Cardiovascular Medicine Third Edition_1 doc

The University of Texas Health Science Center in Houston President-Elect and Medical Director Texas Heart Institute Cardiovascular DivisionUniversity of MinnesotaMinneapolis, MN, USADavi

Third Edition

and David R Holmes, Jr (Eds)

Cardiovascular Medicine Third Edition

The University of Texas Health Science

Center in Houston

President-Elect and Medical Director

Texas Heart Institute

Cardiovascular DivisionUniversity of MinnesotaMinneapolis, MN, USADavid R Holmes, Jr., MDConsultant

Cardiovascular MedicineProfessor of MedicineScripps Professor in Cardiovascular Medicine

Mayo Clinic College of MedicineRochester, MN, USA

British Library Cataloguing in Publication Data

A catalogue record for this book is available from the British Library

Library of Congress Control Number: 2006935533

ISBN-10: 1-84628-188-1 3rd edition eISBN-10: 1-84628-725-1

ISBN-13: 978-1-84628-188-4 3rd edition eISBN-13: 978-1-84628-725-1

ISBN 0-443-07000-8 2nd edition, published in 2000 by Churchill Livingstone

Printed on acid-free paper

© Springer-Verlag London Limited 2007

Apart from any fair dealing for the purposes of research or private study, or criticism or review,

as permitted under the Copyright, Designs and Patents Act 1988, this publication may only

be reproduced, stored or transmitted, in any form or by any means, with the prior permission

in writing of the publishers, or in the case of reprographic reproduction in accordance with the terms of licences issued by the Copyright Licensing Agency Enquiries concerning repro-duction outside those terms should be sent to the publishers

The use of registered names, trademarks, etc in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant laws and regu-lations and therefore free for general use

Product liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature

Preface

he third edition of Cardiovascular Medicine is the product of our continuing

effort to provide an authoritative and comprehensive review of important, clinically relevant cardiovascular disease topics As compared to the second edition, this third edition contains 27 new chapters and a 30% expansion and update

of mechanistic, diagnostic, and therapeutic approaches to cardiovascular medicine Each chapter is written by one or more experts on the topic We have also added two additional editors, Dr Hein J.J Wellens, a leading worldwide authority on cardiac arrhythmias, their recognition and treatment, and Dr David Holmes, Jr.,

a worldwide leader in interventional cardiovascular medicine

The continued rapid growth of new knowledge and new techniques has lenged the physician with a wide array of management options We believe the third

chal-edition of Cardiovascular Medicine will guide the dedicated physician to more

knowledgeable and effective preventive and therapeutic efforts in patients with diovascular disease

car-The specialty of cardiovascular medicine has become increasingly international Advances in basic science and clinical research are not restricted to national bound-aries Global interaction among scientists and physicians has rapidly expanded, as

a result of major improvements in rapid communication, and the growing tion of our interdependence in advancing clinical science We have therefore main-tained our commitment to include contributions from American, European, and Asian-Pacific experts to provide a balanced discussion of opinions from gifted physi-cians active worldwide in the care and treatment of patients with cardiovascular diseases

recogni-We hope that the organization of the third edition will enhance its value nostic approaches are considered from a methodological perspective in separate chapters in the beginning of the book, but are readdressed later in separate chapters dealing with specific disease categories Chapters on clinical syndromes are designed

Diag-to incorporate anaDiag-tomic, mechanistic, diagnostic, and treatment considerations Whether using the book as a quick reference guide or as a source for comprehensive coverage of a topic, the reader should have access to up-to-date information in an accessible format

We are committed to a long-term effort to update this book so that it keeps abreast of new and important advances We shall therefore provide our readers access

to a Web site that will update as necessary all clinically related chapters every three months by adding new diagnostic and therapeutic information of importance

The third edition of Cardiovascular Medicine is accompanied by a CD-rom that

presents the entire book on-line, but that also includes an introductory section on heart sounds of virtually every conceivable cardiovascular abnormality and associ-ated echocardiographic images These images serve as an outstanding educational opportunity for physicians in training, cardiovascular nurses, and cardiovascular technical associates who are involved in cardiovascular imaging procedures and for physicians updating their auscultatory and echocardiographic skills in cardiovascu-lar diagnosis

We wish to express our appreciation to our editors at Springer for the

oppor-tunity to present the third edition of Cardiovascular Medicine and for their

assis-tance and patience in bringing it to fruition Our heartfelt thanks go to our families and our collaborators at the Texas Heart Institute and The University of Texas Health Science Center in Houston, Texas; the University of Minnesota Medical School in Minneapolis, Minnesota; the Mayo Clinic College of Medicine in Roch-ester, Minnesota; and Maastricht University in Maastricht, The Netherlands We deeply appreciate the expertise and dedicated assistance of Suzy Lanier at the Texas Heart Institute and The University of Texas Health Science Center in Houston; Amy Brown and Cheryl Tincher at the University of Minnesota Medical School T

in Minneapolis; and Karyn Hughes and Ann Turner at the Mayo Clinic in Rochester.

We are very grateful to our colleagues throughout the United States and around the world who have contributed important chapters Finally, we express our great appreciation to our teachers, students, and patients from whom we have learned

so much It is to them and our families that we dedicate the third edition of

Cardiovascular Medicine

James T Willerson, MDJay N Cohn, MDHein J.J Wellens, MDDavid R Holmes, Jr., MD

vii

Preface v Contributors xvii ACC/AHA Guidelines xxix

SECTION I Introduction: Cardiac Signs and

Symptoms, and Selected Noninvasive Diagnostic Methods

1 Anatomy of the Heart 3

L Maximilian Buja

2 The History and Physical Examination 19

Thomas C Smitherman and James T Willerson

SECTION II Congenital Heart Disease in the Adult

9 Normal and Abnormal Anatomy 205

Robert H Anderson and Anton E Becker

10 Pathophysiology, Clinical Recognition, and Treatment of

Congenital Heart Disease 233

Steven R Neish and Jeffrey A Towbin

11 Echocardiography in the Adult with Congenital

13 Surgical Treatment 341

Magdi Habib Yacoub, Anselm Uebing, Rosemary Radley-Smith, and Michael A Gatzoulis

SECTION III Valvular Heart Disease

14 Valvular Heart Disease: Anatomic Abnormalities 369

Hugh A McAllister, Jr., L Maximilian Buja, and

†Victor J Ferrans

15 Aortic Valve Disease 381

Blase A Carabello

16 Pulmonary and Tricuspid Valve Disease 393

Otto M Hess, Urs Scherrer, Pascal Nicod, and Blase A Carabello

17 Mitral Valve Diseases 397

Maurice L Enriquez-Sarano and Robert L Frye

20 The Assessment and Therapy of Valvular Heart Disease in the Cardiac Catheterization Laboratory 463

Paul Sorajja and Rick A Nishimura

21 Echocardiographic Assessment of Valvular Heart Disease 487

Raymond F Stainback

22 Magnetic Resonance Imaging of Valvular Disease 537

Scott D Flamm and Raja Muthupillai

23 Balloon Dilatation of the Cardiac Valves 557

Igor F Palacios and Pedro L Sánchez

24 Valvular Heart Disease: Surgical Treatment 581

William E Cohn, O.H Frazier, and Denton A Cooley

SECTION IV Coronary Artery Disease

25 Coronary Artery Disease: Pathologic Anatomy and Pathogenesis 593

L Maximilian Buja and Hugh A McAllister, Jr.

26 Inflammation, C-Reactive Protein, and Vulnerable Plaques 611

Paolo Calabró, James T Willerson, and Edward T.H Yeh

†Deceased

27A Atherosclerotic Vulnerable Plaques: Pathophysiology,

Detection, and Treatment 621

Mohammad Madjid, Samuel Ward Casscells, and

James T Willerson

27B Biomarkers of Inflammation as Surrogate Markers in

Detection of Vulnerable Plaques and Vulnerable Patients 641

Mohammad Madjid, Samuel Ward Casscells, and

Matthew B O’Steen and Neal S Kleiman

32 Coronary Disease in Women 713

Allen P Burke, Frank D Kolodgie, and Renu Virmani

33 Exercise Testing 729

Bernard R Chaitman, Masarrath J Moinuddin, and

Junko Sano

34 Coronary Angiography 745

Robert F Wilson and Carl W White

35 Echocardiographic Evaluation of Coronary Artery Disease 811

Stephanie A Coulter

36A Myocardial Perfusion Imaging Utilizing Single Photon

Emission Computed Tomography Techniques 841

George A Beller

36B Cardiac Positron Emission Tomography 855

K Lance Gould

37A Magnetic Resonance Imaging of the Myocardium 871

Raymond J Kim, Igor Klem, and Robert M Judd

37B Magnetic Resonance Angiography and Evaluation of

Vulnerable Plaque 897

Javier Sanz, Marc Sirol, Zahi A Fayad, and

Valentin Fuster

38 Medical Treatment of Stable Angina 911

James J Ferguson III, Dipsu D Patel, and

James T Willerson

39 Medical Treatment of Unstable Angina, Acute Non–ST-Elevation Myocardial Infarction, and Coronary Artery Spasm 937

James T Willerson and Paul W Armstrong

40 Treatment of Acute ST-Elevation Myocardial Infarction 963

Paul W Armstrong and James T Willerson

Pim J de Feyter and Peter P.T de Jaegere

44 Percutaneous Coronary Intervention for Acute Myocardial Infarction 1021

David R Holmes, Jr.

45 Drug-Eluting Coronary Stents 1031

Carey D Moyer, Peter B Berger, and Christopher J White

46 Surgical Treatment of Coronary Artery Disease 1051

William E Cohn, O.H Frazier, and Denton A Cooley

47 Coronary Artery Bypass Surgery and Percutaneous Coronary Revascularization: Impact on Morbidity and Mortality in Patients with Coronary Artery Disease 1073

James M Wilson, James J Ferguson III, and Robert J Hall

48 Cardiac Rehabilitation 1113

Michael X Pham, Jonathan N Myers, and Victor F Froelicher

SECTION V Basic Aspects of Myocardial Function,

Growth, and Development

49 Cardiac Development: Toward a Molecular Basis for Congenital Heart Disease 1135

Michael D Schneider and Eric N Olson

50 Fueling the Heart: Multiple Roles for Cardiac Metabolism 1157

Heinrich Taegtmeyer

51 Cardiac Hypertrophy 1177

Ali J Marian and James T Willerson

52 Regulation of Cardiac Contraction and Relaxation 1189

SECTION VI Myocardial Disease

54 Myocardial Disease: Anatomic Abnormalities 1219

Hugh A McAllister, Jr., L Maximilian Buja, and

Jay W Mason, Sanjeev Trehan, and Dale G Renlund

60 Evaluation of Myocardial Disease in the Cardiac

Catheterization Laboratory 1349

James J Ferguson III and Tomas Klima

61 Echocardiography in the Evaluation of

65 Heart Transplantation: Pathogenesis, Immunosuppression,

Diagnosis, and Treatment of Rejection 1443

Leslie W Miller

66 Surgical Treatment of Advanced Heart Failure 1461

O.H Frazier, Igor D Gregoric, and William E Cohn

SECTION VII Pericardial Disease

67 Pericardial Disease: Anatomic Abnormalities 1479

Hugh A McAllister, Jr., L Maximilian Buja, and

†Victor J Ferrans

68 Pericardial Disease: Etiology, Pathophysiology, Clinical

Recognition, and Treatment 1483

Ralph Shabetai

†Deceased

SECTION VIII Vascular Disease

69 Molecular and Cellular Physiology of Differentiated Vascular Smooth Muscle 1511

Michael Sturek, Eric A Mokelke, Jürgen R Sindermann, Leonard P Adam, and Keith L March

70 Cardiovascular Regulation: Basic Considerations 1525

Giuseppe Mancia, Thomas F Lüscher, John T Shepherd, George Noll, and Guido M Grassi

71 Vascular Remodeling in Health and Disease 1541

Luis G Melo, Massimiliano Gnecchi, Christopher A Ward, and Victor J Dzau

72 Vascular Endothelial Cell Function and Thrombosis 1567

H Roger Lijnen, Jef M Arnout, and Désiré Collen

73 Atherosclerosis: Pathologic Anatomy and Pathogenesis 1581

L Maximilian Buja and Hugh A McAllister, Jr.

74 Atherosclerosis: Pathogenesis, Morphology, and Risk Factors 1593

Antonio M Gotto, Jr., and John A Farmer

75 Detection of Early Cardiovascular Disease 1615

Daniel A Duprez and Jay N Cohn

76 Diseases of the Aorta 1623

James T Willerson, Joseph S Coselli, Scott A LeMaire, Ross M Reul, Igor D Gregoric, George J Reul, and Denton A Cooley

77 Aneurysms of the Peripheral Arteries 1663

Paul W Wennberg and Henna Kalsi

78 Peripheral Arterial Diseases 1681

Alan T Hirsch, Henna Kalsi, and Thom W Rooke

79 Venous Disease 1705

Samuel Z Goldhaber

80 Angiogenesis 1717

Pinak B Shah and Douglas W Losordo

81 Carotid Artery Intervention 1741

Christopher J White

82 Endovascular Procedures for the Treatment of Peripheral Vascular Disease 1755

Kathryn G Dougherty and Zvonimir Krajcer

83 B-Mode Ultrasound: A Noninvasive Method for Assessing Atherosclerosis 1783

John R Crouse, Curt D Furberg, Mark A Espeland, and Ward A Riley

Henry S Loeb and Jay N Cohn

88 Autonomic Dysfunction and Hypotension 1883

Christopher J Mathias

89 Cerebrovascular Disease/Transient Ischemic Attack 1911

Hashem M Shaltoni and Frank M Yatsu

SECTION IX Electrical Disturbances of the Heart

90 Sinus Node Dysfunction 1925

David G Benditt, Scott Sakaguchi, Keith G Lurie,

and Fei Lu

91 Supraventricular Tachycardias 1943

Hein J.J Wellens

92 Atrial Fibrillation and Flutter 1955

Peter A Brady and Bernard J Gersh

David G Benditt and Scott Sakaguchi

98 Sudden Cardiac Death 2039

Abdi Rasekh, Mehdi Razavi, and Ali Massumi

99 Antiarrhythmic Drugs 2085

Dan M Roden, Dawood Darbar, and Prince J Kannankeril

100 Cardiac Pacemakers 2103

Sanjay Dixit and Francis E Marchlinski

101 The Implantable Cardioverter-Defibrillator 2119

William H Sauer and David J Callans

102 Catheter Ablation of Supraventricular and Ventricular Arrhythmias 2139

Luz-Maria Rodriguez, Carl Timmermans, and Hein J.J Wellens

103 Surgical Treatment of Arrhythmias 2163

Spencer J Melby, James L Cox, and Ralph J Damiano, Jr.

SECTION X Cardiac Effects of Systemic Disorders,

Pregnancy, Aging, and Environmental Changes

104 Pulmonary Thromboembolism 2177

Herbert L Fred, Shahzad Hashim, and Fady A Joudah

105 Pulmonary Arterial Hypertension 2203

Evangelos D Michelakis and Stephen L Archer

106 Chronic Obstructive Pulmonary Disease 2247

Rosa Maria Estrada-Y-Martin and Steven D Brown

107 Tumors of the Heart 2267

Raymond F Stainback, Yasmin S Hamirani, Denton A Cooley, and L Maximilian Buja

108 Endocrine Disorders and the Heart 2295

Victor R Lavis, Michalis K Picolos, and James T Willerson

109 Connective Tissue Diseases and the Heart 2331

Frank C Arnett and James T Willerson

110 Substance Abuse and the Heart 2357

Paul A Grayburn and Eric J Eichhorn

111 Cardiovascular Involvement in Acquired Immune Deficiency Syndrome 2371

Melvin D Cheitlin, Priscilla Hsue, and Merle A Sande

112 Cardiac Involvement in Skeletal Myopathies and Neuromuscular Disorders 2385

Ali J Marian and James T Willerson

113 Hematologic Disease and Heart Disease 2409

Martin D Phillips and James T Willerson

114 Hypercoagulable State 2423

Andrew I Schafer

115 Aging and the Cardiovascular System 2439

Samer S Najjar, Gary Gerstenblith, and

Edward G Lakatta

116 Pregnancy and the Heart 2453

Susan Wilansky, Christina S Reuss, and

James T Willerson

SECTION XI Surgery and the Heart

117 Evaluation of Patients for Noncardiac Surgery 2487

James B Froehlich and Kim A Eagle

118 Anesthesia for Cardiovascular Operations 2501

N Martin Giesecke and John R Cooper, Jr.

119 Intraoperative Hemodynamic Monitoring 2515

Rebecca A Schroeder, Shahar Bar-Yosef, and

123 Genetic Basis for Cardiac Arrhythmias 2577

Connie R Bezzina and Arthur A.M Wilde

124 Genetic Aspects of Congenital Heart Disease 2599

Dianna M Milewicz

SECTION XIII Preventive Cardiology

125 Coronary Risk Factors: An Overview 2609

Donald M Lloyd-Jones and William B Kannel

126 Preventive Cardiology: The Effects of Exercise 2631

Amit Khera, Jere H Mitchell, and Benjamin D Levine

127 Smoking, Secondhand Smoke, and

Cardiovascular Disease 2649

Joaquin Barnoya and Stanton A Glantz

128 Management of Cholesterol Disorders 2667

Scott M Grundy

129 Cardiovascular Complications of Obesity and the Metabolic Syndrome 2693

Paul Poirier and Robert H Eckel

130 Gene Therapy 2721

Robert D Simari and Elizabeth G Nabel

131 Molecular Biology for the Clinician 2731

Sara Arab, Liyong Zhang, Yuichiro Maekawa, Urszula Zurawska, and Peter P Liu

132 Stem Cell Therapy for Cardiac Diseases 2745

Emerson C Perin, Guilherme V Silva, and James T Willerson

133 Cost-Effectiveness Issues 2771

William S Weintraub

134 Erectile Dysfunction and Cardiovascular Disease 2791

Sanjay Kaul and James S Forrester

135 Cardiovascular Disease and Insulin Resistance 2803

Ramzi A Ajjan and Peter J Grant

136 The Heart and the Kidney 2819

Martin R Cowie

Index 2839 Credits 2907

Molecular Vascular Medicine

Faculty of Medicine and Health

University Health Network

Toronto General Hospital

Toronto, Ontario, Canada

Internal Medicine, Pathology

The University of Texas

Houston Medical School

Joaquin Barnoya, MD, MPH

Department of EpidemiologyUniversity of California, San Francisco

San Francisco, CA, USA

and

Departamento de Investigacion y Docencia

Unidad de Cirugía Cardiovascular

de GuatemalaGuatemala

Shahar Bar-Yosef, MD

Department of AnesthesiologyDuke University Medical Center

and

Anesthesiology ServiceVeterans Affairs Medical CenterDurham, NC, USA

Anton E Becker, MD, PhD

Academic Medical CenterUniversity of AmsterdamAmsterdam, The Netherlands

George A Beller, MD

Department of Internal MedicineCardiovascular Division

University of Virginia Health SystemCharlottesville, VA, USA

David G Benditt, MD, FACC, FRCPC, FHRS

Department of MedicineCardiovascular DivisionUniversity of Minnesota Medical School

Minneapolis, MN, USA

Peter B Berger, MD

Interventional CardiologyGeisinger Clinic

Danville, PA, USA

Biykem Bozkurt, MD, FACC

MEDVAMC CardiologyMichael E DeBakey Veterans Affairs Medical Center and Baylor College

of MedicineHouston, TX, USA

Peter A Brady, MD, FRCP

Division of Cardiovascular DiseasesMayo Clinic and Mayo FoundationRochester, MN, USA

Steven D Brown, MD

Department of MedicineThe University of Texas Health Center

at TylerTyler, TX, USA

Hans-Rudolph Brunner, MD (Retired)

Riehen, Switzerland

Dirk L Brutsaert, MD, PhD

Cardiology Department

AZ Middelheim HospitalUniversity of AntwerpAntwerp, The Netherlands

Matthew J Budoff, MD, FACC, FAHA

Division of CardiologyHarbor-UCLA Medical CenterTorrance, CA, USA

Baltimore, MD, USA

Michel Burnier, MD

NephrologyDepartment of MedicineUniversity HospitalCHUV

Lausanne, Switzerland

Paolo Calabró, MD

Department of Cardiothoracic Science

Second University of NaplesNaples, Italy

David J Callans, MD

Cardiology, MedicineHospital of the University of Pennsylvania

Philadelphia, PA, USA

Blase A Carabello, MD

Department of MedicineHouston Veterans Affairs Medical Center

Samuel Ward Casscells, MD

The University of Texas Health Science Center at HoustonTexas Heart InstituteHouston, TX, USA

Bernard R Chaitman, MD

Cardiovascular ResearchDivision of CardiologySaint Louis University School of Medicine

St Louis, MO, USA

Y.S Chandrashekhar, MD, DM

Department of Medicine/Division

of CardiologyVetrans Affairs Medical CenterMinneapolis, MN, USA

Melvin D Cheitlin, MD

Department of MedicineSan Francisco General HospitalSan Francisco, CA, USA

Jay N Cohn, MD

Rasmussen Center for Cardiovascular Disease Prevention

Cardiovascular DivisionUniversity of MinnesotaMinneapolis, MN, USA

Division of Cardiothoracic Surgery

Michael E DeBakey Department of

Department of Adult Cardiology

Texas Heart Institute

Washington University School of

Medicine/Barnes Jewish Hospital

St Louis, MO, USA

John R Crouse, MD

Internal Medicine

Wake Forest University School of

Medicine

Medical Center Boulevard

Winston Salem, NC, USA

Ralph J Damiano, Jr., MD

Surgery

Washington University School of

Medicine/Barnes Jewish Hospital

St Louis, MO, USA

Dawood Darbar, MBChB, MD

MedicineVanderbilt University School of Medicine

Nashville, TN, USA

Sanjay Dixit, MB BS

Department of MedicineUniversity of Pennsylvania Health System

Philadelphia, PA, USA

Kathryn G Dougherty, CRTT, CVT

Clinical Research

St Luke’s Episcopal HospitalTexas Heart InstituteHouston, TX, USA

Daniel A Duprez, MD, PhD

Cardiovascular DivisionUniversity of MinnesotaMinneapolis, MN, USA

Victor J Dzau, MD

Department of MedicineDuke University Medical CenterDurham, NC, USA

Kim A Eagle, MD

Department of Internal MedicineUniversity of Michigan Medical School

Ann Arbor, MI, USA

Rochester, MN, USA

Mark A Espeland, PhD

Department of Biostatistical SciencesWake Forest University Health Sciences

Medical Center BoulevardWinstom-Salem, NC, USA

Rosa Maria Estrada-Y-Martin, MD

Division of Pulmonary, Critical Care and Sleep Medicine

The University of Texas Health Science Center at Houston

Houston, TX, USA

John A Farmer, MD

MedicineBaylor College of MedicineHouston, TX, USA

Diane Fatkin, MD, BSc (Med), FRACP

Molecular CardiologyVictor Chang Cardiac Research Institute

Darlinghurst, NSW, Australia

Zahi A Fayad, PhD

Cardiovascular InstituteMount Sinai School of MedicineNew York, NY, USA

James J Ferguson III, MD

Cardiology ResearchTexas Heart InstituteHouston, TX, USA

†Victor J Ferrans, MD, PhD

Pim J de Feyter, MD, PhD

Department of CardiologyErasmus MC-Thorax CenterRotterdam, The Netherlands

Peter J Fitzgerald, MD, PhD, FACC

Division of Cardiovascular MedicineStanford University Medical CenterStanford, CA, USA

O.H Frazier, MD

Cardiopulmonary TransplantationTexas Heart Institute

and

Division of Thoracic and Cardiovascular SurgeryThe University of Texas Medical SchoolHouston, TX, USA

Herbert L Fred, MD, MACP

Internal MedicineThe University of Texas Health Science Center at HoustonHouston, TX, USA

Robert L Frye, MD

Cardiovascular DiseasesMayo Clinic

Rochester, MN, USA

Curt D Furberg, MD, PhD

Public Health SciencesWake Forest University School of Medicine

Winston-Salem, NC, USA

Valentin Fuster, MD, PhD

Cardiovascular InstituteMount Sinai HospitalNew York, NY, USA

Michael A Gatzoulis, MD, PhD, FACC, FESC

National Heart and Lung InstituteRoyal Brompton Hospital

Sydney StreetLondon, UK

Layne O Gentry, MD

St Luke’s Episcopal HospitalHouston, Texas, USA

Bernard J Gersh, MB, ChB, DPhil

Division of Cardiovascular Diseases and Internal Medicine

Mayo ClinicRochester, MN, USA

Gary Gerstenblith, MD

MedicineThe Johns Hopkins University School of Medicine

University of Pavia and

IRCCS Policlinico San Matteo

Pavia, Italy

Samuel Z Goldhaber, MD

Cardiovascular Division

Department of Medicine

Brigham and Women’s Hospital

Harvard Medical School

Boston, MA, USA

†John Goodwin, SPK, MD, FRCP,

FACC, FESC

Anton P.M Gorgels, MD, PhD

Department of Cardiology

Cardiovascular Research Institute

Maastricht, The Netherlands

Antonio M Gotto, Jr., MD, DPhil

Weill Medical College of Cornell

Molecular Vascular Medicine

Faculty of Medicine and Health

Mechanical Circulatory Support

Texas Heart Institute

Houston, TX, USA

Scott M Grundy, MD, PhD

Center for Human NutritionThe University of Texas Southwestern Medical Center at Dallas

Shahzad Hashim, MD

Internal MedicineThe University of Texas Health Science Center at Houston

Houston, TX, USA

Otto M Hess, MD

Department of CardiologySwiss Cardiovascular CenterBern, Switzerland

Minneapolis, MN, USA

David R Holmes, Jr., MD

Cardiovascular MedicineMayo Clinic College of MedicineRochester, MN, USA

Yasuhiro Honda, MD, FACC

Division of Cardiovascular MedicineStanford University Medical CenterStanford, CA, USA

Priscilla Hsue, MD

Department of MedicineSan Francisco General HospitalSan Francisco, CA, USA

Seung-Ho Hur, MD, PhD

Division of Cardiovascular MedicineStanford University Medical CenterStanford, CA, USA

Peter P.T de Jaegere, MD, PhD

Intervention CardiologyErasmus MC

Rotterdam, The Netherlands

†Deceased

Fady A Joudah, MD

Internal MedicineHouston Veterans Affairs Medical Center

Henna Kalsi, MD

Cardiovascular MedicineMayo Clinic

Rochester, MN, USA

Prince J Kannankeril, MD, MSCI

Department of PediatricsVanderbilt UniversityNashville, TN, USA

William B Kannel, MD, MPH, FACC

Preventive MedicineBoston University School of MedicineBoston, MA, USA

Hirohisa Kato, MD, PhD, FACC

Cardiovascular Research InstituteKurume University

Gilles W de Keulenaer, MD, PhD

Department of Cardiology

AZ Middelheim HospitalUniversity of AntwerpAntwerp, Belgium

Amit Khera, MD

Division of CardiologyThe University of Texas Southwestern Medical Center

Neal S Kleiman, MD

Cardiac Catheterization LaboratoryMethodist DeBakey Heart CenterHouston, TX, USA

Igor Klem, MD

DCMRCDuke University Medical CenterDurham, NC, USA

Tomas Klima, MD

Department of PathologyTexas Heart InstituteHouston, TX, USA

Ann Arbor, MI, USA

Zvonimir Krajcer, MD

Peripheral Vascular ProgramTexas Heart InstituteHouston, TX, USA

Edward G Lakatta, MD

Laboratory of Cardiovascular Science

National Institute on Aging, National Institute of HealthBaltimore, MD, USA

Victor R Lavis, MD

Internal Medicine (Endocrinology)The University of Texas-HoustonMedical School

Houston, TX, USA

Scott A LeMaire, MD

Division of Cardiothoracic SurgeryMichael E DeBakey Department of Surgery

Baylor College of Medicine

and

Cardiovascular SurgeryTexas Heart Institute

St Luke's Episcopal HospitalHouston, TX, USA

Benjamin D Levine, MD

Institute for Exercise and Environmental MedicinePresbyterian Hospital of DallasDallas, TX, USA

H Roger Lijnen, PhD

Center for Molecular and Vascular Biology

KU LeuvenLeuven, Belgium

Department of Preventative Medicine

Northwestern University Feinberg

School of Medicine

Chicago, IL, USA

Henry S Loeb, MD

Department of Cardiology

Edward Hines JR VA Hospital

Hines, IL, USA

Douglas W Losordo, MD

Cardiovascular Medicine

Caritas St Elizabeth’s Medical Center

Boston, MA, USA

Medicine and Emergency Medicine

University of Minnesota Medical

Baylor College of Medicine

Texas Heart Institute

Houston, TX, USA

Warren J Manning, MD

Cardiovascular Division

Beth Israel Deaconess Medical Center

Boston, MA, USA

Keith L March, MD, PhD

Medicine, Cellular and Integrative Physiology, and Biomedical Engineering

Indiana University School

of MedicineIndianapolis, IN, USA

Francis E Marchlinski, MD

Department of MedicineUniversity of Pennsylvania Health System

Philadelphia, PA, USA

Ali J Marian, MD

The University of Texas Health Science Center at HoustonTexas Heart InstituteHouston, TX, USA

Jonathan B Mark, MD

Department of AnesthesiologyDuke University Medical Center

and

Anesthesiology ServiceVeterans Affairs Medical CenterDurham, NC, USA

Ali Massumi, MD

Baylor College of Medicine

St Luke’s Episcopal Hospital

National Hospital for Neurology and Neurosurgery and Institute of Neurology

Hugh A McAllister, Jr., MD (Retired)

Previously at Baylor College of Medicine and The University of Texas-Houston Medical School and Texas Heart Institute

Houston, Texas, USA

Arthur Iain McGhie, MD

Department of Internal MedicineDivision of Cardiology

University of Missouri—Kansas City/

Mid America Heart InstituteKansas City, MO, USA

St Louis, MO, USA

Luis G Melo, PhD

Department of PhysiologyQueen’s UniversityKingston, Ontario, Canada

Evangelos D Michelakis, MD

Division of CardiologyUniversity of AlbertaEdmonton, Alberta, Canada

Dianna M Milewicz, MD, PhD

Internal MedicineThe University of Texas Medical School at Houston

Houston, TX, USA

Leslie W Miller, MD

Department of MedicineGeorge Town UniversityGeorge Town, WA, USA

Eric A Mokelke, PhD

Cellular and Integrative PhysiologyIndiana University School of MedicineIndianapolis, IN, USA

Carey D Moyer, MD

Cardiology Associates of West ReadingThe Reading Hospital and Medical Center

West Reading, PA, USA

Charles E Mullins, MD

Department of PediatricsBaylor College of Medicine/Texas Children’s Hospital

Houston, TX, USA

Raja Muthupillai, PhD

Clinical Science—MR and Department

of RadiologyPhilips Medical Systems and Baylor College of Medicine

Houston, TX, USA

Jonathan N Myers, PhD

Department of CardiologyDepartment of Veterans Affairs Palo Alto Health Care System

Palo Alto, CA, USA

of MedicineOrange, CA, USA

Steven R Neish, MD

Department of PediatricsTexas Children’s HospitalHouston, TX, USA

Massachusetts General Hospital

Boston, MA, USA

Stem Cell Center

Texas Heart Institute

Houston, TX, USA

Michael X Pham, MD, MPH

Department of Cardiology

Department of Veterans Affairs Palo

Alto Health Care System

Palo Alto, CA, USA

Medicine Division of Endocrinology

The University of Texas-Houston

Medical School

Houston, TX, USA

Paul Poirier, MD, PhD, FRCPC

Department of Cardiology

Quebec Heart Institute/Laval

Univer-sity Faculty of Pharmacy

Ste-Foy, Québec, Canada

Philip A Poole-Wilson, MD, FRCP, FMedSci

National Heart and Lung InstituteFaculty of Medicine

Imperial College LondonLondon, UK

Rosemary Radley-Smith, FRCP

Department of PediatricsHarefield HospitalMiddlesex, UK

Cleveland, OH, USA

Dale G Renlund, MD

Division of CardiologyUniversity of Utah School

of MedicineSalt Lake City, UT, USA

George J Reul, MD

Department of SurgeryTexas Heart InstituteHouston, TX, USA

Ward A Riley, BA, MS, PhD

Wake Forest University Health Sciences

Winston-Salem, NC, USA

Dan M Roden, MD

Clinical PharmacologyVanderbilt University School of Medicine

Nashville, TN, USA

Luz-Maria Rodriguez, MD, PhD

Department of CardiologyFaculty of MedicineUniversity of MaastrichtMaastricht, The Netherlands

Thom W Rooke, MD

Vascular CenterMayo ClinicRochester, MN, USA

Mary Ella Round, MD

William H Sauer, MD

Department of MedicineUniversity of ColoradoDenver, CO, USA

Andrew I Schafer, MD

Department of MedicineUniversity of PennsylvaniaPhiladelphia, PA, USA

Urs Scherrer, MD

Department of CardiologyUniversity of LausanneLausanne, Switzerland

Michael D Schneider, MD

Medicine, Molecular and Cellular Biology, and Molecular Physiology and Biophysics

Baylor College of MedicineHouston, TX, USA

Rebecca A Schroeder, MD

Department of AnesthesiologyDuke University Medical Center

and

Anesthesiology ServiceVeterans Affairs Medical CenterDurham, NC, USA

Christine E Seidman, MD

Department of Genetics and Medicine

Howard Hughes Medical InstituteHarvard Medical School

Boston, MA, USA

J.G Seidman, PhD

Department of Genetics and Medicine

Howard Hughes Medical InstituteHarvard Medical School

Boston, MA, USA

Ralph Shabetai, MD, FACC

Department of Cardiology

VA Health Care System

La Jolla, CA, USA

Pinak B Shah, MD

Cardiovascular MedicineCaritas St Elizabeth’s Medical CenterBoston, MA, USA

Hashem M Shaltoni, MD

Neurology—Stroke ProgramThe University of Texas Health Science Center at HoustonHouston, TX, USA

John T Shepherd, MD, PhD

Department of PhysiologyMayo Clinic FoundationRochester, MN, USA

Guilherme V Silva, MD

Stem Cell CenterTexas Heart InstituteHouston, TX, USA

Robert D Simari, MD

Division of Cardiovascular DiseasesMayo Clinic College of MedicineRochester, MN, USA

Thomas C Smitherman, MD

Department of Internal Medicine

Division of Cardiology

and the Cardiovascular Institute

University of Pittsburgh School of

Medicine and University of

Pittsburgh Medical

Center-Presbyterian

Pittsburgh, PA, USA

Paul Sorajja, MD

Division of Cardiovascular Diseases

and Internal Medicine

Mayo Clinic College of Medicine

Rochester, MN, USA

Raymond F Stainback, MD, FACC,

FASE

St Luke’s Episcopal Hospital

Texas Heart Institute

Houston, TX, USA

Michael Sturek, PhD

Cellular and Integrative Physiology

Indiana University School

of Medicine

Indianapolis, IN, USA

Heinrich Taegtmeyer, MD, DPhil

Department of Pediatric Cardiology

Baylor College of Medicine

Houston, TX, USA

Anselm Uebing, MD

Adult Congenital Heart Disease Unit

Royal Brompton Hospital and Harefield

NHS Trust

London, UK

Renu Virmani, MD

CVPath Institute, Inc

International Registry of Pathology

Gaithersburg, MD, USA

Bernard Waeber, MD

Division of Clinical PathophysiologyDepartment of MedicineUniversity HospitalCHUV

Lausanne, Switzerland

Christopher A Ward, PhD

Department of PhysiologyQueen’s UniversityKingston, Ontario, Canada

William S Weintraub

Department of MedicineChristian Care Health SystemNewark, DE, USA

Hein J.J Wellens, MD

Department of CardiologyUniversity of MaastrichtMaastricht, The Netherlands

Christopher J White, MD

Department of CardiologyOchsner Clinic Foundation,New Orleans, LA, USA

Susan Wilansky, MD, FACC, FASE

Department of CardiologyMayo Clinic

Scottsdale, AZ, USA

Arthur A.M Wilde, MD, PhD

Experimental and Molecular Cardiology Group

Department of Clinical and Experimental CardiologyAcademic Medical CenterAmsterdam, The Netherlands

James T Willerson, MD

The University of Texas Health Science Center at HoustonTexas Heart InstituteHouston, TX, USA

Temple W Williams, Jr., MD

The Methodist Hospital Corporation

Houston, Texas

James M Wilson, MD

Department of Cardiology

St Luke's Episcopal HospitalTexas Heart InstituteHouston, TX, USA

Robert F Wilson, MD, PhD

Department of MedicineUniversity of MinnesotaMinneapolis, MN, USA

Walter R Wilson, MD

Mayo Clinic and FoundationRochester, NY, USA

Magdi Habib Yacoub, FRS

Heart Science CentreImperial College LondonHarefield, Middlesex, UK

Frank M Yatsu, MD

Neurology—Stroke ProgramThe University of Texas Health Science Center at Houston

Houston, TX, USA

Edward T.H Yeh, MD

Department of CardiologyThe University of Texas, MD Anderson Cancer Center

Houston, TX, USA

Paul G Yock, MD

Department of BioengineeringStanford University

Stanford, CA, USA

Liyong Zhang, MD

The Heart and Stroke and Richard Lewar Center of ExcellenceUniversity of TorontoToronto, Canada

Urszula Zurawska

University of TorontoToronto, Canada

x x i x

ACC/AHA Guidelines

Many of the topics covered by chapters in Cardiovascular Medicine, Third Edition, are researched by writing groups of the American College of Car-

diology and the American Heart Association, and the research results in guidelines that are prepared jointly by these groups Members of the writing groups are experts in their cardiovascular specialties, and the guidelines undergo a rigid review process before being approved and published in the ACC/AHA Joint Guidelines collection.

A direct link to the Guidelines collection is provided below:

http://www.americanheart.org/presenter.jhtml?identifier =3004542

References to the guidelines and applicable chapters are listed below ACC/AHA/ESC 2006 Guidelines for Management of Patients with Ventric- ular Arrhythmias and the Prevention of Sudden Cardiac Death Executive Summary (Circulation 2006;114:1088–1132)

Chapters 94, 96, 97, 98, 99, 101, 102.

ACC/AHA/ESC 2006 Guidelines for the Management of Patients with Atrial Fibrillation Executive Summary (Circulation 2006;114:700–752) Chapters 92, 93, 95, 99, 103.

AC/AHA 2006 Guidelines for the Management of Patients with Valvular Heart Disease Executive Summary (Circulation 2006;114:450–527) and Full Text.

Chapters 15, 16, 17, 23, 24.

AHA/ACC 2006 Guidelines for Secondary Prevention for Patients with Coronary and Other Atherosclerotic Vascular Disease (Circulation 2006; 113:2363–2372)

Chapters 26, 27a, 27b, 28, 30, 74, 128.

AHA/ACC 2006 Guideline Update on Perioperative Cardiovascular tion for Noncardiac Surgery: Focused Update on Perioperative Beta-Blocker Therapy (Circulation 2006;113:2662–2674)

Chapter 117

ACC/AHA 2006 Clinical Performance Measures for Adults with tion and Non ST-Elevation Myocardial Infarction A Report of the ACC/ AHA Task Force on Performance Measures (Circulation 2006;113:607– 608)

Chapters 39, 40

ACC/AHA 2005 Guidelines for the Management of Patients with Peripheral rial Disease (Lower Extremity, Renal, Mesenteric, and Abdominal Aortic) Executive Summary (Circulation 2006;113:1474–1547) and Full Text (Circulation 2006;113:463–654)

Chapters 77, 78, 80, 81, 82.

ACCF/AHA 2005 Clinical Competence Statement on Cardiac Imaging with Computed Tomography and Magnetic Resonance (Circulation 2005;112: 598–617)

Chapters 7, 8, 22.

ACC/AHA 2005 Guideline Update for the Diagnosis and Management of Chronic Heart Failure in the Adult (Circulation 2005;112:e154–e235) Chapters 62, 63, 64.

ACC/AHA 2005 Key Data Elements and Definitions for Measuring the Clinical Management and Outcomes of Patients with Chronic Heart Failure (Circulation 2005;112:1888–1916)

Chapters 7, 8, 22.

ACC/AHA 2004 Guideline Update for Coronary Artery Bypass Graft Surgery Full Text (Circulation 2004;110:340–437) and Summary Article (Circula- tion 2004;110:1168–1176)

Chapter 46, 47.

ACC/AHA 2004 Guidelines for the Management of Patients with tion Myocardial Infarction Full Text (Circulation 2004;110:82–293) Execu- tive Summary (Circulation 2004;110:588–636)

Chapter 40.

ACC/AHA/ASNC 2003 Guidelines for the Clinical Use of Cardiac nuclide Imaging Full Text and Executive Summary (Circulation 2003;108: 1404–1418).

Chapters 6, 36a, 36b.

ACC/AHA/ESC 2003 Guidelines for the Management of Patients with Supraventricular Arrhythmias Full Text and Executive Summary (Circula- tion 2003;108:1871–1909).

Chapters 90, 91, 92, 93.

ACC/AHA/ASE 2003 Guidelines for the Update for the Clinical Application

of Echocardiography Summary Article (Circulation 2003;108:1146) and Full Text.

Chapters 5, 11,12, 21, 35, 61, 95.

ACC/AHA 2002 Guideline Update for the Management of Patients with Chronic Stable Angina Summary Article (Circulation 2003;107:149) and Full Text.

Chapter 38.

ACC/AHA 2002 Guideline Update for the Management of Patients with Unstable Angina and Non-ST Segment Elevation Myocardial Infarction Full Text and Summary Article (Circulation 2002;106:1893)

Chapter 39

ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices Full Text and Summary Article (Circulation 2002;106:2145)

Chapters 100, 101.

ACC/AHA 2002 Guideline Update for Exercise Testing Summary Article (Circulation 2002;106:1883)

Chapter 33

Introduction:

Cardiac Signs

and Symptoms, and Selected

Noninvasive

Diagnostic

Methods

SECTION I

• Structure-function relationships of the pericardium.

• Distribution of the coronary arteries and anatomic

variations

• Definition of anatomic right and left atria and right and

left ventricles

• Structure of the four cardiac valves and concept of

func-tional valve apparatus

• Anatomy of the cardiac conduction system and cardiac

innervation

This chapter presents basic features of the anatomy of the

heart and great vessels, including the embryologic

devel-opment of these structures and their configuration in the

mature state Basic knowledge of cardiovascular anatomy is

essential for effective diagnosis and treatment of

cardiovas-cular diseases

Embryologic Development

Basic Embryology

Development of the cardiovascular system occurs in the

early first trimester fetus Beginning at about 3 weeks’

gesta-tion, elements of splanchnic mesoderm differentiate into a

primitive cardiac tube and pericardial cavity, and vascular

channels form and fuse to form blood vessels.1–7The

primi-tive cardiac tuberesults from the moving together and fusion

of two lateral endothelial heart tubes Subsequently, the

epimyocardial mantle and cardiac jelly develop These

com-ponents differentiate into the endocardium, producing the

internal endothelial lining of the heart, the myocardium

forming the muscular wall, and the epicardium or visceral

pericardium producing the outside covering of the heart The

recently formed cardiac tube is a single chambered structure and is composed of the following components, extending from inferior (caudal) to superior (cephalad): the sinus venosus, which connects to the major veins; the atrium; the ventricle; the bulbus cordis or conus; and the truncus arterio-sus, which connects through six pairs of aortic arches to two dorsal aortae1–7

(Fig 1.1)

Initially, the single chambered heart is a straight tube

residing in the pericardial cavity The bulboventricular tion grows much more rapidly than the pericardial cavity As

por-a result, further extension in por-a longitudinpor-al direction cpor-annot occur, and the heart tube is forced to bend The cephalic ventricular portion of the tube bends in a ventral and caudal direction and to the right, whereas the caudal atrial portion progresses in a dorsal and cranial direction and to the left

This process, known as d(dextro)-bulboventricular looping,

results in the atrial region establishing a position superior to the ventricular region and the cardiac apex being pointed to the left (Fig 1.1)

The external shape changes are accompanied by a complex process of internal septation that leads to the for-mation of a four-chambered heart (Fig 1.2) In the atrium,

a septum primum forms and then develops two openings: ostium primum and ostium secundum A septum secun-dum then develops on the right of the septum primum The foramen ovale is formed in the midportion of the devel-oping interatrial septum as a result of the growth and positioning of the septum secundum adjacent to the septum primum The sinus venosus is incorporated into the superior portion of the developing atria Ventricular septa-tion is partially accomplished by the upward growth of muscular tissue to form the muscular interventricular septum Endocardial cushion tissue develops and provides the essential tissue for formation of the atrioventricular valves, the closure of the ostium primum in the atrial septum, and the formation of the membranous interven-tricular septum

The primitive bulbus cordis contributes several key

components of the forming heart The proximal third of the

bulbus cordis forms the trabeculated part of the right

ven-tricle; the midportion, or conus cordis, forms the outflow

tracts of both ventricles; and the distal part, the truncus

arteriosus, forms the proximal parts of the aorta and

pulmo-nary artery The junction between the primitive ventricle

and the bulbus cordis is designated the primary

interven-tricular foramen While the veninterven-tricular septation process

proceeds, the primitive ventricle develops into the major

component of the definitive left ventricle, and the proximal

one third of the bulbus cordis gives rise to the major

compo-nent of the definitive right ventricle

Separate aortic and pulmonary channels are formed by

separation of the truncus arteriosus by a spiral

aorticopul-monary septum (Fig 1.3) As a result of extensive remodeling

of the double aortic arch system, the definitive major vessel

system develops into a single aorta with left-sided aortic

arch, a pulmonary trunk with right and left main pulmonary

arteries, and the ductus arteriosus, which connects the aortic

arch and the left pulmonary artery

The heart develops in the fetus as myocardial contractile

activity commences, and a functional circulation is

estab-lished The foramen ovale in the interatrial septum and the

ductus arteriosus remain open In the fetus, pulmonary

vas-cular resistance is high in the unexpanded lungs and

sys-temic vascular resistance is low As a result of these anatomic

and physiologic features, the fetal circulation involves right

to left shunting of blood across the open foramen ovale and

the patent ductus arteriosus to provide oxygenated blood

from the placenta to the general circulation (Fig 1.4) After

birth, the lungs expand, pulmonary vascular resistance

drops, and systemic vascular resistance increases This leads

to functional closure of the foramen ovale and ductus riosus and, subsequently, fibrous closure of these structures These closures establish complete separation of the right-sided and left-sided circulations in the mature cardiovascular system

arte-Relationship to Congenital Heart Disease

Knowledge of normal and abnormal embryologic ment of the heart and blood vessels provides an essential basis for and understanding of the morphogenesis of congeni-tal heart disease.8,9

develop-The position of the atria is determined

by general body habitus The anatomic right atrium is defined

as the atrium receiving the systemic venous drainage The

anatomic left atrium is defined as the atrium receiving the

pulmonary venous drainage In situs solitus, the right atrium

is on the right and the left atrium on the left side of the body

In situs inversus, the anatomic right atrium is on the left side

and the anatomic left atrium is on the right side of the body The right and left ventricles are normally connected to the corresponding atria, but the ventricles may be inverted Inversion of the ventricles is a major feature of a condition known as congenitally corrected transposition of the great vessels This condition arises when the primitive heart undergoes levo(l)-bulboventricular looping rather than the usual dextro(d)-bulboventricular looping; hence, the alterna-tive designation of corrected transposition as l-transposition Other positional anomalies of the heart include dextrocardia and right-sided aortic arch

Abnormalities in atrial septation give rise to three types

of atrial septal defects; in order of frequency they are the ostium secundum defect, ostium primum defect, and sinus venosus defect Ostium secundum defects are located in the midportion of the interatrial septum and result from inadequate formation of septum secundum and/or septum primum to cover the foramen ovale Ostium primum defects are located in the inferior portion of the interatrial septum and result from a defect in formation of endocardial cushion tissue Severe endocardial cushion deficiency can lead to

a common atrioventricular canal anomaly with ostium primum atrial septal defect, membranous ventricular septal defect, and abnormal atrioventricular valve The sinus venosus atrial septal defect is located in the superior portion

of the interatrial septum and results from defective poration of the primitive sinus venosus into the forming heart; this defect is often associated with partial anomalous pulmonary venosus drainage into the right atrium Abnor-malities in ventricular septation give rise to ventricular septal defects, usually in the region of the membranous septum Formation of the membranous interventricular septum involves contributions from the conal ridges of the bulbus cordis and the endocardial cushions Deficiencies in contributions from these embryonic structures leads to the formation of membranous ventricular septal defects Abnor-malities in septation of the great vessels give rise to con-genital complete transposition of the great vessels, or d-transposition, since the abnormal septation of the great vessels occurs with normal dextro-bulboventricular looping and the atria and ventricles are in normal position Con-genital complete transposition is often accompanied by

IIIIVVI

FIGURE 1.1 Ventral views of human embryonic hearts that show

bending of cardiac tube and establishment of major anatomic

com-ponents At., atrium; l., left; r., right; Tr Art., truncus arteriosus

Septum spuriumInteratrialseptum primum

Ostium IAtrio-vent canal

Intervent septum

S sp

Rightatrium

Ostium II(opening)Ostium I(closing)

Leftventricle

Septumspurium

A_V canal cushionIntervent foramen(closes at 15–17 mm.)

Ostium IIOstium I(closed)

Ostium II

in septum I

Cristaterminalis

Foramenovale

Atriovent

valvesBundle

of his

S II

S II

Functional outlet F.O

Septum I(valvula F.O.)

atrial or ventricular septal defects or other congenital

lesions Severe maldevelopment of the heart can give rise to

a hypoplastic left heart or hypoplastic right heart and

asso-ciated valvular atresia Other anomalies include vascular

rings, persistent patent ductus arteriosus, and coarctation of

the aorta

Cardiovascular malformations occur in about 0.8% of

live births.10 However, the incidence of congenital heart

disease is significantly higher because cardiac

malforma-tions occur 10 times more often in stillborn than in liveborn infants Children with congenital heart disease are predomi-nantly male However, specific defects have a definite sex preponderance: females have a higher incidence of patent ductus; arteriosus and atrial septal defect are more common

in females; and males have a higher incidence of valvular aortic stenosis, congenital aneurysm of the sinus of Valsalva, coarctation of the aorta, tetralogy of Fallot, and congenital complete transposition of the great arteries.10

FIGURE 1.2 Longitudinal sections of embryonic heart in frontal

plane that show extent of growth of various cardiac septa at

progres-sive stages of development These diagrams depict the stages of

partitioning of the human embryo Stippled areas indicate the

dis-tribution of endocardial cushion tissue; muscle is shown in diagonal

hatching, and epicardium in solid black The lightly stippled areas

in the atrioventricular canal in B and C indicate location of dorsal and ventral endocardial cushions of the atrioventricular canal before they have grown sufficiently to fuse with each other in the plane of the diagram

Dextrodorsalconus ridge

Dextrodorsaltruncus ridge

Conovent flange

Arrow

in aorticchannelAtrioventricularcanalDorsal atriovent

canal cushionInterventricularseptum

Pulmonarychannel S.-v truncus

ridgeAorticchannelS.-v

conusridge

Ventralatriovent

canalcushion

Leftventricle

FIGURE 1.3 Frontal plane dissections of developing heart show

important relations in establishing aortic and pulmonary outlets

The truncus arteriosus has been drawn with its cut end turned

upward, in order to show the absence of truncus ridges in the early stages and their relationships in later stages

Superior vena cavaCrista

terminalisSeptum II

Limbus offoramenovaleValve ofinferiorvena cavaValve ofcoronarysinusTricuspid valveSeptummembranaceum

Interventricular septum(muscular portion)

Right ventricle

Trabeculaecarneae ofleft ventricle

Papillarymuscle

Tendinouscords

Left atrium

Orifices ofpulmonary veins

Septum I = valvulaforaminis ovalis

Margin of interatrialforamen II

Septum I(remnant)

Mitral valve

FIGURE 1.4 Schematic drawing to show interrelations of septum

primum and septum secundum during the latter part of fetal life

Note especially that the lower part of septum primum is positioned

so as to act as a one-way valve at the oval foramen in septum

secun-dum The split arrow indicates that a considerable part of the blood

from the inferior vena cava passes through the foramen ovale to the left atrium while the remainder eddies back into the right atrium

to mix with the blood being returned by way of the superior vena cava

External Anatomy of the Heart and

Great Vessels

The anatomy of the heart and great vessels has been well

documented in previous publications.11–17 The normal

loca-tion of the heart is in the mediastinum to the left of the

midline with the cardiac apex pointed to the left (Fig 1.5)

The heart is rotated and tilted in the chest, and as a result

about two thirds of the anterior surface of the heart is

com-posed of the right ventricle, and the left third of the anterior

surface is composed of the left ventricle The right inferior

border (obtuse border) of the heart is formed by the right

ventricle and the left lateral border is formed by the left

ventricle Located superior to the right and left ventricles

are the right and left auricles of the right and left atria,

respectively The anterior superior surface of the heart is

constituted by the outflow portion (conus) of the right

ventricle and the pulmonary trunk, which extends from

right to left as it exits the pericardium The pulmonary

trunk then gives rise to the left and right main pulmonary

arteries The aorta is located posterior to the pulmonary

trunk The aorta takes origin from the left ventricle and is

oriented from left to right as it exits the pericardium The

aorta then curves to the left and inferiorly, creating a

left-sided aortic arch The aortic arch gives origin to the right

innominate, left common carotid, and left subclavian

arter-ies The aorta then continues inferiorly as the descending

thoracic aorta

Pericardium

The pericardial cavity is a fluid-filled space that surrounds

the heart and proximal great vessels The pericardial space

is enclosed by a thin layer of connective tissue that is lined

by a single layer of mesothelial cells There are two nents: the visceral and parietal pericardium The visceral pericardium covers the epicardium of the heart, and the parietal pericardium forms the outer layer The superior extent of the pericardial cavity representing the transition

compo-of visceral to parietal pericardium occurs approximately 2 to

3 cm superior to the heart at the level of the great vessels, thereby enclosing the proximal aorta and pulmonary trunk

in the pericardial cavity (Fig 1.5) The pericardial cavity normally contains about 20 cc of serous fluid, which serves

to lubricate the heart and facilitate cardiac motion

The pericardium can be affected by a variety of matory and neoplastic conditions Hemorrhage into the peri-cardium may occur as a result of cardiac rupture, usually secondary to acute myocardial infarction, or rupture of the proximal aorta following aortic dissection The severity of cardiac dysfunction secondary to pericardial disease is influ-enced acutely by the amount and rapidity of fluid accumula-tion in the pericardial cavity and chronically by the severity

inflam-of inflammation and fibrosis Rapid accumulation inflam-of 100 to

200 mL of fluid or blood in the pericardial cavity can induce

cardiac tamponade, whereas the slow accumulation of several hundred milliliters can be accommodated in the

InnominatearteryRight innomi-nate vein

R int mam veinThymus glandReflection ofpericardiumVena cavasup

Asc aortaProbe intransv

sinusRightauricleRightatriumCoronarysulcusRightventricle

MargoacutusIncisuraapiciscordisPleura

Apex

Anteriorlongi-tudinalsulcus

Leftventricle

Margoobtusus

Leftauricle

Conusarteriosus

PulmonaryarteryPleuraFibrouspericardiumSerouspericardium

Left subclavian arteryLeft innominate veinPleura

FIGURE 1.5 Ventral view of heart in situ

with the pericardial sac opened

cavity with stretching of the pericardial lining before

impaired cardiac function develops

Heart

Basic Structure

The anatomy of the heart has been documented in detail.11–17

The heart is composed of three layers: the epicardium, the

myocardium, and the endocardium The epicardium consists

of fatty connective tissue and is lined by the visceral

peri-cardium The major coronary arteries and veins traverse the

epicardium The myocardium constitutes most of the mass

of the heart and is composed of cardiac myocytes, vessels,

and connective tissue The cardiac myocytes represent

approximately 80% of the mass but only 20% of the number

of cells in the myocardium The endocardium is divided into

the nonvalvular (visceral) and valvular endocardium The

endocardium consists of thin fibrocellular connective tissue,

which is lined by a single layer of endothelial cells

Cardiac Dimensions

Several sources have provided information regarding

dimen-sions and measurements of the heart.11–17The weight of the

heart varies in relationship to body dimensions, including

length and weight Hudson11 has published a useful guide

regarding fresh heart weight in males and females The adult

male heart weight has the following parameters: 0.45% of

body weight, average 300 g, range 250 to 350 g The adult

female heart weight has the following parameters: 0.40% of

body weight, average 250 g, range 200 to 300 g Selective

mea-surements of right ventricular and left ventricular weights

can be made, and ranges have been established for

determina-tion of selective enlargement of right and left ventricles as

well as biventricular enlargement.15

The thickness of the walls of the cardiac chambers is as follows: right and left atria, 0.1 to 0.2 cm; right ventricle, 0.4 to 0.5 cm; left ventri-cle, 1.2 to 1.5 cm (free wall, excluding papillary muscles and large trabeculae) The average circumferences of the cardiac valves are as follows: aortic, 7.5 cm; pulmonic, 8.5 cm; mitral, 10.0 cm; and tricuspid, 12.0 cm

Coronary Vasculature

The anatomy and physiology of the coronary circulation have been described in detail.18–25 In the normal heart, oxygenated blood is supplied by two coronary arteries that are the first branches of the aorta The origin of the left and right coro-nary arteries from the aorta is through their ostia positioned

in the left and right aortic sinuses of Valsalva, which are located just distal to the left and right cusps, respectively, of

the aortic valve (Figs 1.5, 1.6, and 1.7) The left main nary artery is a short vessel with a length of 0.5 to 1.5 cm The left main coronary artery divides into left anterior descending and left circumflex branches and, occasionally, a

coro-left marginal branch The coro-left anterior descending coronary artery and its left diagonal and septal branches supply the anterior portion of the left ventricles and interventricular

septum The left circumflex coronary artery and its

circum-flex marginal branches supply the lateral left ventricle The

right coronary artery supplies the right ventricle and, in about 90% of hearts, it extends posteriorly to give rise to the posterior descending coronary artery

There is considerable variation in the anatomic tion of the coronary arterial branches However, in most hearts, branches from both the left circumflex and right coronary arteries contribute to the blood supply of the pos-terior left ventricle, resulting in a so-called balanced circula-tion In about 10% of hearts, the right coronary artery is small, and the left circumflex coronary artery gives origin

distribu-Right pulmonary veins

Vena cava superior

Incisura apicis cordis

Left pulmonary veins

Left auricleLeft atriumCircumflex branchLeft coronaryarteryLeft aortic sinusGreat cardiac veinLeft pulmonarysemilunar valve

Anterior longitudinalsulcusLeft ventricle

Anterior descendingbranch of leftcoronary artery

Right and anteriorpulmonary semilunarvalves

Pulmonary arteryConus arteriosus

FIGURE 1.6 Cephalic view of the heart with

the epicardium removed to expose the injected coronary vessels

cardiac collaterals); and intramural branches, which municate with the cardiac cavities (arterioluminal vessels)

com-In the normal adult heart, the collateral vessels are thin walled, small channels, usually less than 50 μm in diameter, and they contribute little to total coronary blood flow In response to coronary arterial narrowing and myocardial ischemia, the capacity of the coronary collateral system can greatly increase The myocardial collateral vessels can increase in diameter into the range of 200 to 600 μm or

to the posterior descending coronary artery and provides the

sole blood supply for the posterior left ventricle, creating a

left dominant circulation Rarely, the converse right

domi-nant circulation exists when the left circumflex is small and

the posterior left ventricle is supplied primarily by left

ven-tricular branches of the right coronary artery

The major blood supply to the sinoatrial node via the

sinus node artery is derived from the proximal right

coro-nary artery in about 60% of hearts and from the left

circum-flex coronary artery in about 40% of hearts.18 The major

atrioventricular nodal artery is derived from the coronary

artery that gives rise to the posterior descending branch,

which is the right coronary artery in about 90% and the left

circumfl ex coronary artery in about 10% of hearts

The epicardial coronary arteries deliver oxygenated blood

to the intramyocardial arteries, arterioles, and capillaries

leading to oxygen and substrate extraction in the

myocar-dium (Fig 1.8) A small amount of unoxygenated blood flows

directly into the ventricular cavities via the thebesian veins

However, most desaturated blood traverses the myocardial

venules and veins into the epicardial veins, which drain into

the coronary sinus located in the inferoposterior region of

the right atrium

Collateral blood vessels form during embryologic

devel-opment of the heart, and they connect different components

of the coronary arterial circulation.18–25The coronary

collat-eral system is composed of four types of vessels: intramural

branches of the same coronary artery (homocoronary

collat-erals); intramural branches of two or more coronary arteries

(intercoronary collaterals); atrial branches, which connect

with the vasa vasorum of the aorta and other vessels

(extra-Superiorleft pulmonary

vein

Inferior leftpulmonary veinLeft atriumOblique vein ofleft atriumInteratrial sulcusGreat cardiac veinCoronary sinus

Posterior vein ofleft ventricle

Diaphragmaticsurface of leftventricle

Posterior longitudinal sulcus

Posterior ing branch of rightcoronary artery

descend-Middle cardiacvein

Rightventricle

Rightcoronaryartery

Smallcardiac vein

Vena cavainferiorValvula venaecavaeRight atrium

Terminalsulcus

Inferior rightpulmonary vein

Vena cava superiorSuperior rightpulmonary vein

FIGURE 1.7 Dorsocaudal view of the heart

with the epicardium removed to expose the

injected coronary vessels

EndocardiumArterioluminalvesselMyocardial sinusoidIntertrabecular spaceAnastomosis betweenmyocardial sinusoidsArteriosinusoidalvesselTrabeculae carneaeCapillary bedMyocardial sinusoidCapillaries emptying intomyocardial sinusoidsThebesian veinAnastomosis betweenthebesian veins

Coronary vein

Venovenousanastomosis

Arteriovenousanastomosis

Anastomosesbetweencoronary arteriesCoronary artery

EpicardiumMyocardium

FIGURE 1.8 Diagram of the ventricular wall, showing the

relation-ship between the various intramural vascular channels

greater, develop muscular media, and transport significant amounts of blood flow (Fig 1.9) In addition to the types of collateral vessels described above, collateral channels also can develop proximal and distal to a stenosis in a given coro-nary artery.

Right and Left Atria and Ventricles

The right and left atria and right and left ventricles have

distinctive anatomic features (Figs 1.10 to 1.17) The tomic right ventricle is characterized as follows: distinct muscular infundibulum (conus) arteriosus, which separates the right semilunar (pulmonary) valve and the right atrioven-tricular (tricuspid valve); single large anterior papillary muscle; and coarse trabecular muscles (trabeculae carneae cordis) at the apical and inflow portion of the chamber.11–17Key landmarks of the right ventricular infundibular (conus) region from inferior to superior are the membranous inter-ventricular septum; the crista supraventricularis, an inverted V-shaped structure with parietal and septal limbs; and the

ana-pulmonic valve The anatomic left ventricle has the

follow-ing features: fibrous continuity of annulus of left semilunar (aortic) valve and anterior leaflet of left atrioventricular (mitral) valve, two well-developed papillary muscles (antero-lateral and posteromedial), and fine trabecular muscles at the apical and inflow portion of the chamber.11–17These features allow determination of the anatomic right ventricle and ana-tomic left ventricle in complex congenital anomalies involv-ing displacement of the various components of the heart

Cardiac Valves

The four-chambered heart has four valves: the right minar or pulmonic valve; the right atrioventricular or tricus-pid valve; the left semilunar or aortic valve; and the left

semilu-FIGURE 1.9 Radiograph of postmortem coronary arteriogram

dem-onstrating an extensive coronary collateral system in a case of

coro-nary heart disease The proximal part of the left anterior descending

artery is obliterated by old disease (O) The more distal part of the

anterior descending artery (A) has filled through a rich anastomotic

network (X) that has formed in the substance of the interventricular

septum between branches of the anterior (A) and posterior (P)

Orifice of coronary sinus

Valvula sinus coronarii

Valvula venae cavae

Vena cava inferior

Probe in transverse sinus

Right auricle Vena cava superior Right ventricle Crista terminalis Left ventricle Right atrium Tricuspid valve:

Anterior cusp Medial cusp Posterior cusp Musculus pectinatus Right coronary artery Small cardiac vein

Ligamentum arteriosum

Aorta

FIGURE 1.10 Right anterior oblique

view of the excised heart, with the right atrium opened to show its internal configuration