Table Of Content
ATLAS OF
SECOND EDITION
ATLAS
ISEASE
SECOND EDITION
EDITOR
MARK A. CREAGER, MD
Associate Professor
Department of Medicine
Harvard Medical School
Cardiovascular Division
Simon C. Fireman Scholar in Cardiovascular Medicine
Director, Vascular Center
Brigham and Women5 Hospital
Boston, Massachusetts
SERIES EDITOR
EUGENE BRAUNWALD
MD, MD (HON), SeD (HON)
Distinguished Hersey Professor of Medicine
Faculty Dean for Academic Programs at Brigham and Women's
Hospital and Massachusetts General Hospital
Harvard Medical School
Vice President for Academic Programs
Partners HealthCare System
Boston, Massachusetts
With 19 contributors
eM
CURRENT.
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Library of Congress Cataloging-in-Publication Data
Atlas of vascular disease I editor, Mark A. Creager.-- 2nd ed.
p.; cm.
Rev. ed. of: Vascular disease I volume editor, Mark A. Creager. c1996.
Includes bibliographical references and index.
ISBN 978-1-4757-4566-5 ISBN 978-1-4757-4564-1 (eBook)
DOI 10.1007/978-1-4757-4564-1
1. Blood-vessels--Diseases--Atlases. I. Title: Vascular disease. II. Creager, Mark A.
we
[DNLM: 1. Vascular Diseases--Atlases. 17 A884683 2002]
RC682 .A825 2002
61 6.1 '3--dc21
2002067395
ISBN 978-1-4757-4566-5
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Originally published by Current Medicine, Inc in 2003
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IV
PREFACE
Vascular diseases constitute the most common causes of This second edition of the Atlas of Vascular Disease reviews a
disability and death in Western society. Atherosclerosis, throm number of important vascular disorders that are encountered in
bosis, hypertension, and their clinical sequelae, including clinical practice, including aortic aneurysms and dissection,
myocardial infarction, disabling claudication, limb loss, and peripheral arterial occlusive disease, renovascular hypertension,
stroke, occur far too frequently. Indeed, vascular pathology in cerebrovascular disease, vasospasm, venous thromboembolism,
one form or another is responsible for over one hundred billion venous insufficiency, and lymphedema. For each major vascular
dollars of costs annually in the United States for medical condition, the pathophysiology, clinical presentation, associated
expenses and lost productivity. conditions (eg, atherosclerosis, hypertension, and vasculitis),
There have been rapid advancements in vascular biology, contemporary diagnostic testing (eg, vascular ultrasonography,
pharmacology, and technology for vascular disease. Basic magnetic resonance imaging, and angiography), and therapeutic
science discoveries have led to our recognition of the human strategies are discussed. One of the most perplexing issues
vasculature as not simply a passive conduit, but as a complex to face the clinician is how to manage the patient undergoing
organ that responds to both intrinsic and extrinsic stimuli with vascular surgery, since many of these patients have coexisting
definable pathologic processes. In addition, technologic coronary artery disease. Therefore, we have devoted a chapter
advances have redefined the role of imaging modalities. The to preoperative risk assessment, decision analysis, and
enhanced resolution of techniques such as ultrasonography, medical management.
computed tomography, and magnetic resonance angiography The chapter on vascular neoplasms logically categorizes and
has enabled the clinician to accurately diagnose vascular beautifully illustrates the pathology of these blood vessel
diseases noninvasively, thereby avoiding the risks inherent to tumors. We have also dedicated a chapter to Kawasaki disease,
invasive procedures. As diagnostic modalities have become less an important infectious disease that affects blood vessels,
invasive, therapeutic options have moved from the purely particularly in children, and one that is of growing interest
surgical arena. Innovations in endovascular intervention have to clinicians.
provided less invasive therapeutic strategies for patients Our understanding of vascular biology is increasing rapidly and
affected by clinically significant atherosclerosis of peripheral our ability to favorably influence outcome in patients with vascular
and renal arteries. Endovascular placement of stented grafts is disease has never been greater. This atlas is designed to provide the
now employed for selected patients with aortic aneurysms. physician with a practical and informative framework, illustrating
Advances in surgical techniques coupled with perioperative the clinical options in a logical and algorithmic fashion. In addition,
cardiac risk assessment and management have yielded lower it will serve as a useful teaching aid for those who educate medical
operative mortality and morbidity rates and consequently students, house staff, fellows, and colleagues. It is hoped that this
improved the outcome for patients with arterial aneurysms, atlas will enable the readers to deal effectively with a broad range
ischemic extremities, and cerebrovascular disease. of vascular diseases that are encountered in practice.
MARK A. CREAGER, MD
v
CONTRIBUTORS
ANDREW W. BRADBURY, BSC, JONATHAN L. HALPERIN, MD JEFFREY W. OLIN, DO
MBCHB (HCUS), MD, FRCSED Professor Director, Vascular Medicine Program
Professor Department of Cardiology The Zena and Michael A. Wiener
Department of Surgery Mount Sinai School of Medicine Cardiovascular Institute
University of Birmingham Director, Cardiology Clinical Service Mount Sinai School of Medicine
Birmingham Heartlands Hospital The Zena and Michael A. Wiener New York, New York
Birmingham, United Kingdom Cardiovascular Institute
Mount Sinai Medical Center JOSEPH F. POLAK, MD, MPH
MARK A. CREAGER, MD New York, New York Associate Professor
Associate Professor Department of Radiology
Department of Medicine MARIE GERHARD-HERMAN, MD Harvard Medical School
Harvard Medical School Assistant Professor Director of Noninvasive Vascular Imaging
Cardiovascular Division Department of Medicine Brigham and Women's Hospital
Simon C. Fireman Scholar in Cardiovascular Division Boston, Massachusetts
Cardiovascular Medicine Medical Director, Vascular Diagnostic Laboratory
Director, Vascular Center Brigham and Women's Hospital KHETHER E. RABY, MD
Brigham and Women's Hospital Boston, Massachusetts Assistant Clinical Professor
Boston, Massachusetts Department of Medicine
ALAN T. HIRSCH, MD Boston University School of Medicine
MAGRUDER C. DONALDSON, MD Associate Professor Boston, Massachusetts
Associate Professor Departments of Medicine and Radiology
Department of Surgery Director, Vascular Medicine Program STANLEY ROCKSON, MD
Harvard Medical School University of Minnesota Medical School Associate Professor
Brigham and Women's Hospital Minneapolis, Minnesota Division of Cardiovascular Medicine
Boston, Massachusetts Stanford University School of Medicine
ERIC M. ISSELBACHER, MD Chief, Consultative Cardiology
SAMUEL Z. GOLDHABER, MD Assistant Professor Stanford University Medical Center
Associate Professor Department of Medicine Stanford, California
Department of Medicine Harvard Medical School
Harvard Medical School Medical Director, Thoracic Aortic Center C.VAUGHAN RUCKLEY, MB,
Staff Cardiologist Massachusetts General Hospital CHM, FRCSE, FRCPE
Director, Venous Thromboembolism Boston, Massachusetts Professor Emeritus
Research Group Department of Vascular Surgery
Director, Anticoagulation Service FRANCES E. JENSEN, MD The University of Edinburgh
Brigham and Women's Hospital Director, Cardiovascular Division Edinburgh Royal Infirmary
Boston, Massachusetts Brigham and Women's Hospital Edinburgh, Scotland
Boston, Massachusetts
MARDI GOMBERG-MAiTlAND, MD, MSC KATHRYN A. TAUBERT, PHD
Mount Sinai School of Medicine ALEXANDER J. F. LAZAR, MD, PHD Professor
New York, New York Clinical Fellow Department of Physiology
Department of Pathology University of Texas Southwestern Medical School
SCOTT R. GRANTER, MD Harvard Medical School Vice President, Science and Medicine
Associate Professor Brigham and Women's Hospital American Heart Association
Department of Pathology Boston, Massachusetts National Center
Harvard Medical School Dallas, Texas
Associate Pathologist
JANE W. NEWBURGER, MD, MPH
Brigham and Women's Hospital
Professor
Boston, Massachusetts
Department of Pediatrics
Harvard Medical School
Associate Cardiologist-in-Chief
Children's Hospital of Boston
Boston, Massachusetts
VI
CONTENTS
CHAPTER 1 CHAPTER 3
ARTERIAL OCCLUSIVE DISEASES
AORTIC AND ARTERIAL ANEURYSMS
OF THE EXTREMITIES
Mardi Gomberg-Maitland, Jonathan L. Halperin,
and Mark A. Creager Alan T. Hirsch
The Normal Aorta .............................. .3 Epidemiology of Peripheral Arterial Disease ...... .48
Classification of Aortic Aneurysms ............... .3 Lower Extremity Arterial Anatomy, Limb
Cystic Medial Necrosis and the Blood Flow, and Symptoms .................. .50
Marfan Syndrome ........................... .4 Natural History and Prognosis .................. .51
Miscellaneous Causes of Aortic Clinical Presentation ........................... .53
Aneurysmal Disease ..........................6 Diagnostic Evaluation ......................... .54
Aortic Imaging Methods .........................7 Atherosclerotic Lower Extremity Peripheral
Thoraco-abdominal Aortic Aneurysms .............9 Arterial Disease ............................ .59
Abdominal Aortic Aneurysms ...................1 0 Thromboangiitis Obliterans' and
Peripheral Arterial Aneurysms ...... : ............2 0 Fibromuscular Dysplasia .....................6 9
Fibromuscular Dysplasia ........................7 1
CHAPTER 2 Popliteal Entrapment Syndrome ................ .72
Acute Arterial Occlusion ........................7 3
AORTIC DISSECTION
Atheroembolism ...............................7 6
Eric M. Isselbacher
CHAPTER 4
Pathogenesis ..................................2 6
Classification ..................................2 7
RENAL ARTERY DISEASE
Clinical Manifestations ..........................2 9
w.
Jeffrey Olin
Diagnostic Techniques ......................... .32
Selecting an Imaging Modality .................. .40 Etiology of Renovascular Disease .................8 2
Early Medical and Operative Therapy ........... .41 Prevalence of Renal Artery Disease ...............8 6
Other Acute Aortic Syndromes .................. .44 Clinical Manifestations and Diagnosis of
Survival and Late Follow-Up ................... .45 Renal Artery Disease ........................9 0
Natural History of Renovascular Disease ..........9 9
Management of Renovascular Disease ...........1 01
CHAPTER 5
MESENTERIC VASCULAR DISEASE
Magruder C. Donaldson
Mesenteric Vascular Anatomy ...................1 12
Regulation of Mesenteric Blood Flow ............1 14
Acute Mesenteric Ischemia .....................1 16
Chronic Mesenteric Ischemia ...................1 22
VII
CHAPTER 6 CHAPTER 10
CEREBROVASCULAR DISEASE DEEP VEIN THROMBOSIS, PULMONARY
Frances E. Jensen and Mark A. Creager EMBOLISM, AND PRIMARY
PULMONARY HYPERTENSION
Clinical Presentation ...........................1 30
Diagnostic Tests ...............................1 31 Samuel Z. Goldhaber and Joseph E Polak
Treatment of Cerebral Atherothrombosis .........1 34
Anatomy and Pathophysiology .................2 06
Vertebrobasilar Disease ........................1 42
Epidemiology ............................... .207
Vasculitis .....................................1 45
Diagnostic Tests .............................. .208
Cerebral Embolism ............................1 46
Upper Extremity Venous Thrombosis ............2 12
Treatment of Deep Vein Thrombosis .............2 13
CHAPTER 7 Pulmonary Embolism ..........................2 15
EVALUATION AND MEDICAL MANAGEMENT Management of Pulmonary Embolism ...........2 18
Thrombolysis Versus Embolectomy ..............2 19
OF THE VASCULAR SURGERY PATIENT
Primary Pulmonary Hypertension ...............2 24
Khether E. Raby
Cardiovascular Mortality in Patients CHAPTER 11
with Vascular Disease .......................1 52
CHRONIC VENOUS INSUFFICIENCY
Risk Assessment Derived from Clinical Features ...1 55
Andrew W. Bradbury and C. Vaughan Ruckley
Exercise Testing ...............................1 56
Dipyridamole-Thallium-201 /99mTc-Sestamibi Classification ................................ .230
SPECT Imaging ............................1 57 Pathophysiology ..............................2 31
Echocardiography .............................1 60 Clinical Presentation ...........................2 32
Holter (Ambulatory) ECG Monitoring ...........1 62 Assessment ...................................2 33
Effect of Medical Management on Cardiac Risk ...1 66 Treatment ....................................2 35
Effect of Coronary Artery Bypass Surgery
on Cardiac Risk ............................1 68
CHAPTER 12
Decision Analysis .............................1 69
LYMPHATIC DISEASE
CHAPTER 8 Stanley Rockson
RAYNAUD'S PHENOMENON AND OTHER Anatomy and Physiology of the
Lymphatic Circulation ......................2 42
VASOSPASTIC DISORDERS
Lymphedema Classification ....................2 44
Marie Gerhard-Herman and Mark A. Creager
Physical Findings and Diagnosis ................2 46
Raynaud's Phenomenon .......................1 74 Treatment of Lymphedema .................... .250
Other Temperature-related Disorders ............1 85
CHAPTER 13
CHAPTER 9
NEOPLASTIC AND NON-NEOPLASTIC
KAWASAKI DISEASE VASCULAR TUMORS
Kathryn A. Taubert and Jane W. Newburger Alexander J. E Lazar and Scott R. Granter
Epidemiology ................................1 90 Benign Vascular Tumors .......................2 56
Pathology ....................................1 91 Intermediate or Borderline Vascular Tumors ......2 63
Diagnosis and Clinical Features .................1 92 Malignant Vascular Tumors .....................2 66
Laboratory Findings ...........................2 00
Therapy and Long-term Follow-up ..............2 01
INDEX ......................................2 69
VIII
AORTIC AND ARTERIAL
ANEURYSMS
CHAPTE R
Mardi Gomberg-Maitland, Jonathan L. Halperin,
and Mark A. Creager
The aorta is the body's major conductance vessel, through which all oxygenated blood
passes. As an elastic artery, the aortic wall consists of three layers: intima, media, and adven
titia. The arteries arising along its course give rise to the vasa vasorum, which supply a capil
lary network to the adventitia and media of the thoracic aorta; however, vasa vasorum do not
supply the media of the abdominal aorta. Systolic ejection of blood from the left ventricle
creates a pressure wave that traverses the aorta producing radial expansion and contraction
and transfer of energy to the aortic wall. During diastole, the aortic wall recoils, transforming
potential to kinetic energy and driving blood into the peripheral vessels.
The term aneurysm originates from the Greek aneurysma, for dilation. The pathologic
concept is distinct from ectasia, the modest generalized arterial dilation that normally
accompanies aging. Aneurysms may be classified anatomically or etiologically; true
aneurysms may be either fusiform or saccular. The limited orifice of a saccular aneurysm
may protect the thin wall from aortic pressure, thereby reducing the risk of rupture, while
the entire circumference of a fusiform aneurysm is exposed to distending forces. Infrarenal
aortic aneurysms, the most common type, are almost exclusively fusiform. Aneurysms are
classified according to the segment of aorta affected, since clinical features depend largely
on location. Aneurysms of more peripheral arteries carry a lower risk of rupture than those
of the aorta, but a greater propensity to thromboembolic complications. A pseudoaneurysm,
or false aneurysm, is essentially a contained arterial rupture, in which the wall of the
aneurysm is composed of thrombotic material; the relatively narrow communication
between the sac and main lumen may resemble a saccular true aneurysm.
Dilatation of the aorta may occur as a consequence of aging, as well as of atherosclerosis,
infection, inflammation, trauma, congenital anomalies, and medial degeneration. The patho
logic changes that accompany these conditions cause the aorta to thicken, thin, bulge, tear,
rupture, stenose or dissect, or be altered by combinations of these conditions. Atherosclerotic
lesions occur more extensively in the abdominal aorta than in any part of the arterial tree
and initially affect only the intima, but fibrocaIcific degeneration leads to secondary atrophy
of the medial layer. The ascending aorta is generally spared by atherosclerosis until the most
advanced stages, and aneurysmal disease of the ascending aorta is more often nonathero-
sclerotic. Cystic medial necrosis, with elastic fiber degen surgical and nonsurgical management of patients with
eration, necrosis of muscle cells, and cystic spaces filled abdominal aortic aneurysms of 4.0 to 5.5 cm. There is no
with mucoid material, is most frequent in the ascending survival advantage of early elective repair of small
aorta but may be seen in the remainder of the aorta as aneurysms compared with careful surveillance when
well, particularly in Marfan syndrome. surgical repair is deferred until aneurysm size exceeds 5.5
In addition to the etiology and location of aortic cm or expands at a rate of 1 cm or more per year.
aneurysms, the risk of rupture of aortic aneurysms corre The 5-year survival rate in patients with untreated
lates with diameter, accounting for at least 30% of deaths. aneurysms larger than 5 cm in diameter ranges between
Aneurysms typically produce no symptoms prior to 5% and 10%, while the survival rate for such patients
rupture, but a variety of symptom complexes may arise after surg'ery is over 50%. After recovery from surgical
depending on size and location. Diagnosis of an abdom repair, long-term survival is limited principally by
inal aortic aneurysm may be suggested by prominent concomitant coronary or cerebrovascular disease [9]. It is
widening of the epigastric pulsation. Epigastric bruit is a hoped that the introduction of intraluminal stent graft
nonspecific finding, and other findings on physical repair will reduce the operative mortality of aortic
examination reflect co-existing atherosclerosis. Plain aneurysm correction, but the relative durability of this
roentgenograms may reveal calcification in the opposing approach compared with conventional open aneurysm
walls, and ultrasonography helps confirm its size and orrhaphy must be established.
demonstrate thrombus formation on the intimal surface Evaluation and management of patients with
extending into the lumen. Computed tomography accu aneurysmal disease of the aorta and peripheral arteries
rately outlines the configuration, especially when requires appreciation of the various factors that bear
enhanced by the intravenous injection of contrast mate upon prognosis and careful balancing of the complexity
rial. Magnetic resonance imaging is a sensitive tool for of surgical intervention against alternative measures to
early detection of dissection. Contrast aortography is reduce the risk of rupture. Control of hypertension,
reserved for preoperative definition of the relationship of medications that reduce the rate of rise of arterial pres
the aneurysm to adjacent vascular structures in patients sure during systole, and general steps to prevent throm
with concomitant arterial occlusive disease. boembolic complications and retard progression of
The surgical technique for repair of aortic aneurysms atherosclerotic disease seem of diminishing value as the
has evolved over the past 50 years since the first resec expansion of an aneurysm threatens cardiovascular
tion of an abdominal aortic aneurysm was reported in catastrophe. A successful outcome depends upon close
1952 [1]. Today, most surgeons use a modification of the serial evaluation by means of appropriate noninvasive
method advanced by Crawford and Coselli. Following imaging methodology and timely, judicious intervention.
dissection just beyond the proximal and distal margins of Evaluation and management of patients with
the aneurysm, heparin is administered and the aorta is aneurysmal disease of the aorta and peripheral arteries
cross-clamped. The aorta is incised, and a woven Dacron require an appreciation of the factors that influence prog
(Dupont, Wilmington, DE) graft is inserted within the nosis and careful balancing of the risk of surgical inter
aneurysmal sac and anastomosed to the less diseased vention against the limited nonoperative measures avail
portions. After the anastomoses are complete, the clamps able to reduce the chance of rupture. Control of
are released and the wall of the aneurysm is then hypertension, medications that reduce aneurysmal wall
wrapped around the outside of the prosthetic graft. stress, and measures to prevent thromboembolic condi
Operative repair of thoraco-abdominal aneurysms still tions and retard progression of atherosclerotic disease
carries a high mortality rate; major complications include diminish in value as the expansion of an aneurysm
paraplegia, renal insufficiency, and myocardial infarction. threatens cardiovascular catastrophe. A successful
The outlook for repair of abdominal aortic aneurysms is outcome depends on close serial evaluation by means of
considerably better, with in-hospital mortality rates around appropriate noninvasive imaging methodology and
1 %. There have been several controlled trials comparing timely, judicious intervention.
ATLAS OF VASCULAR DISEASE
2
