Handbook of Pathology and Pathophysiology of Cardiovascular Disease PDF
Preview Handbook of Pathology and Pathophysiology of Cardiovascular Disease
HANDBOOK OF PATHOLOGY AND PATHOPHYSIOLOGY OF CARDIOVASCULAR DISEASE Developments in Cardiovascular Medicine 214. P. A. Doevendans, R.S. Reneman and M. Van Bilsen (eds): Cardiovascular Specific Gene Expression. 1999 ISBN:0-7923-5633-0 215. G. Pons-Lladó, F. Carreras, X. Borrás, Subirana and L.J. Jiménez-Borreguero (eds.): Atlas of Practical Cardiac Applications of MRI. 1999 ISBN: 0-7923-5636-5 216. L.W. Klein, J.E. Calvin, Resource Utilization in Cardiac Disease. 1999. ISBN:0-7923-8509-8 217. R. Gorlin, G. Dangas, P. K. Toutouzas, M.M Konstadoulakis, Contemporary Concepts in Cardiology, Pathophysiology and Clinical Management. 1999 ISBN:0-7923-8514-4 218. S. Gupta, J. Camm (eds.): Chronic Infection, Chlamydia and Coronary Heart Disease. 1999. ISBN:0-7923-5797-3 219. M. Rajskina: Ventricular Fibrillation in Sudden Coronary Death. 1999. ISBN:0-7923-8570-5 220. Z. Abedin, R. Conner: Interpretation ofCardiacArrhythmias: SelfAssessment Approach. 1999. ISBN:0-7923-8576-4 221. J. E. Lock, J.F. Keane, S. B. Perry: Diagnostic and Interventional Catheterization In Congenital Heart Disease. 2000. ISBN:0-7923-8597-7 222. J.S. Steinberg: Atrial Fibrillation after Cardiac Surgery. 2000. ISBN:0-7923-8655-8 223. E.E. van der Wall, A. van der Laarse, B.M. Pluim, A.V.G. Bruschke: Left Ventricular Hypertrophy: Physiology versus Pathology. 2000 ISBN:0-7923-6038-9 224. J.F. Keaney, Jr. (ed.): Oxidative Stress and Vascular Disease. 2000. ISBN: 0-7923-8678-7 228. B.E. Jaski: Basicsof HeartFailure. 2000 ISBN:0-7923-7786-9 229. H.H. Osterhues, V. Hombach, A.J. Moss (eds.): Advances in Non-Invasive Electrocardiographic Monitoring Techniques. 2000. ISBN: 0-7923-6214-4 230. K. Robinson (ed.): Homocysteine and Vascular Disease. 2000 ISBN: 0-7923-6248-9 231. C.I. Berul, J.A. Towbin (eds.): Molecular Genetics of Cardiac Electrophysiology. 2000. ISBN:0-7923-7829-6 232. A. Bayés de Luna, F. Furlanello, B.J. Maron and D.P. Zipes (eds.): Arrhythmias and Sudden Death in Athletes. 2000 ISBN: 0-7923-6337-X 233. J-C. Tardif and M.G. Bourassa (eds): Antioxidants and Cardiovascular Disease. 2000. ISBN: 0-7923-7829-6 234. J. Candell-Riera, J. Castell-Conesa, S. Aguadé Bruiz (eds): Myocardium at Risk and Viable Myocardium Evaluation by SPET. 2000.ISBN: 0-7923-6724-3 235. M.H. Ellestad and E. Amsterdam (eds): Exercise Testing:New Concepts forthe New Century. 2001. ISBN: 0-7923-7378-2 236. Douglas L. Mann (ed.): TheRoleof InflammatoryMediators in theFailing Heart. 2001 ISBN: 0-7923-7381-2 237. Donald M. Bers (ed.): Excitation-Contraction Coupling and Cardiac Contractile Force, Second Edition. 2001 ISBN: 0-7923-7157-7 238. Brian D. Hoit, Richard A. Walsh (eds.): Cardiovascular Physiology in the Genetically Engineered Mouse, Second Edition. 2001 ISBN 0-7923-7536-X 239. P.A. Doevendans, A.A.M. Wilde (eds.): CardiovascularGenetics for Clinicians. 2001 ISBN 1-4020-0097-9 240. S.M. Factor, M.A. Lamberti-Abadi, J. Abadi, (eds.): Handbook of Pathology and Pathophysiology of Cardiovascular Disease. 2001 ISBN 0-7923-7542-4 Previous volumes are still available HANDBOOK OF PATHOLOGY AND PATHOPHYSIOLOGY OF CARDIOVASCULAR DISEASE by STEPHEN M. FACTOR, MD, FCAP, ACC Professor of Pathology and Medicine Albert Einstein College of Medicine Bronx, New York MARIA A. LAMBERTI – ABADI, MD Assistant Professor of Pathology Albert Einstein College of Medicine Bronx, New York and JACOBO ABADI, MD Assistant Professor of Pediatrics Albert Einstein College of Medicine Bronx, New York KLUWER ACADEMIC PUBLISHERS NEW YORK, BOSTON, DORDRECHT, LONDON, MOSCOW eBookISBN: 0-306-47575-8 Print ISBN: 0-7923-7542-4 ©2002 Kluwer Academic Publishers NewYork, Boston, Dordrecht, London, Moscow Print ©2002 Kluwer Academic Publishers Dordrecht All rights reserved No part of this eBook maybe reproducedor transmitted inanyform or byanymeans,electronic, mechanical, recording, or otherwise, without written consent from the Publisher Created in the United States of America Visit Kluwer Online at: http://kluweronline.com and Kluwer's eBookstore at: http://ebooks.kluweronline.com To my darling wife, and my lovely children with deepest appreciation for all your help and encouragement along the way. To my parents, for making me what I am. And, of course, with love to Raja. SMF With love to my husband Yaco, our children Daniela, Adriana and David, my mom Renee, my brother Victor, and especially to my dad Jose A. (Cucho) Lamberti, MD, whose memory is a driving force in my life. MA To my wife Lala and our kids, with all my HEART. You are the light of my life. To my parents, sister, and grandmother Sara, with love. JA TABLE OF CONTENTS Preface viii 1. Practical Cardiac Anatomy 1 2. Atherosclerotic HeartDisease andIschemia 19 3. Diabetes andthe Heart 67 4. Cardiomyopathies 75 Hypertrophic Dilated Endocrinopathies Alcoholic –Nutritional Deficiencies Human ImmunodeficiencyVirusInfection 5. Valvulopathies 119 Rheumatic Heart Disease Mitral Valve Prolapse 6. Congenital Heart Disease 137 Tetralogy ofFallot Turner’s Syndrome PatentDuctusArteriosus 7. Endocarditis 171 8. Myocarditis 191 9. Cardiac Tumors 203 10. The Pericardium 221 11. Aortic Aneurysms 229 12. CollagenVascularDiseasesandVasculitis: 241 Syphilis Scleroderma Systemic Lupus Erythematosus Takayasu’s disease Kawasaki’s disease 13. Medicine, LawandtheHeart 281 Appendix 299 Index 305 PREFACE Autopsy derives from the greek word autopsia, which means act of seeing with one’s own eyes. It remains the most objective and accurate method to understand human.disease. Unfortunately, the volume of autopsies in teaching hospitals has decreased dramatically over the past years. The crucial factors that account for this are the recent progress and development of new technologies, especially in diagnostic imaging, immunology, cell biology and genetics. Additionally, the perpetual fear of legal liability by physicians accounts for its further decline. Consequently, physicians and medical students are engaged in fewer autopsies and are not reaping the rich educational rewards that accompany these examinations. The purpose of the autopsy is not only to establish the cause of death, but also to determine the nature and course of the disease process. Our goal with this book is to emphasize the importance of the post-mortem exam and the correlation between pathologic material and clinical data by analyzing actual cases with problem-based methodology. The focus of this handbook is on cardiovascular disease, and when appropriate, other disease categories are included if they have an impact on cardiovascular function. The approach is more than the usual clinico-pathological correlation. Rather, we attempt to present the material from the perspective of the autopsy table. We use the clinical data as the initial framework and the autopsy findings to develop a true understanding of the disease and the associated pathophysiology of the condition. This approach is similar to the “gross” autopsy conferences that have been carried out at our institution for over 30 years. The format of the case presentation is logical and uniform with emphasis in teaching current medical concepts. Relevant clinical information is correlated and evaluated in the context of the macroscopic and microscopic findings obtained in the post-mortem exam. All cases are followed by a comprehensive discussion and review of the disease and pertinent literature is cited. We use extensive photographic material to illustrate each case and facilitate learning. We address the new issue of legal liability by presenting cases that have medico-legal implications. A thorough discussion on the impact of significant discrepancies between the autopsy findings and clinical diagnoses is also included. This is particularly important in an era that demands quality assurance and performance improvement from the medical professionals. Scientific methodology and common sense are still essential despite the forever growing knowledge of the medical sciences. Hopefully, with this book, we will succeed in guiding physicians and medical students in their continuous search for answers. Chapter 1 PRACTICAL CARDIAC ANATOMY: FROM A TO Z Introduction It may represent a bias ofthe authors, but it appears to be a truism to state that the heart is the most complex anatomical structure in the body. Yes, the brain has an intricate spatial and electrical anatomy with numerous nuclei and interconnections, but it does not approach the complexity of the heart anatomically or physiologically. No organ other than the heart is divided into pumping chambers dependent on blood flow moving between two separate circulatory circuits, with the timing ofblood movement within the chambers occurring within fractions ofa second. This movement must be coordinated by a neural network (e.g. conduction system), heart valves, vascular supply, connective tissue and muscle cells. The heart is dependent on maintaining its own nutrition, while at the same time supplying oxygenated blood to the entire body. During the process ofpumping, chamber contraction affects the supply of blood to the tissue performing the pumping, sometimes with adverse consequences. There must be an adequate flow ofoxygenated blood to the heart cells, because they have very high metabolic requirements. Although myocytes are organized as individual cells and do not form a syncytium, they must ‘talk’ to each other electrically and functionally. The cells must each contract, but this contraction must then be coordinated so that groups and layers of cells are shortening sequentially within a very short period of time. Otherwise, this would lead to hypokinesia (decreased contraction), dyskinesia (abnormal contraction), or akinesia (absence of contraction). Many books have been written on cardiac anatomy, both descriptive and illustrated. Detailed descriptions are available ofthe complex structural and functional anatomy ofthe heart; but most ofsuch works do not provide adequate explanations for acquired pathophysiology or contractile dysfunction. The rationale for this book is different: we are attempting to correlate actual case material with clinically relevant findings, and to show how clinical manifestations, pathophysiology and pathology interact to produce disease. Furthermore, we want to show how there is a certain logic and analytical process that goes into each case, if one understands clinical findings and pathology. Our goal in this book is not to re-teach cardiac anatomy. Those who are interested or stimulated to do so can find any number ofadequate books and atlases on the subject. We want to provide a brief outline of some basic features of cardiac anatomy, and by doing so, make pathophysiology and disease manifestations meaningful. The following provides an alphabetical functional anatomical glossary. It is not proposed as a complete list, but one that illustrates the principle that at least some cardiovascular diseases and their complications are determined by cardiac anatomy and structure-function relationships. Annulus The fibrous ring that supports each of the 4 cardiac valves. The annulus for the tricuspid, mitral, and aortic valves is virtually a continuous structure arranged like a pretzel. The pulmonic valve annulus is somewhat separate from the other 3 (it arises from distinctly independent embryologic tissue, e.g. bulbus cordis). The valve base inserts into the annulus with inter- digitating connective tissue fibers. Infection ofthe valve tissue (endocarditis) can affect these connections thereby causing leaflet or cuspal dehiscence, or it can lead to an infection ofthe annulus itself(e.g. valve ring abscess). The proximity of the annulae of the 3 valves means that infection can spread readily from one to the other. The annulus also anchors the base ofthe heart through connective tissue attachment fibers that extend into the muscle of the ventricle. Sections ofthe base ofthe heart just below the annulus typically reveal fibrous tissue; however, this should not be interpreted as pathological scar since it represents normal anatomy. Dilatation ofthe ventricular or atrial chambers can stretch the annulae, most likely through side to side slippage of the connective tissue fibers. The dilatation may pull the valve base away from the center channel through which blood flows, and where there is closure with valve leaflet/cusp coaptation. The result is valvular insufficiency, as the valve tissue can no longer come together properly to close the orifice. By providing a surgical shortening oftheannulus by placing crimping sutures, or by sewing in a prosthetic ring (annuloplasty), the annulus may be repaired with a decrease of insufficiency. Aortic Valve The 3 cusped valve separating the outflow tract ofthe left ventricle from the aorta. The cusps attach to the annulus, and insert as thin fibrous bands surfaced by endothelial cells (endocardium) into the aortic wall. These fibrous bands, immediately adjacent to each other but separated by less than 1 mm, form 3 commissures. Dilatation of the root of the aorta, the area immediately superior to the valve, may occur with connective tissue disease (e.g. Marfan’s syndrome), atherosclerosis, or inflammation, and may lead to aortic valvular insufficiency. In particular, with syphilis, Takayasu’s arteritis, 2 or rheumatoid arthritis, the inflammation and damage to these attachments leads to commissural separation, thus preventing cusp coaptation. The area behind the cusps, by the aortic surface, is a pocket known as the sinus of Valsalva (see below). The sinuses are potential sites of stasis, and with aging, they may develop calcification followed by aortic valve degeneration with stenosis. The sinuses also contain the two coronary ostia (see below). The aortic valve is directly continuous with the ventricular surface ofthe mitral valve. Thus, infection, or degeneration ofone valve can spread relatively easy to the other. Apex, ventricle A generally disregarded area of the heart, but one that may have important functional significance. It is composed of the most dependent portions ofboth left and right ventricles, with the myocardial fibers spiraling towards the tip of the chamber. The muscle and the associated connective tissue matrix (see below) come together as a button-like structure at this point. In fact, during normal contraction there is dimpling ofthe apex (dimples in the face are the result of connective tissue attachments between skeletal muscle and the dermis). It appears that the apex serves as a fulcrum for muscle shortening; thus, damage to the apex may have significant adverse consequences for ventricular contractile function, by ‘untethering’ the distal chamber attachments. Since the coronary supply to the apex is dependent on vessels derived from all 3 coronary arteries, obstruction ofany one vessel may lead to either generalized infarction or no damage, depending on the collateral inter-connections between the vessels. Atrio-ventricular node A modified mass of myocardial cells that serves as the electrical pacemaker ofthe ventricles. Situated in the lower inter-atrial septum, it lies immediately superior to the tricuspid valve and its annulus and the mitral valve and its annulus. Blood supply is provided by a branch of the right coronary artery in most individuals, or a branch ofthe circumflex artery in the minority of cases. Thus disease ofeither vessel may lead to ischemic damage to the node, with the potential for conduction block. The node sends a bundle of fibers into the ventricles (the bundle ofHis, see below), by penetrating the annulus of the tricuspid and mitral valves. Thus, partial or complete heart block may occur with disease processes involving the annulus such as calcific degeneration ofthe annulus, infection, or surgical trauma following prosthetic valve replacement orrepair ofa congenital defect. 3
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