, tired, sometimes crampy Pain moves distal to proximal

Any large Peripheral artery disease muscles of leg and or buttocks [1] e.g. calves and thighs

Worsens

Table 2.1  Diagnoses of leg pain and associated features Diagnosis Location Pain characteristic Exercise

16 Chapter 2.  Evaluation of Leg Pain

Chapter 2.  Evaluation of Leg Pain

17

stant pain in the calf could be due to ruptured Baker’s cyst. If the calf discomfort occurs with significant exertion and is described as tightness, this suggest venous claudication. Timing is important, as it is useful to know if the pain is present on waking in the morning or only after exercise. Pain in the joints upon waking in the morning is strongly suggestive of arthritis. Understanding how the pain is relieved is a significant clue in understanding the etiology. Does the individual need to rest or is sitting required for relief? Relief with flexion of the spine suggests spinal stenosis is causing the leg pain. Distribution of pain will also help determine the etiology (Fig. 2.1). Pain that involves the large muscles typically has an arterial etiology, seen in red on the figure. This can be peripheral artery disease or myositis, for example. The posterior calf can ache due to venous claudication or ruptured Baker’s cyst. Medial calf discomfort that is worse with standing is often due to venous insufficiency. The posterior and lateral thigh pain is often due to spinal stenosis and nerve root compression. The areas that can be involved with joint pain include those represented by green circles. Clinical Vignette Pain that radiates into both legs and is relieved by flexion of the spine is consistent with spinal claudication secondary to spinal stenosis. This is confirmed by resting and exercise ABIs of 1.05 in both limbs, thus confirming his symptoms are not due to atherosclerotic peripheral artery disease.

18

Chapter 2.  Evaluation of Leg Pain

Joint

Vein

Artery

Nerve

Figure 2.1  The distribution of leg pain according to cause. This is a schematic of the anterior and side views of the leg. The areas commonly involved in etiologies of leg discomfort due to joint (green), artery (red), vein (blue) and nerve are indicated

References

19

References 1. Gerhard-Herman MD, Gornik HL, Barrett C, Barshes NR, Corriere MA, Drachman DE, et al. 2016 AHA/ACC guideline on the management of patients with lower extremity peripheral artery disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on clinical practice guidelines. J Am Coll Cardiol. 2017;69(11):1465–508. 2. Lurie J, Tomkins-Lane C. Management of lumbar spinal stenosis. BMJ. 2016;352:h6234. 3. Li Y, Fredrickson V, Resnick DK. How should we grade lumbar disc herniation and nerve root compression? A systematic review. Clin Orthop Relat Res. 2015;473:1896-1902 4. Gloviczki P, Comerota AJ, Dalsing MC, Eklof BG, Gillespie DL, Gloviczki ML, et al. The care of patients with varicose veins and associated chronic venous diseases: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum. J Vasc Surg. 2011;53:2S–48S. 5. Abid A, Kelley JF, Flemming DJ, Silvis ML. A young male runner with a posterior knee mass  – not just your typical Baker’s cyst. BMJ Case Rep. 2016. 6. Benjamin EJ, Blaha MJ, Chiuve SE, Cushman M, Das SR, Deo R, et al. Heart disease and stroke statistics’ 2017 update: a report from the American Heart Association. Vol. 135, Circulation. Lippincott Williams and Wilkins; 2017. p. e146–e603.

Chapter 3 Peripheral Artery Disease

Vignette  A 70 year old woman presents to the office for follow up of hypertension. She is taking her prescribed medications and eating a healthy diet. She does not walk 30 minutes a day. She describes right calf heaviness when she walks up a flight of stairs or when her walk takes her up hills. This goes away when she stands and rests. No particular position triggers the discomfort. Examination is notable for rubor of her right foot and calves when she is sitting on the examination table. Rubor goes away when she is lying flat on the examination table. Recognition of peripheral artery disease (PAD) begins with recognition of symptoms resulting from transient decrease in blood flow to the limb. The clinical history includes understanding of cardiovascular risk factors and precisely how much walking an individual performs daily. Rarely does one elicit classic claudication symptoms; rather, there is wide variation in how limb discomfort in PAD is described. Classically, the discomfort occurs in the calves, thighs, and/ or buttocks during exertion and resolves within minutes of resting [1]. The time to onset of symptoms with a defined activity and time to resolution when activity ceases is usually reproducible within an individual. Examination may reveal abnormal pulses, arterial bruits, elevation pallor or dependent rubor of the limb, or dry ulcer on the distal extremity. PAD is recognized by identifying an ankle- brachial index (ABI) measurement ≤ 0.90 (Fig. 3.1). The ABI can be mea© Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_3

21

22

Chapter 3.  Peripheral Artery Disease

150

140

100

150

90

110

Right ABI 0.66 100 150

Left ABI 1.0 150 150

Figure 3.1  The ankle brachial index (ABI) measurement. The person lies supine for 10 minutes before measurement is taken, allowing resting hemodynamics to be restored. An appropriately sized blood pressure cuff is placed above the antecubital fossa and ankles. Systolic blood pressure is measured on the posterior tibial, dorsalis pedis and brachial arteries with a Doppler probe. Each leg ABI is calculated by dividing the highest ankle pressure by the highest arm pressure. For example, if the highest ankle pressure is 100 mm Hg and the highest arm pressure in 150 mm Hg the ABI is 0.75. Doppler probe is used to auscultate the artery and the cuff is inflated to suprasystolic pressure. The pressure when the Doppler signal is again audible is the pressure for that measurement

Chapter 3.  Peripheral Artery Disease

23

sured after exercise if the resting ABI is normal but clinical suspicion of PAD remains high [2]. As described in the previous chapter, Duplex ultrasonography, CT angiography, and MR angiography can all provide additional information on the location and severity of PAD (Fig. 3.2). ABI normally increases with age. This occurs earlier than otherwise expected in those individuals with diabetes and cigarette smoking. The risk of abnormal ABI is modest with hypertension and dyslipidemia. However, treatment of hypertension results in lower central blood pressure and further decrease in absolute pressure beyond a stenosis in the artery. Disabling claudication may become evident and merits treatment in this case. Diabetes and cigarette smoking are both strong risk factors for PAD [3]. PAD can progress to critical limb ischemia, which includes leg pain at rest, non-healing arterial ulcer, and/or gangrene. Although this occurs in the minority of patients with PAD, it may be the first manifestation in some individuals. Abnormal ABI is a very strong predictor of cardiovascular morbidity and mortality [4]. Specific

aorta right iliac stenosis left iliac stenosis

Figure 3.2 MRA abdomen and pelvis. Arrows indicate the aorta and iliac arteries. Tight ostial right common iliac artery stenosis is evident. The proximal left common iliac artery is occluded, with a narrow common iliac artery apparent after the origin and proximal segment due to filling via a collateral vessel

24

Chapter 3.  Peripheral Artery Disease

to the leg, decreased ABI results in skeletal muscle changes over time such as increased fat content. Acute limb ischemia is an important cause of leg pain that, if unrecognized, can result in limb loss of death. It occurs due to sudden obstruction of arterial flow to the limb due to thromboembolism from a proximal arterial segment or the heart itself. Time urgency is due to the fact that the skeletal muscle cannot tolerate lack of arterial blood flow for more than 4 to 6 hours. Patients typically present with leg pain and pallor. Loss of arterial pulse by Doppler indicates that the limb is threatened. Loss of capillary refill and sensation indicates the need for emergent revascularization. Causes for PAD other than atherosclerosis may also need to be considered if an individual is young, does not have diabetes or have a history of smoking (Table 3.1). History will be an important clue in many types of PAD not due to atherosclerosis. Exposures such as adrenergic stimulants increase the likelihood of vasospasm. Heavy cigarette smoking is an exposure that can lead to thrombo angiitis obliterans. There is often accompanying Raynaud’s phenomenon, superficial thrombophlebitis and distal sensory abnormality [5]. Abrupt onset can suggest embolus or thrombosis of the artery. Examination can yield findings in addition to the decreased ABI, such as popliteal fossa mass in the presence of adventitial cyst or popliteal artery aneurysm. Treatment of PAD due to atherosclerosis can include lifestyle modification, medication and revascularization. The goal is to improve both limb and cardiovascular outcomes. A heart healthy diet [6] is important and can be taught with examples like the Healthy Plate Eating advocated by the Harvard School of Public Health. Smoking cessation clearly decreases both claudication and risk of limb loss as well as improves cardiovascular outcomes like myocardial infarction and stroke [1]. Smoking cessation programs can be more successful if pharmacotherapy is used as part of the program. Every cigarette a patient does not smoke should be applauded as cessation rates are very low, typically around 5%. Regular exercise is important for overall health and exercise can be

Chapter 3.  Peripheral Artery Disease Table 3.1  PAD not due to atherosclerosis Entity Clues Arteritis Uniform size arteries affected (small, medium, large). Systemic disease evident. Elevated inflammatory markers and disease specific antibodies.

25

Imaging Tubular stenosis. Artery with thick edematous wall

Embolus or thrombosis

Sudden onset PAD. History of atrial fibrillation, cardiomyopathy, hypercoagulable state, deep vein thrombosis plus patent foramen ovale

Abrupt cut off of arterial flow in artery

Fibromuscular dysplasia

Often with carotid or renal disease

Artery looks like “beads on a string”

Vasospasm

Stimulants, cocaine, chemotherapy, radiation, or ergots

Tubular stenosis, possible with bea­k -like appearance

Dissection

Multiple sites in systemic medial arteriolysis, iatrogenic after arterial intervention or extension from aortic dissection

Intimal flap

Adventitial cyst, popliteal artery aneurysm

Mass in popliteal fossa

Obstructive syndrome

Popliteal artery entrapment

Popliteal artery lumen narrowed by cyst or by thrombus lining a popliteal artery aneurysm. Thrombosis of popliteal aneurysm with no flow. Evaluation at rest and with plantar flexion shows increase in flow velocity or obliteration of lumen

26

Chapter 3.  Peripheral Artery Disease

Table 3.2  Exercise for PAD Intervention Location Supervised Cardiac exercise rehabilitation therapy facility

Features Training at least 30 min for 3 times a week. Intermittent walking to claudication followed by rest

Home or community exercise

Patient choses where they would like to walk (e.g. outside or mall)

Prescribe walking to claudication followed by rest for 30 min at least 3 times a week

Arm ergometry

In home

Arm cycling with one pound weight or arm cycle beginning with 5 min and working up to 30 min for 3 times a week

powerful therapy for individuals with PAD. There is a range of options for exercise in PAD, with the strongest proven benefit in improved walking time for supervised exercise programs [7] (Table 3.2). A number of different medications (Table 3.3) can improve outcomes for patients with PAD and will have applications unique to the individual patient. Treatment of hypertension will decrease cardiovascular outcomes with no clear advantage for specific classes of antihypertensive medication. Diabetes increases the risk and progression of PAD [8]. Appropriate glycemic control remains a core aspect of medical management. Novel classes of diabetes therapies, including sodium glucose cotransporter 2 (SGLT2) inhibitors and glucagon like peptide 1 (GLP-1) agonists, have additional cardiovascular benefits independent of their glucose lowering effects. Although inflammation is a key part of the pathogenesis of PAD, no targeted anti-inflammatory therapy has been shown to benefit individuals with PAD. Lipid lowering decreases cardiovascular risk in those with PAD, and both statins and proprotein convertase subtilisin kexin type 9 (PCSK9) inhibitors reduce limb events in individuals with PAD [9, 10].

Chapter 3.  Peripheral Artery Disease

27

Table 3.3  Medications for patients with PAD Cardiovascular Medication benefit Antihypertensive Yes

No

Cholesterol lowering

Yes

Yes

Glucose lowering

Yes

Not shown

Antiplatelet therapy

Yes

Yes

Antithrombotic therapy

Yes

Yes (low-dose rivaroxaban)

Cilostazol

No

Yes

Pentoxyfylline

No

Maybe

L-carnitine

No

Yes

Ranolazine

No

Not shown

Limb benefit

Patients with atherosclerotic PAD also benefit from antithrombotic therapy. Monotherapy with clopidogrel or aspirin is indicated for symptomatic patients. High risk individuals, including those with a history of prior lower extremity revascularization, may warrant more intense regimens. Amputation rates appear lower for patients with PAD on the proteaseactivated receptor (PAR)-1 antagonist vorapaxar [11]. More intensive antithrombotic regimens have the clearest benefits in individuals with concomitant coronary artery and/or cerebrovascular disease. Anticoagulation with warfarin did not benefit patients with PAD [12]. In contrast, low dose factor Xa inhibition with rivaroxaban in conjunction with aspirin improved both cardiovascular and limb outcomes in individuals with PAD [13]. Revascularization is used when there is disabling claudication despite optimal medical therapy or critical limb ischemia [14]. Important considerations in selecting the type of revascularization include both peripheral artery anatomy and the need to improve both vascular inflow and outflow in the limb (Fig. 3.3). Techniques include percutaneous angio-

28

Chapter 3.  Peripheral Artery Disease

Significant co-morbidities Staged approach possible No veins to use for grafts

FAVOR ENDOVASCULAR

Location, e.g. CFA lesion Single artery outflow Heavily calcified lesions

FAVOR SURGERY

Figure 3.3 Choosing revascularization strategy for PAD.  All features of anatomy, urgency and individual perioperative risk are weighed together in coming to a final decision

plasty and stenting, bypass grafting and endarterectomy. Local expertise, evolving technology and individual perioperative risk will also play a role in determination of optimal revascularization in each individual patient. Clinical Vignette  Calf discomfort with activity that goes away with rest is suggestive of PAD. This diagnosis is supported by the presence of dependent rubor on examination. This diagnosis is confirmed with an ABI measurement of 0.72 in the right limb. She is managed with the addition of aspirin and atorvastatin to her medical regimen, and she is prescribed supervised exercise therapy. At her follow-up visit 6 months later, her symptoms are much improved and no longer limit her daily activities.

References 1. Gerhard-Herman MD, Gornik HL, Barrett C, Barshes NR, Corriere MA, Drachman DE, et al. 2016 AHA/ACC Guideline on the Management of Patients with Lower Extremity Peripheral Artery Disease: Executive Summary. Circulation. 2017;135:e686–725. 2. Aday AW, Kinlay S, Gerhard-Herman MD. Comparison of different exercise ankle pressure indices in the diagnosis of peripheral artery disease. Vasc Med (UK). 2018;23:541–48.

References

29

3. Sigvant B, Lundin F, Wahlberg E. The risk of disease progression in peripheral arterial disease is higher than expected: A metaanalysis of mortality and disease progression in peripheral arterial disease. Eur J Vasc Endovasc Surg. 2016;51:395–403. 4. Criqui MH, McClelland RL, McDermott MM, Allison MA, Blumenthal RS, Aboyans V, et al. The ankle-brachial index and incident cardiovascular events in the MESA (Multi-Ethnic Study of Atherosclerosis). J Am Coll Cardiol. 2010;56:1506–12. 5. Piazza G, Creager MA. Thromboangiitis obliterans. Circulation. 2010;121:1858–61. 6. Arnett DK, Blumenthal RS, Albert MA, Buroker AB, Goldberger ZD, Hahn EJ, et al. ACC/AHA guideline on the primary prevention of cardiovascular disease: executive summary: A report of the American college of cardiology/American heart association task force on clinical practice guidelines. Circulation. 2019;140(11):e563–95. 7. Treat-Jacobson D, McDermott MM, Bronas UG, Campia U, Collins TC, Criqui MH, et al. Optimal exercise programs for patients with peripheral artery disease: A scientific statement from the American heart association. Circulation. 2019;139:e10–33. 8. Singh S, Armstrong EJ, Sherif W, Alvandi B, Westin GG, Singh GD, et al. Association of elevated fasting glucose with lower patency and increased major adverse limb events among patients with diabetes undergoing infrapopliteal balloon angioplasty. Vasc Med (UK). 2014;19:315–16. 9. Bonaca MP, Nault P, Giugliano RP, Keech AC, Pineda AL, Kanevsky E, et al. Low-density lipoprotein cholesterol lowering with evolocumab and outcomes in patients with peripheral artery disease: Insights from the FOURIER trial (Further Cardiovascular Outcomes Research With PCSK9 Inhibition in Subjects With Elevated Risk). Circulation. 2018;137:338–50. 10. Arya S, Khakharia A, Binney Z, DeMartino R, Brewster L, Goodney P, et al. Association of statin dose with amputation and survival in patients with peripheral artery disease. Circulation. 2018;137:1435–46. 11. Bonaca MP, Scirica BM, Creager MA, Olin J, Bounameaux H, Dellborg M, et al. Vorapaxar in patients with peripheral artery disease. Circulation. 2013;127:1522–29. 12. Warfarin Antiplatelet Vascular Evaluation Trial Investigators, Anand S, Yusuf S, Xie C, Pogue J, Eikelboom J, Budaj A, Sussex B, Liu L, Guzman R, Cina C, Crowell R, Keltai M, Gosselin G.

30

Chapter 3.  Peripheral Artery Disease

Oral anticoagulation and antiplatelet therapy and peripheral arterial disease. N Engl J Med. 2007;357:217–27. 13. Anand SS, Bosch J, Eikelboom JW, Connolly SJ, Diaz R, Widimsky P, et al. Rivaroxaban with or without aspirin in patients with stable peripheral or carotid artery disease: an international, randomised, double-blind, placebo-controlled trial. Lancet. 2018;391:219–29. 14. Aboyans V, Ricco JB, Bartelink MLEL, Björck M, Brodmann M, Cohnert T, et al. 2017 ESC guidelines on the diagnosis and treatment of peripheral arterial diseases, in collaboration with the European Society for Vascular Surgery (ESVS). Eur Heart J. 2018;39:763-16.

Chapter 4 Aortic Disease

Objective  To recognize and treat aortic aneurysm and dissection. Vignette  A 54-year-old male with no significant past medical history presents with 3 day history of sharp chest and back pain. Pain has been similar in character since it began. On examination he appears uncomfortable with a heart rate of 80  bpm and a systolic blood pressure of 150 mm Hg in both arms. A tambour second heart sound is appreciated. His extremities are warm, and peripheral pulses are symmetric throughout. Anatomy The aorta is a single vessel yet differs in structure throughout its length. Diameter averages 4  cm at the root, 3  cm in the ascending aorta, 2.5 cm in the descending aorta and 2 cm in the abdomen. The layers of the aortic wall from innermost to outermost are the intima, media, and adventitia. The vascular smooth muscles cells of the media derive from different embryological sources (Fig.  4.1). Aneurysm is defined as a 50% increase in diameter when compared to a proximal normal segment. Expansion in size can be circumferential (fusiform) or a focal outpouching (saccular) of the aortic wall [1].

© Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_4

31

32

Chapter 4.  Aortic Disease Aortic arch

Ascending aorta

Descending Aorta (after subclavian) Sample of spinal arteries Diaphragm Abdominal Aorta (below diaphragm)

Secondary heart field

Neural crest

Somites

Splanchnic mesoderm

Mesothelium

Figure 4.1  Aorta anatomic site and smooth muscle cell embryologic origin. The anatomic site is indicated by arrow. The ascending, arch and descending aorta are all located above the diaphragm. The abdominal aorta begins at the level of the diaphragm. The embryologic origin of the smooth muscle cells is identified by color with the legend at the right. The figure includes branch artery locations including an example of the spinal arteries present along the entire length of the aorta

Chapter 4.  Aortic Disease

33

In contrast, disruption of the wall can result in a contained rupture known as a pseudoaneurysm. Aneurysm Thoracic aortic aneurysm [2] is most likely in the ascending aorta, then descending aorta and finally in the arch. Prevalence is not defined since most are detected incidentally. The natural history of an aneurysm will depend on the rate of expansion. Prior dissection can double the annual average expansion rate. For most individuals, referral for cardiac surgical repair is made when the diameter is greater than 5.5  cm [3]. A diameter of 5.0  cm may be considered as the threshold for repair at high volume centers if there is a biscuspid aortic valve. Those individuals with a genetic disorder may require intervention at an even lower diameter such as 4.5 cm. Potential causes of aortic aneurysms include many genetic disorders, congenital abnormalities, arteritis, trauma and prior dissection (Table  4.1). Recognition of these disorders will inform both surveillance in the case of congenital and structural abnormalities, and medical therapy with arteritis or infection [4]. Arteritis can be evident with a halo of inflammation surrounding the aorta. Pain, fever, and bacteremia are clues to an infectious etiology.

Table 4.1  Disorders associated with aneurysm and dissection Congenital Arteritis Infection Structure Bicuspid Giant cell or Takayasu Tuberculosis Trauma aortic valve arteritis Looeys-­ Dietz syndrome

Seronegative spondyloarthropathies

Syphilis

Cystic medial necrosis

Aortic coarctation

Behcet’s syndrome

Salmonella

Dissection

Marfan syndrome

Systemic lupus erythematosis

Vascular Ehlers-­ Danlos syndrome

Rheumatoid arthritis

34

Chapter 4.  Aortic Disease

Figure 4.2  Abdominal aortic aneurysm. The infrarenal aortic diameter increases three fold in size below the normal aortic diameter seen below the renal arteries (in blue). The aneurysm extends to the iliac bifurcation. This is a fusiform shape. A saccular outpouching shape instead would be suggestive of infection

Abdominal aortic aneurysm (AAA) is more common that thoracic aortic aneurysm (Fig. 4.2). Advancing age, male sex and cigarette smoking all contribute to the number of aneurysms. Only cigarette smoking is modifiable. In addition, there is an increased frequency of AAA in relatives of those with AAA. Aortic repair is generally recommended at diameters of 5.5 cm [5]. Women have an increased rupture rate at smaller diameters, suggesting that diameter may need to be evaluated with respect to body size [6]. The United States Preventive Services Task Force recommends one-time screen-

Chapter 4.  Aortic Disease

35

ing for AAA with ultrasound in men 65–75 years old who have ever smoked cigarettes. At present, the Task Force does not routinely recommend screening in women. Treatment of aneurysm focuses on limiting rupture risk and evaluating expansion size. For example, once AAA is diagnosed surveillance imaging is recommended in 6 months if diameter is 5–5.4 cm or 3 years if diameter is 3–3.9 cm [7]. Smoking cessation along with blood pressure control may limit expansion [8]. Increase in rupture risk occurs with diameter increase, ongoing cigarette use, high blood pressure and chronic lung disease [9]. Evaluation for surgical or endovascular repair depends on aortic diameter, patient co-morbidities, life expectancy and patient preference. Traditional open repair is durable, but also is a difficult to tolerate procedure with notable complications [19, 20]. It is crucial to understand an individual’s perioperative risk and life expectancy. Complications to all organs including the spinal cord can be seen but are uncommon. Indications for repair, including size and rate of aneurysm growth, are the same for open surgical repair and endovascular repair (EVAR). This less invasive technique for the abdominal aorta does require up to 15 mm of non-aneurysmal aorta distal to the renal arteries for adequate stent graft positioning in addition to adequate renal artery diameters. The main longterm complications are endoleak [11], stent graft migration and limb thrombosis. Dissection Acute aortic dissection is a life-threatening emergency [12]. The diagnosis is made by identifying an intimal flap on imaging [13]. The initiating aortic dissection event is an intimal tear through which blood rapidly flows into the media under pressure and splits the layers of the aortic wall, thus creating an intimal flap that separates the true and false lumens (Fig. 4.3). Propagation can contribute to rupture into the pericardial sac as well as interference with end organ perfusion. Forces that weaken the media (Table 4.1) can contribute to

36

Chapter 4.  Aortic Disease

Figure 4.3 Aortic dissection. This CTA identifies a flap (arrow) extending in to the aorta from the arch to the abdominal aorta

dissection. Acute aortic syndromes include dissection, intramural hematoma, penetrating aortic ulcer (PAU) and trauma (Fig. 4.4) [14]. In PAU blood enters the media through disruption of an inflamed plaque. The true occurrence of aortic dissection rate may be unknown because of misdiagnosis and because many individuals present with sudden death. Nonetheless, the incidence of aortic dissection is estimates to be 15 per 100,000 patient years. Location (Fig. 4.5) and time of onset are used to guide treatment [15]. Any onset less than

Chapter 4.  Aortic Disease A

37

B

C

Figure 4.4  Acute aortic syndromes. (A) Aortic dissection with a discrete intimal flap (arrow) dividing the lumen. (B) Intramural hematoma with blood collecting in the medial layer (arrow), with no collection to the lumen. (C) Penetrating aortic ulcer with blood entering the media through the disrupted atherosclerotic plaque (arrow)

14 days is considered acute. Adverse remodeling of the aorta, including aneurysmal expansion and false lumen thrombosis, begins shortly after onset of dissection. The index of suspicion for dissection must be high [16]. Clinical appearance is often abrupt onset of sharp chest or back pain that is maximal intensity from the time of onset [17]. Syncope is a more ominous presentation and may indicate rupture into the pericardium or impairment of cerebral perfusion. Patients appear apprehensive, ill and uncomfortable [18]. Examination findings can include increased jugular venous pressure (type A), short aortic insufficiency murmur (type A), thoracic dullness to percussion, and asymmetric

38

Chapter 4.  Aortic Disease Stanford A DeBakey I

Stanford A DeBakey II

Stanford B DeBakey III

Indicates dissection location

Figure 4.5 Classification of aortic dissection. These aortic figures demonstrate classification according to anatomic location. This helps guide selection of the treatment strategy. The legend indicates the dissection location. Stanford classification is based on involvement of the ascending aorta. Any involving of the ascending aorta is classified as type A. All others will be type B. DeBakey classification is based on both ascending aorta and arch involvement (type 1), ascending only (type 2) and descending aorta involvement exclusively (type 3)

peripheral pulses or blood pressures. Symptomatic pleural effusions are quite common with all aortic dissections. The only way to diagnose dissection is with imaging of the aorta. CT angiography is often the fastest and most widely available modality. MR angiography is also highly sensitive, but this modality takes more time to acquire imaging and is often not appropriate for acutely ill patients. In cases when CT imaging

Chapter 4.  Aortic Disease

39

Suspect aortic dissection History and physical examination Control HR and BP Diagnostic imaging (CTA, TEE or MRA) Is ascending aorta involved?

YES Type A Emergency surgery (additional considerations regarding aortic valve and arch vessel perfusion

NO Type B Is there end-organ malperfusion, rupture, or refractory pain?

YES Endovascular repair

NO Control HR and BP Follow pain, creatinine, lactic acid and aortic remodeling to consider repair

Continue HR and BP control Long term surveillance of aorta

Figure 4.6  Algorithm for assessment and management of aortic dissection

is unavailable, transesophageal echocardiography is another option, although certain segments, including the aortic arch, may be difficult to image. Transthoracic echocardiography is not appropriate to diagnose dissection and typically only delays definitive diagnosis. Imaging findings include discrete intimal flap creating two lumens and crescentic or circumferential wall thickening [19]. Poor prognostic signs include aortic intramural hematoma greater than 11 mm thick as well as aortic diameter greater than 4.8 cm. Pharmacologic therapy targeting a heart rate less than 60 beats per minute and systolic blood pressure near 110 mmHg are important to initial care. Metoprolol, labetolol, esmolol and propranolol have all been beneficial in this regard. Treatment is outlined in Fig. 4.6 [20]. Type A and complicated type B aortic dissections merit emergent repair.

40

Chapter 4.  Aortic Disease

Uncomplicated type B dissection means there is no visceral malperfusion, acute renal failure, periaortic hematoma, rupture, refractory pain or refractory hypertension, and these cases are typically managed medically, at least in the acute phase [21]. Patients that do not undergo surgical repair require continued heart rate and blood pressure control [22]. Imaging of the entire aorta at 1, 3, 6, 12 months and yearly thereafter is recommended whether there has been repair or not in order to monitor for adverse remodeling of the aorta [23]. If there was thoracic abdominal aneurysm, the first-degree relatives should be screened. They are advised to exercise, but avoid weight lifting that requires Valsalva and interval training. Long term care requires a discussion of the patient’s current symptoms and what new symptoms might prompt concern [21]. Clinical Vignette  This patient with acute and steady chest and back pain underwent CT angiography that demonstrated dissection in the aorta extending from the aortic root to the iliac arteries (Stanford A, DeBakey I). He was treated with esmolol for heart rate and blood pressure control followed by emergent ascending aorta and hemiarch repair and implantation of a mechanical aortic valve. He had no complications. Following surgery, additional evaluation revealed a tall stature (6’ 5”) along with a family history of sudden death of the patient’s father at age 40. He is currently undergoing genetic testing to investigate heritable causes of his aortic disease.

References 1. Mohler ER III, Gornik HL, Gerhard-Herman M, Misra S, Olin JW, Zierler RE.  ACCF/ACR/AIUM/ASE/ASN/ICAVL/ SCAI/SCCT/SIR/SVM/SVS 2012 Appropriate use criteria for peripheral vascular ultrasound and physiological testing part I: arterial ultrasound and physiological testing. J Am Coll Cardiol. 2012;60(3):242–76.

References

41

2. Goldfinger JZ, Halperin JL, Marin ML, Stewart AS, Eagle KA, Fuster V.  Thoracic aortic aneurysm and dissection. J Am Coll Cardiol. 2014;64(16):1725–39. 3. Hirsch AT, Haskal Z, Hertzer N, Bakal C, Creanger M, Halperin J, et  al. ACC/AHA 2005 practice guidelines for the management of patients with peripheral arterial disease (lower extremity, renal, mesenteric, and abdominal aortic). Circulation. 2005;113(11):1474–547. 4. Hirsch AT, Haskal ZJ, Hertzer NR, Bakal CW, Creager MA, Halperin JL, et  al. ACC/AHA 2005 practice guidelines for the management of patients with peripheral arterial disease (Lower extremity, renal, mesenteric, and abdominal aortic). Circulation. 2006;113(11):e463–654. 5. Chaikof EL, Brewster DC, Dalman RL, Makaroun MS, Illig KA, Sicard GA, et al. SVS practice guidelines for the care of patients with an abdominal aortic aneurysm: executive summary. J Vasc Surg. 2009;50(4):880–96. 6. Scott RAP, Ashton HA, Buxton MJ, Day NE, Kim LG, Marteau TM, et  al. The multicentre aneurysm screening study (MASS) into the effect of abdominal aortic aneurysm screening on mortality in men: a randomised controlled trial. Lancet. 2002;360(9345):1531–9. 7. Danzer D, Becquemin JP.  Abdominal aortic aneurysm. In: Vascular surgery: cases, questions and commentaries. 2018. 8. Elefteriades JA, Farkas EA. Thoracic aortic aneurysm. Clinically pertinent controversies and uncertainties. J Am Coll Cardiol. 2010;55(9):841–57. 9. Baxter BT, Terrin MC, Dalman RL. Medical management of small abdominal aortic aneurysms. Circulation. 2008;117(14):1883–9. 10. Brady AR, Thompson SG, Fowkes FGR, Greenhalgh RM, Powell JT. Abdominal aortic aneurysm expansion: risk factors and time intervals for surveillance. Circulation. 2004;110(1):16–21. 11. Lee WA. Predicting aneurysm enlargement in patients with persistent type II endoleaks. Yearb Vasc Surg. 2006;39(6):1157–62. 12. Connolly SJ, Eikelboom JW, Bosch J, Dagenais G, Dyal L, Lanas F, et  al. Rivaroxaban with or without aspirin in patients with stable coronary artery disease: an international, randomised, double-blind, placebo-controlled trial. Lancet. 2018;391:205–18. 13. Larsen M, Pape LA, Awais M, Bossone E, O’Gara P, Evangelista A, et  al. Presentation, diagnosis, and outcomes of acute aortic dissection. J Am Coll Cardiol. 2015;66(4):350–8.

42

Chapter 4.  Aortic Disease

14. Tsai TT, Nienaber CA, Eagle KA.  Acute aortic syndromes. Circulation. 2005;112(24):3802–13. 15. Lempel JK, Frazier AA, Jeudy J, Kligerman SJ, Schultz R, Ninalowo HA, et al. Aortic arch dissection: a controversy of classification. Radiology. 2014;271(3):848–55. 16. Klompas M. Does this patient have an acute thoracic aortic dissection? JAMA. 2002;287(17):2262–72. 17. Mészáros I, Mórocz J, Szlávi J, Schmidt J, Tornóci L, Nagy L, et al. Epidemiology and clinicopathology of aortic dissection. Chest. 2000;117:1271–8. 18. Karthikesalingam A, Holt PJE, Hinchliffe RJ, Thompson MM, Loftus IM. The diagnosis and management of aortic dissection. Vasc Endovasc Surg. 2010;44(3):165–9. 19. Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA. Acute aortic dissection and intramural hematoma a systematic review. JAMA. 2016;316(7):754–63. 20. Hughes GC, Andersen ND, RL MC. Management of acute type B aortic dissection. J Thorac Cardiovasc Surg. 2013;145:S202–7. 21. Fattori R, Cao P, De Rango P, Czerny M, Evangelista A, Nienaber C, et al. Interdisciplinary expert consensus document on management of type B aortic dissection. J Am Coll Cardiol. 2013;61(16):1661–78. 22. Nauta FJH, Trimarchi S, Kamman AV, Moll FL, Van Herwaarden JA, Patel HJ, et al. Update in the management of type B aortic dissection. Vasc Med (UK). 2016;21(3):251–63. 23. Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey  DE, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/ SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease. Circulation. 2010;121(13).

Chapter 5 Renal and Mesenteric Disease

Objective  Recognize impaired perfusion of the kidneys and mesentery. Vignette  A 91 year old gentleman with Parkinson’s disease presents with confusion and upper quadrant pain. Examination reveals mild right upper quadrant pain to palpation without peritoneal signs. Laboratory testing demonstrates a slight elevation in total bilirubin concentration. Anatomy Mesenteric vessels are the arteries and the veins of the abdomen. They are responsible for transporting blood to and from these organs (Fig. 5.1). One ‘vein’ that does not drain to the heart is the portal vein, as it delivers blood with nutrients and toxins to the liver. The portal vein is formed by the confluence of the mesenteric, gastric, splenic and cystic veins. It is responsible for the majority of blood flow into the liver. Renal Artery Stenosis Renal artery stenosis (RAS) may be diagnosed during the evaluation of hypertension or pulmonary edema, but it is more often an incidental finding. Interestingly, incidental identification of RAS predicts mortality [1]. Hypertension in © Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_5

43

44

Chapter 5.  Renal and Mesenteric Disease

Liver

From liver Stomach

Liver

Spleen

Kidney

Kidney Kidney

Kidney

Spleen

Colon

Gastrointestinal tract

Colon

Colon

Colon Gonad

Colon Gonad Colon

Aorta and branches

IVC and branches

Portal vein

Blood flow direction

Figure 5.1 Mesenteric vasculature. This schematic identifies the arterial, venous and portal venous blood flow and direction. The legend indicates color given to arterial, venous and portal venous anatomy. The arrow indicates flow direction toward end organ, toward the heart (inferior vena cava) or from organ to organ

Chapter 5.  Renal and Mesenteric Disease

45

an individual younger than 30 years old as well as pulmonary edema in individuals without heart disease are among the clinical presentations suggesting possible renal artery disease (Table  5.1). Atherosclerosis in the most common cause of RAS. In those under 30 years of age it is more likely another cause such as fibromuscular dysplasia [2]. The presence of microaneurysms at renal artery branch points suggests polyarteritis nodosa [3]. If there is hypertension but no RAS in a young person then etiologies like stimulant use, oral contraceptives and obesity are more likely to play a role [4, 5]. Diagnosis of RAS [1] depends on identifying an significant narrowing of the renal artery (Table 5.2). There are benefits and risks to each available imaging modality. Bowel gas and morbid obesity will obscure imaging with ultrasound. MRA is difficult if the individual has claustrophobia or metal implants within their body. CTA provides the best resolution, but is Table 5.1 Clinical presentation of RAS

Clinical presentations suspicious for RAS Hypertension onset under age 30 Acute kidney injury after ACE inhibition or ARB therapy Simultaneous increase in blood pressure and creatinine Pulmonary edema without heart disease Abdominal bruits

Table 5.2  Imaging in detection of RAS Result MRA Identify renal arteries +

CTA +

Ultrasound +

Hemodynamic assessment

–

–

+

Identify associated disease, e.g. aorta

+

+

–

Characterize renal mass

+

+

+

46

Chapter 5.  Renal and Mesenteric Disease

Figure 5.2 MRI of the abdomen. The red arrow indicates portal vein thrombosis in the portal vein entering the liver

less safe if the glomerular filtration rate is less than 30 ml/min [6]. Imaging may rarely detect renal vein thrombosis, prompting evaluation for hypercoagulable states (Fig. 5.2). In most cases, RAS is not the sole cause of hypertension, and many individuals will respond to standard antihypertensive pharmacotherapy. However, in select cases, such as those with refractory hypertension, acute kidney injury with initiation of antihypertensive therapy, and those with recurrent pulmonary edema, further intervention is warranted. Treatment of hypertension in the setting of renal artery stenosis is guided by the cause of the stenosis. Angioplasty of the stenosis is rarely enough unless the etiology of the stenosis is fibromuscular dysplasia [7]. Medical therapy is often directed at blocking the renin-angiotensin-aldosterone system. The diuretic chlorthalidone has been quite useful, and loop diuretics may be needed in advanced renal disease [4]. In cases of refractory hypertension and worsening renal function with pharmacotherapy, it is possible that renal artery angioplasty and stenting may be needed, although such a strategy has not been supported by clinical trial evidence [8, 9]. The role of antiplatelet therapy is unclear [10]. Surgical revascularization

Chapter 5.  Renal and Mesenteric Disease

47

with bypass graft or endarterectomy is rarely needed. Renal artery aneurysm may develop and should be evaluated for treatment at diameters greater than 2.5 cm [11]. Mesenteric Ischemia Acute mesenteric ischemia presents with severe pain out of proportion to physical examination. There may also be vomiting, diarrhea, rectal bleeding and leukocytosis [12]. Mortality can be high, particularly without prompt recognition and treatment [13]. Rapid imaging of the mesenteric vasculature is required for diagnosis, and this is typically accomplished with CTA due to its widespread availability [14, 15]. Non-occlusive mesenteric ischemia is possible, and imaging may demonstrate tubular narrowing of the vesselsrather than total vessel occlusion [16, 17]. Hypotension, such as from severe dehydration or shock, and diffuse vasoconstriction can also cause hypoperfusion leading to acute mesenteric ischemia. Survival is highest for those with focal arterial dissection, and they are also the individuals most likely to develop arterial aneurysms (Table 5.3). Mesenteric venous thrombosis can also cause acute mesenteric ischemia. Causes are either primary or secondary (Table  5.4). Mortality increases if abdominal pain worsens Table 5.3  Acute mesenteric ischemia Artery finding Causes Emboli Atrial fibrillation Atheroemboli Left ventricular thrombus Thrombosis

Hypercoagulable state Local inflammatory disease

Dissection

Aortic dissection Isolated mesenteric artery dissection Trauma

Normal artery

Low perfusion, e.g. shock Vasoconstriction

48

Chapter 5.  Renal and Mesenteric Disease

Table 5.4  Mesenteric vein thrombosis Thrombosis stimulus Diagnosis Local Portal hypertension Splenomegaly Trauma Surgery Inflammation

Diverticulitis Pancreatitis Infection

Hypercoagulability

Clotting protein deficiency Antiphospholipid syndrome Factor V Leiden Oral contraceptive

Increased viscosity

Myeloproliferative disease Sickle cell disease

over time. Conversely, collateral venous channels can develop over time and decrease ischemia [18]. Urgent anticoagulation and fluid resuscitation are indicated to limit end organ damage. Laparotomy is indicated if there are peritoneal signs or necrosis on imaging [19]. Endovascular procedures may be indicated such as transjugular intrahepatic portosystemic shunting (TIPS) [20]. Anticoagulation is typically continued indefinitely unless the cause of thrombosis is identified and removed. Chronic mesenteric ischemia is more rare [21] and results from atherosclerosis in 90% of cases. These individuals with ongoing cardiovascular risk factors typically have a high burden of polyvascular atherosclerosis [22]. Mesenteric artery atherosclerosis is evident long before there is symptomatic arterial stenosis. Symptoms begin with pain 20 min after eating and eventually can last up to 3–4  h. Patients develop food fear and avoid eating. Substantial weight loss often results. Endovascular treatment is preferred in this population with high morbidity [23]. Clinical Vignette  CT imaging identified both cho­ lecystitis  and  portal vein thrombosis. Magnetic resonance

References

49

cholangiopancreatography additionally demonstrated cho­ led­ ocholithiasis. Portal vein thrombosis was presumed secondary to inflammation in this region from his cholecystitis. He underwent gallstone removal accomplished by biliary sphincterotomy and balloon extraction. He was subsequently treated with therapeutic anticoagulation, with dosing appropriate to his age, weight and renal function.

References 1. Khangura KK, Eirin A, Kane GC, Misra S, Textor SC, Lerman A, et  al. Extrarenal atherosclerotic disease blunts renal recovery in patients with renovascular hypertension. J Hypertens. 2014;32(6):1300–6. 2. Khoury MH, Gornik HL. Fibromuscular dysplasia (FMD). Vasc Med (UK). 2017;22(3):248–52. 3. Bilateral renal infarct in otherwise healthy man. Am J Kidney Dis. 2014;63(5):A1–A121 4. Whelton PK, Carey RM, Aronow WS, Ovbiagele B, Casey DE, Smith SC, et  al. 2017 Guideline for the prevention, detection, evaluation, and management of high blood pressure in adults. A report of the American College of Cardiology/American Heart Association T. American College of Cardiology Foundation and the American Heart Association. 2017. 5. Bavishi C, Bangalore S, Messerli FH.  Outcomes of intensive blood pressure lowering in older hypertensive patients. J Am Coll Cardiol. 2017;69(5):486–93. 6. Sarkodieh JE, Walden SH, Low D. Imaging and management of atherosclerotic renal artery stenosis. Clin Radiol. 2013;68:627–35. 7. Trinquart L, Mounier-Vehier C, Sapoval M, Gagnon N, Plouin PF. Efficacy of revascularization for renal artery stenosis caused by fibromuscular dysplasia: a systematic review and meta-­ analysis. Hypertension. 2010;56:525–32. 8. Cooper CJ, Murphy TP, Cutlip DE, Jamerson K, Henrich W, Reid DM, et al. Stenting and medical therapy for atherosclerotic renal-artery stenosis. N Engl J Med. 2014;370:13–22. 9. Tendera M, Aboyans V, Bartelink M-L, Baumgartner I, Clément D, Collet J-P, et al. ESC Guidelines on the diagnosis and treatment of peripheral artery diseases. Eur Heart J [Internet].

50

Chapter 5.  Renal and Mesenteric Disease

2011;32(22):2851–906.. http://eurheartj.oxfordjournals.org/ content/32/22/2851 10. Aboyans V, Ricco JB, Bartelink MLEL, Björck M, Brodmann M, Cohnert T, et  al. 2017 ESC guidelines on the diagnosis and treatment of peripheral arterial diseases, in collaboration with the European Society for Vascular Surgery (ESVS). Eur Heart J. 2018;39(9):763–816. 11. Klausner JQ, Lawrence PF, Harlander-Locke MP, Coleman DM, Stanley JC, Fujimura N. The contemporary management of renal artery aneurysms. J Vasc Surg. 2015;61(4):978–84. 12. Wyers MC. Acute mesenteric ischemia: diagnostic approach and surgical treatment. Semin Vasc Surg. 2010;23:9–20. 13. Singh M, Long B, Koyfman A.  Mesenteric ischemia: a deadly miss. Emerg Med Clin N Am. 2017;35(4):879–88. 14. Martinez JP, Hogan GJ. Mesenteric ischemia. Emerg Med Clin N Am. 2004;22(4):909–28. 15. Carver TW, Vora RS, Taneja A. Mesenteric ischemia. Crit Care Clin. 2016;32(2):155–71. 16. Luckner G, Jochberger S, Mayr VD, Knotzer H, Pajk W, Wenzel V, et  al. Vasopressin as adjunct vasopressor for vasodilatory shock due to non-occlusive mesenteric ischemia. Anaesthesist. 2006;55(3):283–6. 17. Yoshida T, Kikuchi O, Tsuji Y, Okumura A, Matsueda K, Yamamoto H.  Non-occlusive mesenteric ischemia diagnosed by angiography at the beginning of continuous regional arterial infusion therapy is a strong predictive factor for mortality due to severe acute pancreatitis. Gastroenterology. 2012;142:S-851. 18. Harnik IG, Brandt LJ. Mesenteric venous thrombosis. Vasc Med. 2010;15(5):407–18. 19. Wu J, Li Z, Wang Z, Han X, Ji F, Zhang WW. Surgical and endovascular treatment of severe complications secondary to noncirrhotic portal hypertension: experience of 56 cases. Ann Vasc Surg. 2013;27(4):441–6. 20. Chawla YK, Bodh V. Portal vein thrombosis. J Clin Exp Hepatol. 2015;5:22–40. 21. Biolato M, Miele L, Gasbarrini G, Grieco A. Abdominal angina. Am J Med Sci. 2009;338(5):389–95. 22. Zeller T, Rastan A, Sixt S.  Chronic atherosclerotic mesenteric ischemia (CMI). Vasc Med. 2010;15(4):333–8. 23. Vos JA, de Vries JPPM, van Strijen MJL. Endovascular management of chronic mesenteric ischemia. In: endovascular interventions: a case-based approach. 2014.

Chapter 6 Vasospastic Disease

Objectives  Distinguish vasoreactivity from fixed arterial disease. Vignette  A 62 year old female presents to the outpatient office with episodes of cold, numb, white fingers in her left hand after her hands have been on the steering wheel for a short time [1]. A photograph is shown in Fig. 6.1. If she cannot take her hands off the steering wheel she notices that the fingertips turn dusky. When she stops driving, she has been able to warm her fingers until the color returns to normal. She has never had ulcers or tissue loss of the affected fingers. Epidemiology and Diagnosis The age of onset and features of the presentation are key in distinguishing primary from secondary Raynaud’s phenomenon [2]. Taking a clear history is essential in making the diagnosis and can often be supported by photographs provided by the patient of the episodic color change of concern. Episodic, symmetric digital vasospasm beginning in the teens and twenties is typical in primary Raynaud’s [3]. This is often familial and not associated with digital loss or ulcers. Affected individuals may describe stiffness, numbness, or even pain in the affected digits. In contrast, older individuals may present

© Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_6

51

52

Chapter 6.  Vasospastic Disease

Figure 6.1  Well demarcated pallor of the fingers. This photograph demonstrates well-demarcated pallor of the second and third digits. The shiny appearance is due to moisture barrier that has been applied to protect the skin

Chapter 6.  Vasospastic Disease

53

with digital vasospastic attacks due to an underlying condition (secondary Raynaud’s phenomenon). Unlike primary Raynaud’s phenomenon, secondary cases often cause asymmetric vasospasm affecting one or a small number of digits. Cracks in the affected digits or poorly healing ulcers are consistent with secondary Raynaud’s phenomenon. The list of secondary causes and conditions that result in Raynaud’s phenomenon is extensive and is divided into categories in Table 6.1. Digital arterial walls may be damaged by an underlying systemic disease, or increased vasoconstriction may happen with lower intravascular pressure or excessive stimulus for vasoconstriction leading to vasospasm [4]. Importantly, Raynaud’s can be evident decades before secondary causes like systemic sclerosis become clinically apparent. In addition, many of these secondary causes are relevant to other forms of episodic color change in the extremities. Episodic Color Change in the Extremities Other Than Raynaud’s Phenomenon Primary acrocyanosis is a benign cyanosis that extends proximally beyond the digits and has no distinct demarcation of color change [5]. It is often present with increased sympathetic drive and hyperhidrosis. The extremities may be cool, but there is no numbness, pain, or ulceration. Variation in the cyanosis relates to external temperature and sympathetic tone. Erythromelalgia is a vasoactive disorder with red, hot discoloration in the extremities. Burning pain is worse with warming and better with cooling [6]. Affected individuals often expose the affected area to extreme cold for prolonged periods in order to alleviate the extreme pain, which can ultimately lead to arterial damage akin to frostbite or even trench foot. Blanching of the red skin indicates primary rather than secondary erythromelalgia. Secondary forms are often associated with myeloproliferative disease, and a complete history and physical in addition to complete blood count should be part of the workup for erythromelalgia.

• Systemic sclerosis • Systemic lupus erythematosus • Mixed connective tissue disease • Sjogren’s syndrome • Rheumatoid arthritis • Dermatomyositis/ polymyositis • Primary biliary cirrhosis • Necrotizing vasculitis

• Anti-­migraine • Non-selective beta blocker • Cyclosporin • Bromocriptine • Interferon alpha and beta • Cocaine • Amphetamines (including ADHD medication) • Estrogen • Ephedrine • Cytotoxic

• Vibrating tools • Vinyl chloride exposure • Silica and solvents • Percussive injury including hypothenar hammer syndrome • Electric shock • Frostbite and cold injury • Hypothyroidism • Pheochromo­ cytoma • Paraneoplastic • Essential thrombocytosis • Cold agglutinin • Cryofibri­ nogenemia • Cryoglobulinemia • Low body mass (BMI  30 kg/m

2

+

perfusion studies and invasive angiography are rarely used for diagnosis. Diagnosis of PE in pregnancy is challenging. Pregnancy increases risk due to hypercoagulability, obstruction of venous return due to gravid uterus and increase in venous volume. The can result in compression of left leg venous return, thus causing left lower leg swelling and increasing DVT risk. At the same time lower extremity edema and dyspnea on exertion are common in pregnancy. CTPA radiation includes breast radiation. Nonetheless, this risk to mother and fetus is warranted if the clinical suspicion is high [4]. Risk assessment tools for PE include the revised Geneva score [5] (Table 9.2). Simplified Pulmonary Embolism Severity Index (sPESI) score allows assessment of long-term prognosis. The prognosis is worse with tachycardia, hypotension, hypoxemia, chronic heart and lung disease, cancer and age greater than 80 years. Right ventricular dysfunction also indicates worse

Chapter 9.  Venous Disease

81

Figure 9.1  Left deep vein thrombosis. Left leg on the individual in this photograph has proximal DVT with no DVT on the right. Photograph demonstrates mild edema, erythema and increased varicosities (arrows)

82

Chapter 9.  Venous Disease

Table 9.2 Revised Geneva score for PE (score ≥ 11 = high probability)

Factor Heart rate > 95 bpm

Points 2

Heart rate 75–94 bpm

1

Unilateral limb pain or edema

1

Prevous VTE

1

Active cancer

1

Hemoptysis

1

Surgery within 4 months

1

Age  100 kg 7.5 mg if 50–100 kg 5 mg if  60 kg 30 mg daily if 3 mm. Up to 25% of men and 15% of men develop varicose veins (Fig.  9.2). Family history, pregnancy, prolonged standing, hormone therapy and vascular malformations increase the risk of varicose veins and venous insufficiency [14].

Figure 9.2  Varicose veins. Photograph demonstrates varicose veins on the shin (arrow). These distensible tubes protrude and have a diameter >3 mm

86

Chapter 9.  Venous Disease

Medical treatment in all cases includes compression therapy, exercise, and weight control. Compression is typically achieved with gradient compression stocking using a 20–30  mm Hg gradient or extrinsic compressive wraps. A number of assessment tools are used to describe the clinical manifestations. The most simple of these is the Venous Clinical Severity Score (VCSS) [15]. This includes pain, varicose vein number, inflammation, edema, skin indurations, ulcers and impact of compression therapy. Another common tool is the Clinical-Etiology-Anatomy-Pathophysiology (CEAP) nomenclature (Table 9.4). Venous reflux refers to incompetency of the one-way valves presents in both superficial and deep veins. Reflux is common in the greater saphenous vein and results in distended veins and edema (Fig.  9.3). The diagnosis of venous reflux should be suspected based on physical exam and is confirmed using venous ultrasound documenting retrograde flow through incompetent valves using Doppler. In addition to compression, exercise, and weight loss, treatment may also require chemical ablation [16], thermal or radiofrequency ablation [17] and rarely surgical removal. Ablation is i­ ndicated for those individuals with CEAP category greater of 3 or greater (Table  9.4). CEAP class 4a is present in Fig.  9.4. Vascular malformations are focal abnormalities that may include multiple types of vessels and can be high or low flow. Both congenital abnormalities and vascular tumors occur [18] and are classified by the International Society for the Study of Vascular Abnormalities.

Chapter 9.  Venous Disease

87

Figure 9.3 Greater saphenous vein reflux. Arrows indicate the dilated, tortuous greater saphenous vein in this photograph of the left leg. Venous ultrasound confirmed reflux in this vessel

88

Chapter 9.  Venous Disease

Table 9.4  CEAP classification Clinical C0 no detectable venous disease C1 telangiectasia or reticular veins C2 varicose veins C3 edema C4a pigment deposition, eczema C4b lipodermatosclerosis, atrophy C5 healed ulcer C6 open ulcer Etiology congenital, primary, secondary, none Anatomy superficial, perforator, deep, no location Pathophysiology reflux, obstruction, reflux and obstruction, no identification

Figure 9.4  CEAP 4a findings. Photograph of the shin demonstrates hyperpigmentation and evidence of excoriation

References

89

Clinical Vignette  This gentleman with calf pain and family history of DVT underwent additional testing. Repeat ultrasound identified left gastrocnemius (calf) vein DVT. On further review, it was noted that the original ultrasound did not evaluate the calf vessels. Anticoagulation therapy was initiated.

References 1. Nanchal R, Kumar G, Taneja A, Patel J, Deshmukh A, Tarima S, et  al. Pulmonary embolism: the weekend effect. Chest. 2012;142:690–6. 2. Lim W, Le Gal G, Bates SM, Righini M, Haramati LB, Lang E, et  al. American Society of Hematology 2018 guidelines for management of venous thromboembolism: diagnosis of venous thromboembolism. Blood Adv. 2018;2:3226–56. 3. Robb CL, Bhalla S, Raptis CA.  Pitfalls in the diagnosis of acute pulmonary embolism on computed tomography: common pathologic and imaging mimics. Curr Radiol Rep. 2018;33:74–84. 4. Di Nisio M, van Es N, Büller HR.  Deep vein thrombosis and pulmonary embolism. The Lancet. 2016;388:3060–73. 5. Klok FA, Mos ICM, Nijkeuter M, Righini M, Perrier A, Le Gal G, et al. Simplification of the revised Geneva score for assessing clinical probability of pulmonary embolism. Arch Intern Med. 2008;168:2131–6. 6. Moorjani N, Price S. Massive pulmonary embolism. Cardiol Clin. 2013;31:503–18. 7. Wells PS, Tritschler T, Kraaijpoel N, Le Gal G.  Venous thromboembolism: advances in diagnosis and treatment. JAMA. 2018;320:1583–94. 8. Kearon C, Akl EA, Comerota AJ, Prandoni P, Bounameaux H, Goldhaber SZ, et al. Antithrombotic therapy and prevention of thrombosis, 9th ed: ACCP guidelines. Chest. 2012;141:e419S–96S. 9. Sharifi M, Bay C, Skrocki L, Lawson D, Mazdeh S. Role of IVC filters in endovenous therapy for deep venous thrombosis: the FILTER-PEVI (filter implantation to lower thromboembolic risk in percutaneous endovenous intervention) trial. Cardiovasc Intervent Radiol. 2012;35:1408–13.

90

Chapter 9.  Venous Disease

10. Singh P, Lai HM, Lerner RG, Chugh T, Aronow WS. Guidelines and the use of inferior vena cava filters: a review of an institutional experience. J Thromb Haemost. 2009;7:65–71. 11. Klok FA, Dzikowska-Diduch O, Kostrubiec M, Vliegen HW, Pruszczyk P, Hasenfuß G, et al. Derivation of a clinical prediction score for chronic thromboembolic pulmonary hypertension after acute pulmonary embolism. J Thromb Haemost. 2016;14:121–8. 12. Kim NH, Delcroix M, Jenkins DP, Channick R, Dartevelle P, Jansa P, et al. Chronic thromboembolic pulmonary hypertension. J Am Coll Cardiol. 2013;62:D92–9. 13. Noack F, Schmidt B, Amoury M, Stoevesandt D, Gielen S, Pflaumbaum B, et  al. Feasibility and safety of rehabilitation after venous thromboembolism. Vasc Health Risk Manag. 2015;11:397–401. 14. Gloviczki P, Gloviczki ML.  Guidelines for the management of varicose veins. Phlebology. 2012;27:2–9. 15. Tan MKH, Sutanto SA, Onida S, Davies AH.  The relationship between vein diameters, clinical severity, and quality of life: a systematic review. Eur J Vasc Endovasc Surg. 2019;57:851–7. 16. Blaise S, Bosson JL, Diamand JM.  Ultrasound-guided sclerotherapy of the great saphenous vein with 1% vs. 3% polidocanol foam: a multicentre double-blind randomised trial with 3-year follow-up. Eur J Vasc Endovasc Surg. 2010;39:779–86. 17. Rasmussen L, Lawaetz M, Serup J, Bjoern L, Vennits B, Blemings A, et al. Randomized clinical trial comparing endovenous laser ablation, radiofrequency ablation, foam sclerotherapy, and surgical stripping for great saphenous varicose veins with 3-year follow-up. J Vasc Surg Venous Lymphat Disord. 2013;1:349–56. 18. Nozaki T, Nosaka S, Miyazaki O, Makidono A, Yamamoto A, Niwa T, et  al. Syndromes associated with vascular tumors and malformations: a pictorial review. Radiographics. 2013;33:175–95.

Chapter 10 Lymphedema

Objective  To recognize and treat lymphedema. Vignette  A 41 year old female presents to the office with bilateral, symmetric lower extremity edema. This has increased over months to years, and she can no longer wear shoes comfortably. She has no pain in the affected area. Elevating her legs improves the swelling somewhat, but her legs never return to their normal size. End organ failure of the lymphatic vasculature results in accumulation of interstitial fluid and regional swelling [1]. Lymph stasis leads to adipose hypertrophy in the subcutis as well as local immune compromise. Lymphatic capillaries are blind - ended tubes that connect to form a network of vessels lined with endothelium and vascular smooth muscle cells. The lymphatic vessels drain into main channels in the thoracic duct [2]. The lymphatic system transports waste, immune cells and lipids from the intestinal lumen. Lymphatic fluid moves by intrinsic and extrinsic pumps responding to filling pressure and sympathetic nerve activity. Primary lymphedema is commonly classified as congenital lymphedema, lymphedema praecox, and lymphedema tarda. These disorders manifest at birth, before 35 years of age, or later in life, respectively. Primary lymphedema can be classified also by the number and appearance of lymphatic vessels.

© Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_10

91

92

Chapter 10.  Lymphedema

There are many hereditary forms, some characterized by dysmorphic features. Secondary lymphedema follows an injury to the lymphatic channels. Infections ranging from streptococcal lymphangitis to the nematode - born infection of filariasis can cause lymphatic damage. Injury may stem from surgical removal of lymph nodes, such as following mastectomy for breast cancer. Groin injury following multiple cardiac catheterizations for congenital heart disease can

Figure 10.1  Swelling extends to the forefoot and there is squaring of the toes in both feet

Chapter 10.  Lymphedema

93

Table 10.1  Medical therapy of lymphedema Decongestive lymphatic therapy Massage (manual lymphatic massage) Skin care (water soluble emollients) Bandaging (nonelastic compressive bandage) Exercise (activating the calf muscle pump; performed with compression in place) Compressive elastic garments Control of skin infection No benefit for diuretics

cause local trauma and lymphatic dysfunction. Cancer cells may migrate to the lymphatics and cause obstruction. Frequent subcutaneous injections or tourniquet application can result in lymphedema. Obesity leading to immobility and a large pannus causing pressure on proximal lymphatic channels is another important cause of lymphedema. Longstanding, untreated venous insufficiency may lead to subcutaneous fibrosis that damages lymphatic vessels and ultimately causes secondary lymphedema, often termed phlebolymphedema. The swelling of lymphedema may be pitting initially but over time becomes indurated with a woody texture and cobblestone appearance as subcutaneous scarring and fibrosis develop (Fig. 10.1). Foot or hand involvement is a hallmark of lymphedema and is an important clue to distinguish it from venous causes of edema. Lymphedema causes swelling of the dorsal foot or hand, often termed a buffalo hump. This ultimately creates typical squaring of the toes. Upsloping toenails are often common. Stemmer’s sign is the inability to pinch a fold of tissue at the base of the digits and is diagnostic of lymphedema [3]. There is no pain or pigment deposition. Lipedema is another subcutaneous disorder characterized by non-pitting edema and abnormal fat accumulation that spares the feet.

94

Chapter 10.  Lymphedema

The physical exam is often sufficient to make the diagnosis. Definitive diagnosis is made with lymphoscintigraphy, the injection of radiolabeled material into the dorsum of the feet. Delayed proximal progression and dermal back flow are signs of lymphedema [4]. This procedure can be extremely painful and is not necessary unless the diagnosis if unclear on exam. CT can show a honeycomb pattern in the subcutaneous tissue. MRI shows particular strength in differentiating lymphedema from lipedema. Medical therapy has multiple components (Table  10.1) and potentially includes pneumatic compression in addition to daily use of compression garments, massage, and exercise. Therapy must continue indefinitely to prevent regression. Improvement can be significant, although advanced fibrosis of the subcutantaneous tissue will limit the response. Lymphovenous anastomosis and other microsurgical techniques are being developed [5, 6]. Clinical Vignette  Venous ultrasound does not show DVT or venous insufficiency but does demonstrate lymphatic lakes. MR lymphangiography confirms the diagnosis of lymph­ edema. She responds to medical therapy eventually combined with use of the pneumatic pump.

References 1. Gianesini S, Obi A, Onida S, Baccellieri D, Bissacco D, Borsuk D, et al. Global guidelines trends and controversies in lower limb venous and lymphatic disease: narrative literature revision and experts’ opinions following the vWINter international meeting in Phlebology, Lymphology & Aesthetics, 23–25 January 2019. Phlebology. 2019;34:4–66. 2. Alitalo K.  The lymphatic vasculature in disease. Nat Med. 2011;17:1371–80. 3. Brenner E, Putz D, Moriggl B.  Stemmer’s (Kaposi-Stemmer-) sign – 30 Years later. Phlebologie. 2007;36:320–4.

References

95

4. Maclellan RA. The lymphatic system. In: Greene AK, Slavin SA, Brorson H, editors. Lymphedema: presentation, diagnosis, and treatment. Cham: Springer; 2015. 5. Lähteenvuo M, Honkonen K, Tervala T, Tammela T, Suominen E, Lähteenvuo J, et al. Growth factor therapy and autologous lymph node transfer in lymphedema. Circulation. 2011;123:613–20. 6. Campisi CC, Boccardo F, Piazza C, Campisi C.  Evolution of chylous fistula management after neck dissection. Curr Opin Otolaryngol Head Neck Surg. 2013;21:150–6.

Chapter 11 Vascular Compressive Syndromes

Objective  Recognize symptoms attributable to positional vascular compression. Vignette  An 18 year old water polo patient arrives in the emergency department with a swollen, bluish arm. He reports this occurred over the preceding 24 h. He had been playing in a championship and had no trauma. Bedside ultrasound of the arm shows no evidence of DVT. The subclavian artery, vein, or brachial plexus can be compressed in the thoracic outlet. These structures all pass through a canal created by the first rib, clavicle and strap muscles on their way to the arm (Fig.  11.1). The clinical presentation of thoracic outlet syndrome (TOS) is determined by the vessel being compressed. Venous TOS presents with arm swelling, typically in a younger athletic person [1]. Repeated compression of the vein causes endothelial injury, luminal fibrosis, and eventually thrombosis of the subclavian vein. Patients often experience sudden arm swelling and bluish discoloration. Vascular ultrasound may demonstrate subclavian thrombosis, but this can be technically challenging. Both CT and MR venography [2] are accurate in detecting subclavian vein narrowing as the patient extends the arms above the head. Subclavian vein thrombosis may also be identified. Catheterbased upper extremity venography allows definitive diagnosis, and catheter-delivered thrombolysis may be needed to © Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_11

97

98

Chapter 11.  Vascular Compressive Syndromes

Scalene muscles

Clavicle Sternum Subclavian artery Subclavian vein

First rib (under muscle) Second rib

Figure 11.1  Subclavian vein and artery in the thoracic outlet. The subclavian artery is seen in red and the subclavian vein in blue. The brachial plexus follows the same pathway as the subclavian artery. The subclavius muscle creates a narrow channel that adds to the compression of the subclavian vein

decrease clot burden and restore venous outflow. Thoracic outlet decompression, which typically involves surgical removal of the first rib, is often recommended 6 weeks following a good response to thrombolysis [3]. Arterial TOS is less common than venous TOS [4]. Arterial stenosis can develop at the site of repeated compression and may result in post stenotic aneurysm. The presence of fibrous bands extending from the C7 vertebral body spinous process to the first rib make arterial TOS more likely. Post stenotic aneurysm can become lined with thrombus that may embolize to the distal limb [5]. Patients can present with arm claudication, rest ischemia of the arm, or digital ischemia from distal emboli. CT and MR angiography at rest and with arm elevation can confirm the diagnosis. Surgical treatment often

Chapter 11.  Vascular Compressive Syndromes

99

involves bony resection [6]. Another cause of arterial compression in the region is axillary artery compression s­ yndrome, which typically happens when there is repeated overhead arm movement. An example of this movement is pitching a baseball. Both CT and MR angiography for diagnosis and surgery for treatment apply here as well. Median arcuate ligament syndrome is caused by compression of the celiac artery by the diaphragmic crura and the median arcuate ligament during expiration [7]. This diagnosis of exclusion presents with abdominal pain of many types [8]. There is occasionally epigastric tenderness and bruit on examination. CT and MR angiography or ultrasound can confirm the diagnosis by demonstrating celiac artery compression during expiration and resolution during inspiration [9]. Historicallyc treatment has been surgical decompression of the vessel [8]. Nutcracker syndrome [10] results from compression of the renal vein between the superior mesenteric artery (SMA) and the aorta (Fig. 11.2) with symptoms of left flank pain, hematuria, positional proteinuria, and pelvic and lower extremity varicose veins [11]. Low body weight, a retroaortic renal vein and acute angle between the aorta and SMA increase the likelihood of renal vein compression. Once the anatomy is seen the diagnosis requires identification of a gradient across the vein, which typically requires invasive

SMA Left renal vein Aorta

Figure 11.2 Nutcracker anatomy of left renal vein compression. Abdominal CT on the left and diagram on the right identify compression of the left renal vein between the SMA and the aorta prior to the renal vein junction with the inferior vena cava

100

Chapter 11.  Vascular Compressive Syndromes

A

Right iliac artery

Left Iliac vein

Figure 11.3  May-Thurner anatomy. CTA on the left and diagram on the right demonstrate the right common iliac artery crossing over the left common iliac vein. This is normal anatomy, with some compression evident in one third of the population. Additional soft tissue in the region varies among individuals and can contribute to increased compression. On the CT image, the left common iliac vein (arrow) is compressed between the right common iliac artery (arrowhead) and the vertebral body. The diagram shows the right iliac artery crossing over the left iliac vein

venography [12]. Treatment includes angiotensin converting enzyme inhibition. Both endovascular and surgical treatments have been proposed [13]. May-Thurner anatomy is compression of the left common iliac vein by the right common iliac artery (Fig.  11.3). May-­ Thurner syndrome is when this results in left lower extremity venous hypertension, edema, and/or left iliofemoral DVT [14]. Patients can also present with PE [15] or even stroke in the setting of patent foramen ovale following thromboembolism of the DVT [16]. The risk of DVT increases with the number of underlying risk factors for DVT in addition the degree of venous injury from iliac vein compression [17]. Treatment includes anticoagulation and potentially catheter-delivered thrombolysis. Endovenous stents are often placed following initial treatment [18].

Chapter 11.  Vascular Compressive Syndromes

101

Figure 11.4  Adventitial cyst. Duplex ultrasound on the left identifies the echo lucent cyst (asterisk) and the narrow popliteal artery lumen (lumen). The Doppler on the right shows elevated systolic velocity at the site of aliasing consistent with a significant arterial stenosis

There are three additional vascular compression syndromes in the lower extremities: the very rare popliteal artery entrapment, rare adventitial cyst (Fig. 11.4) and the more common popliteal artery aneurysm. Young active patients with popliteal entrapment typically present with claudication [19]. Examination is normal at rest. However, with plantar flexion, the distal pulses are diminished or abolished [20]. In this congenital anomaly the popliteal artery has an aberrant course with regard to the gastrocnemius or the popliteus muscle and becomes compressed with flexion of these muscles. The popliteal vein is rarely involved [21]. The diagnosis is made using CT or MR angiography of the popliteal artery at rest and with plantar flexion, and both imaging modalities allow excellent imaging of the surrounding soft tissue structures. Open surgical repair is used to alter the popliteal artery course [22]. Adventitial cysts are rare mucinous cysts [23] that occur in the adventitia of the popliteal artery or vein. It is less commonly described in other arteries [24]. The typical presentation is claudication in middle aged adults, often without typical risk factors of atherosclerotic peripheral artery disease. CT and MR angiography often show a scimitar sign at the cyst location, which is due to progressive stenosis and lateral

102

Chapter 11.  Vascular Compressive Syndromes

­isplacement of the artery [25]. Unlike ultrasound, these d modalities can clearly delineate the surrounding tissues. Multiple surgical techniques have been tried, with a nearly 20% reintervention rate at 2 months. Popliteal artery aneurysm can compress the popliteal vein with consequences of local venous hypertension and DVT [26]. Patients may be asymptomatic or may also present with digital ischemia due to thromboembolism from the aneurysm. Risk factors include age and tobacco use, and up to 19% of individuals with abdominal aortic aneurysms also have popliteal artery aneurysms [27]. Treatment is typically surgical to prevent progressive enlargement or rupture. Clinical Vignette  He underwent MR venography, which demonstrated subclavian vein thrombosis. He was treated with therapeutic anticoagulation and a compression sleeve of the affected arm. This was followed by invasive venography, pulse spray thrombectomy and thrombolysis. After 4 weeks more of anticoagulation, he underwent left supraclavicular first rib resection, subclavian venolysis, brachial plexus neuroplasty, subclavian arteriolysis, and anterior and middle scalenectomy for definitive decompression of the thoracic outlet.

References 1. Illig KA, Doyle AJ. A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg. 2010;51:1538–47. 2. Sridhar S, Bhalla S, Raptis CA, Fowler KJ, Thompson RW.  Imaging of the patient with thoracic outlet syndrome. RadioGraphics. 2016;36:984–100. 3. Sanders RJ, Hammond SL.  Venous thoracic outlet syndrome. Hand Clin. 2004;20:37–42. 4. Daniels B, Michaud L, Sease F, Cassas KJ, Gray BH.  Arterial thoracic outlet syndrome. Curr Sports Med Rep. 2014;13:75–80. 5. Gannon MX.  Thoracic outlet syndrome. In: Parikh D, Rajesh PB, editors. Tips and tricks in thoracic surgery. London: Springer; 2018.

References

103

6. Smith FC, Winterborn RJ.  Thoracic outlet syndrome. Surgery (UK). 2019;37:112–8. 7. Kim EN, Lamb K, Relles D, Moudgill N, DiMuzio PJ, Eisenberg JA.  Median arcuate ligament syndrome  – review of this rare disease. JAMA Surg. 2016;151:471–7. 8. Weber JM, Boules M, Fong K, Abraham B, Bena J, El-Hayek K, et  al. Median arcuate ligament syndrome is not a vascular disease. Ann Vasc Surg. 2016;30:22–7. 9. Mussa FF, Horton JD, Moridzadeh R, Nicholson J, Trimarchi S, Eagle KA. Acute aortic dissection and intramural hematoma a systematic review. JAMA. 2016;316:754–63. 10. Kurklinsky AK, Rooke TW.  Nutcracker phenomenon and nutcracker syndrome. Mayo Clin Proc. 2010;85:552–9. 11. Venkatachalam S, Bumpus K, Kapadia SR, Gray B, Lyden S, Shishehbor MH.  The nutcracker syndrome. Ann Vasc Surg. 2011;25:1154–64. 12. Jolley I. Nutcracker syndrome. Radiography. 2014;20:286–7. 13. Avgerinos ED, McEnaney R, Chaer RA. Surgical and endovascular interventions for nutcracker syndrome. Semin Vasc Surg. 2013;26:170–7. 14. Mousa AY, AbuRahma AF.  May  - Thurner syndrome: update and review. Ann Vasc Surg. 2013;27:984–95. 15. Streiff MB, Agnelli G, Connors JM, Crowther M, Eichinger S, Lopes R, et  al. Guidance for the treatment of deep vein thrombosis and pulmonary embolism. J Thromb Thrombolysis. 2016;41:32–67. 16. Doyen D, Castellani M, Moceri P, Chiche O, Lazdunski R, Bertora D, et  al. Patent foramen ovale and stroke in intermediate-­risk pulmonary embolism. Chest. 2014;146:967–73. 17. Carroll S, Moll S.  Inferior vena cava filters, May-Thurner syndrome, and vein stents. Circulation. 2016;133:e383–7. 18. Ibrahim W, Al Safran Z, Hasan H, Abu Zeid W.  Endovascular management of May-Thurner syndrome. Ann Vasc Dis. 2012;5:217–21. 19. Noorani A, Walsh SR, Cooper DG, Varty K.  Entrapment syndromes. Eur J Vasc Endovasc Surg. 2009;37:213–20. 20. Wright LB, Matchett WJ, Cruz CP, James CA, Culp WC, Eidt JF, et  al. Popliteal artery disease: diagnosis and treatment. Radiographics. 2004;24:467–79. 21. Hameed M, Coupland A, Davies AH.  Popliteal artery entrapment syndrome: an approach to diagnosis and management. Br J Sports Med. 2018;52:1073–4.

104

Chapter 11.  Vascular Compressive Syndromes

22. Lejay A, Delay C, Georg Y, Gaertner S, Ohana M, Thaveau F, et  al. Five year outcomes of surgical treatment for popliteal artery entrapment syndrome. Eur J Vasc Endovasc Surg. 2016;51:557–64. 23. Desy NM, Spinner RJ.  The etiology and management of cystic adventitial disease. J Vasc Surg. 2014;60:235–45. 24. Jarraya M, Simmons S, Farber A, Teytelboym O, Naggara N, Guermazi A. Uncommon diseases of the popliteal artery: a pictorial review. Insights Imaging. 2016;7:679–88. 25. Motaganahalli RL, Smeds MR, Harlander-Locke MP, Lawrence PF, Fujimura N, DeMartino RR, et al. A multi-institutional experience in adventitial cystic disease. J Vasc Surg. 2017;65:157–61. 26. Ascher E, Markevich N, Schutzer RW, Kallakuri S, Jacob T, Hingorani AP. Small popliteal artery aneurysms: are they clinically significant? J Vasc Surg. 2003;37:755–60. 27. Viktoria Tuveson, Hedvig E Löfdahl, Rebecka Hultgren. Patients with abdominal aortic aneurysm have a high prevalence of popliteal artery aneurysms. Vascular Medicine. 2016;21:369–75.

Chapter 12 Special Populations

Objective  Understand the unique vascular disease risk in adults with congential heart disease, individuals receiving cancer therapies and those with fibromuscular dysplasia. Vignette  A 59-year-old female who has had tetralogy of Fallot repair and a mechanical pulmonary valve presents with lower extremity edema and varicosities. Leg swelling is decreased when she wears thigh-high gradient compression stockings. Examination is notable for normal vital signs, diminished left arm pulses, lower extremity varicosities and brawny edema with CEAP class 4 manifestations. Adult Congenital Heart Disease Residual right heart disease in individuals with adult congenital heart disease can lead to systemic venous hypertension even with appropriate therapy to venous hypertension [1]. There can be pain in the legs when the individual is upright if the venous blood pressure is elevated. Patients may develop exertional leg pain in the calf despite the absence of atherosclerotic PAD, which is consistent with venous claudication. Chronic venous hypertension leads to impaired venous return and limb swelling and potentially venous ulcers. Those with a family history of varicose veins are more likely to develop significant superficial venous varicosities and © Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1_12

105

106

Chapter 12.  Special Populations

SPIDER VEINS

VARICOSE GREATER SAPHENOUS VEIN

VARICOSE VEIN RUPTIRE

Figure 12.1 Venous insufficiency. Photograph from an adult with congenital heart disease demonstrating superficial venous disease in the setting of venous hypertension. Arrows indicate spider veins, varicose greater saphenous vein and site of superficial venous rupture in the setting of venous hypertension

­insufficiency [2]. This is illustrated in Fig. 12.1. They are likely to develop CEAP3 or above venous insufficiency and merit venous ablation. Treatment is the same for these individuals with the caveat that the prior femoral access site is adequately evaluated. Patients with congenital heart disease undergo frequent cardiac catheterizations using femoral artery and vein access [3]. Vessel injury can heal with narrowing or stenosis, thus causing venous outflow obstruction. Femoral artery stenosis can result in claudication beginning in the calf. Venous stenosis can lead to unilateral limb swelling and increased risk of venous thrombosis as well as worse venous

Chapter 12.  Special Populations

107

insufficiency in the affected limb. Many patients with adult congenital heart disease also develop vascular symptoms in the upper extremities. Surgical repair of many congenital abnormalities can directly decrease subclavian artery flow due to either left subclavian flap aortoplasty for coarctation or Blalock-Taussig shunt transection of the subclavian artery for a subclavian - to pulmonary artery shunt [4]. This will result in a lower blood pressure on the intervened arm that the other arm, and often a minimal pulse detected at the wrist. The clinical ­complications are unilateral arm claudication, unilateral Raynaud’s phenomenon and subclavian steal. Vascular Toxicity with Cancer Therapy Diffuse vasospasm, Raynaud’s phenomenon, vasculitis, PAD, ischemic stroke and thromboembolism (arterial and venous) have all been observed in patients with cancer undergoing treatment [5]. Each of these etiologies is suspect when a patient undergoing therapy for cancer presents with a possible vascular problem. This can be thought of interms of the clinical presentation (Table  12.1). The literature has not always distinguished between episodic (e.g. Raynaud’s phenomenon) and non-episodic digital ischemia [6], but this framework is essential in determining the likely culprit as well as potential therapies. In the extremity, the initial clinical presentation may be non-episodic digital ischemia (Fig. 12.2). This can be due to an arterial embolism or the result of vasospasm throughout the limb. Diffuse vasospasm may have a larger role than has been suspected and should be considered when patients develop digital ischemia along with new onset systolic hypertension. It should also be suspected when imaging demonstrates diffuse narrowing of the arteries without clear abnormality of the arterial wall or lumen [7]. Diffuse vasospasm should be suspected in particular when all the digits are ischemic [8]. Vasculitis [9] can be detected with imaging of the arterial wall [10] and potentially biopsy in rare cases. Large vessel vasculitis has been seen with immune checkpoint inhibitor therapy [9]. Both PAD and cerebrovascular ischemia

108

Chapter 12.  Special Populations

Table 12.1  Vascular toxicities evaluated by clinical presentation Affected Cerebrovascular Coronary Peripheral Arteries Vascular (TIA/Stroke) Territory • Nilotinib • 5-FU Associated • Nilotinib • Placlitaxel • Ponatinib Cancer • Ponatinib • Cisplatin • Cisplatin Therapy • Tyrosine • 5-FU • VEGF kinase inhibitors • Anti-­metabolites inhibitors • Monoclonal • Monoclonal • Nilotinib antibodies • Ponatinib antiboides • Erlotinib Diagnostic Testing

• Carotid duplex • Head/neck CTA or MRA

Site-Specific Yes Medical Therapy

A

• I nvasive • Ultrasound angiography • CTA • Physiologic testing Yes

Yes

B

Figure 12.2 Toe photographs in patients on cancer therapy. (A) demonstrates blue toes. They are cool to examination. The color change persists despite warming, consistent with ischemia rather than vasospasm. (B) demonstrates the fixed, dark color change of gangrene at the tips of the toes as a consequence of inadequate arterial flow to the toes

Chapter 12.  Special Populations

109

may result from vasospasm, vasculitis or acceleration of atherosclerosis attributable to cancer therapy. TIA and stroke risk are not at increased risk in those undergoing cancer therapy [11]. However, there are presentations that merit discussion. A Moyamoya-like process, which manifests as intracranial artery stenosis, vascular fragility, and intracranial hemorrhage, has been seen with interferon alpha [12] and nilotinib. Treatment trials for acute ischemic stroke have not directly addressed patients receiving cancer therapy. Fibromuscular Dysplasia Fibromuscular dysplasia (FMD) can be observed in nearly every artery of the body. True prevalence and etiology are not known. It is classified according to lesion appearance; multifocal FMD demonstrates an angiographic appearance of “string of beads,” and unifocal FMD demonstrates a focal, smooth stenosis. The diagnosis is often incidental and due to isolated findings on imaging (Fig. 12.3). FMD also increases

Figure 12.3  CT angiogram of the common iliac arteries in a patient with FMD. The undulation of the string of beads is noted (arrows)

110

Chapter 12.  Special Populations

individuals’ risk of spontaneous coronary artery dissection (SCAD). Most patients have signs or symptoms referable to the organ supplied by the artery with FMD, e.g. brain, heart, kidney, extremity. Arterial dissection can result in major vascular events of stroke and myocardial infarction. Lesions over time can develop flow limiting stenoses and aneurysms. Unifocal FMD may be responsible for findings such as middle aortic syndrome, which causes significant narrowing of the distal aorta and lower extremity claudication. FMD must be distinguished from atherosclerosis (plaque will be evident) and vasculitis (constitutional symptoms and elevated acute phase reactants). In some individuals, the cause of arterial dissection may be a heritable connective tissue disorder such as vascular Ehlers-Danlos syndrome rather than FMD. Comprehensive initial imaging of arteries form the brain to the pelvis is recommended at the time of initial evaluation. This allows detection of previously undetected aneurysms. Follow up imaging should focus only on the affected areas identified in the aforementioned vascular scans. Medical and lifestyle therapy targeting arterial health are recommended. Revascularization is reserved for high risk lesions. This has been most studied in the renal arteries, where revascularization is reserved for refractory hypertension or renal function decline after angiotensin inhibition. Coiling should be evaluated for non-­ruptured cerebral aneurysm greater than 7 mm in diameter. Much of the evaluation will be periodic reimaging in this typically nonprogressive disease, with the focus on aneurysm detection and symptom control. Clinical Vignette  Venous ultrasound demonstrated no deep venous thrombosis. There was right greater than left reflux in the greater saphenous veins. No femoral vein stenosis was seen on magnetic resonance venography. She underwent successful endovenous saphenous vein ablation with continuous of anticoagulation for her mechanical pulmonic valve. Following the procedure, she was prescribed customsized, bilateral 20–30mmHg gradient compression stockings to be worn indefinitely except while sleeping.

References

111

References 1. Warnes CA.  Adult congenital heart disease. importance of the right ventricle. J Am Coll Cardiol. 2009;54:1903–10. 2. Valente AM, Bhatt AB, Cook S, Earing MG, Gersony DR, Aboulhosn J, et al. The CALF (Congenital heart disease in adults lower extremity systemic venous health in fontan patients) study. J Am Coll Cardiol. 2010;56(2):144–50. 3. Tsetis D.  Endovascular treatment of complications of femoral arterial access. Cardiovasc Intervent Radiol. 2010;33(3):457–68. 4. Warnes CA. Adult congenital heart disease: the challenges of a lifetime. Eur Heart J. 2017;38:2041–7. 5. Herrmann J, Yang EH, Iliescu CA, Cilingiroglu M, Charitakis K, Hakeem A, et al. Vascular toxicities of cancer therapies: the old and the new – an evolving avenue. Circulation. 2016;133:1272–89. 6. Vogelzang NJ, Bosl GJ, Johnson K, Kennedy BJ. Raynaud’s phenomenon: a common toxicity after combination chemotherapy for testicular cancer. Ann Intern Med. 1981;95:288–92. 7. Kim TD, Rea D, Schwarz M, Grille P, Nicolini FE, Rosti G, et al. Peripheral artery occlusive disease in chronic phase chronic myeloid leukemia patients treated with nilotinib or imatinib. Leukemia. 2013;27:1316–21. 8. Khaddour K, Singh V, Shayuk M. Acral vascular necrosis associated with immune-check point inhibitors: case report with literature review. BMC Cancer. 2019;19:449. 9. Boland P, Heath J, Sandigursky S. Immune checkpoint inhibitors and vasculitis. Curr Opin Rheumatol. 2020;32:53–6. 10. Ravi C, Gang M, Reviewer P, Steven M Winograd MD. The keys to identifying toxicity from checkpoint inhibitor therapy are knowing the patient has received such therapy and connecting the various symptoms and signs to one cause. The toxicity from checkpoint inhibitor therapy resembles autoimmune disorde with skin, et  al. Managing complications of new-age cancer therapy. Emerg Med Rep. 2019;40. 11. Kerber KA, Brown DL, Lisabeth LD, Smith MA, Morgenstern LB. Stroke among patients with dizziness, vertigo, and imbalance in the emergency department: a population-based study. Stroke. 2006;37:2484–7. 12. Buchbinder D, Steinberg G, Linetsky M, Casillas J.  Moyamoya in a child treated with interferon for recurrent osteosarcoma. J Pediatr Hematol Oncol. 2010;32:476–8.

Index

A Abdominal aortic aneurysm (AAA), 34 Ablation, 86 Abnormal nailfold capillaries, 56 Acute aortic syndromes, 37 Acute limb ischemia, 24, 74 Acute mesenteric ischemia, 47 Adult congenital heart disease, 105 Adventitial cysts, 101 Ankle brachial index (ABI), 1–4, 21–23, 28 Anticoagulation, 82–84 Aorta abdominal aortic aneurysm, 34 acute aortic dissection, 35 acute aortic syndromes, 37 anatomic site, 32 anatomy, 31 aneurysm, 33 aortic dissection, 36 embryologic origin, 32 imaging, 40 Arterial dissection, 110 Arterial TOS, 98 Artifacts, 9 Atherosclerosis, 45

B Bilateral limb swelling, 76 Blood testing, 10 C Carotid artery stenosis, 67 CEAP classification, 88 Cellulitis, 74 Cerebrovascular disease, 65 Chronic venous insufficiency, 75 Complex regional pain syndrome, 76 Computed tomography angiography (CTA), 8, 9 D Deep vein thrombosis (DVT) diagnosis of, 80 focal cramping pain, 79 risk scores for, 79 Diffuse vasospasm, 107 Distal internal carotid artery, 67 Doppler angle, 5 Doppler shift frequencies, 5 Duplex ultrasound, 12

© Springer Nature Switzerland AG 2020 M. Gerhard-Herman, A. Aday, Manual of Vascular Medicine, https://doi.org/10.1007/978-3-030-44715-1

113

114

Index

E Erythromelalgia, 53 Extremity, 107 F Femoral artery stenosis, 106 Fibromuscular dysplasia (FMD), 109 H High blood pressure, 43 High-risk asymptomatic ICA stenosis, 70 Hypertension, 46, 65 I Inferior vena cava (IVC) filter, 82 Invasive angiography, 80 Ischemic stroke, 63, 64 L Left deep vein thrombosis, 81 Leg pain calf discomfort, 17 causes and features of, 15 diagnoses of, 16 distribution of, 17, 18 location and clinical characteristics, 15 pain timing, 17 primary muscular abnormalities, 15 ruptured Baker’s cyst, 17 spinal stenosis and nerve root compression, 17 Lifestyle therapy, 57 Limb swelling bilateral limb swelling, 76 cellulitis, 74 clinical symptoms, 73 lymphedema, 75

patterns in, 74 ruptured Baker’s cyst, 74 venous obstruction and venous insufficiency, 73 Livedo, 55 Lymphedema, 75–77 diagnosis, 94 medical therapy of, 93 primary lymphedema, 91 secondary lymphedema, 92 Lymphovenous anastomosis, 94 M Magnetic resonance angiography (MRA) imaging, 7, 8, 23, 45, 66 May-Thurner anatomy, 100 Median arcuate ligament syndrome, 99 Mesenteric ischemia, 47 Mesenteric vein thrombosis, 48 N Nifedipine, 58 Nonatherosclerotic arterial disease, 10 Noninvasive vascular testing, 10 CT, 8 magnetic resonance, 7 physiologic testing, 1, 3 ultrasound, 6 Normal limb oximetry, 3 Nutcracker anatomy, 99 Nyquist limit, 5 P Paroxysmal digital hematoma, 55 Peripheral artery disease (PAD), 1 ankle brachial index measurement, 21, 22 clinical history, 21 exercise for, 26, 27

Index glucagon like peptide 1 agonists, 26 heart healthy diet, 24 MRA abdomen and pelvis, 23 regular exercise, 24 revascularization, 27, 28 smoking cessation, 24 sodium glucose cotransporter 2 inhibitors, 26 statin and PCSK9 inhibition, 26 treatment of, 24 Photoplethysmography, 2 Physiologic testing, 1, 3 Popliteal artery aneurysm, 102 Popliteal entrapment, 101 Primary acrocyanosis, 53 Primary muscular abnormalities, 15 Pulmonary embolism response teams (PERT), 82 Pulmonary Embolism Severity Index (PESI), 80 Pulse volume recording, 4 R Raynaud’s phenomenon chilblains (pernio), 55 diagnosis of, 55 drug therapy for, 59 epidemiology and diagnosis, 51 erythromelalgia, 53 lifestyle therapy, 57 livedo, 55 medication and surgical therapy, 58 paroxysmal digital hematoma, 55 physical examination, 55 primary acrocyanosis, 53 secondary conditions and causes of, 54 Reflux, 86 Renal artery stenosis (RAS)

115

atherosclerosis, 45 clinical presentation of, 45 diagnoses of, 43 high blood pressure, 43 hypertension treatment, 46 imaging in detection, 45 surgical revascularization, 46 Revascularization, 27, 110 S Segmental Doppler pressure, 4 Sodium glucose cotransporter 2(SGLT2) inhibitors, 26 Spectral waveform, 6 Stemmer’s sign, 93 Stroke carotid dissection, 64 hypertension, 65 imaging, 65 impact of cigarette smoking, 65 ischemic strokes, 63 neurologic deficits, 64 transient ischemic attack, 63 treatable risk for, 66 Subclavian vein and artery, 98 T Thoracic aortic aneurysm, 33 Thoracic outlet syndrome (TOS), 97 Transient ischemic attack, 63 U Ultrasound, 4–6 V Varicose veins, 85 Vascular compression arterial TOS, 98 May-Thurner syndrome, 100

116

Index

median arcuate ligament syndrome, 99 thoracic outlet syndrome, 97 venous TOS, 97 Vascular diagnosis, role of imaging, 11 Vascular toxicities, 108 Vasculitis, 107 Venous Clinical Severity Score (VCSS), 86

Venous hypertension, 105 Venous insufficiency, 106 Venous stenosis, 106 Venous thromboembolism (VTE) anticoagulation, 82 long term treatment, 85 risk factors, 79, 80 Venous thoracic outlet syndrome (TOS), 97