Pediatric Cardiac Surgery E-Book

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Sprache: Englisch

Seitenzahl: 3061

Veröffentlichungsjahr: 2012

Beschreibung

Pediatric cardiac surgery is a dynamic, fast-moving field. Busy practitioners, like you, need clear and comprehensive guidance you can rely on to ensure optimal patient care.

For over 25 years Pediatric Cardiac Surgery has been the gold-standard reference for pediatric and adult congenital heart surgeons, pediatric and congenital cardiologists, intensivists, anesthesiologists, residents and nurses.

Now, in this thoroughly revised fourth edition, you again get trusted, complete coverage of the field with timely new features and expert reviews of critical topics including heart transplantation, emerging modalities for diagnosing congenital heart and tracheal defects,  the surgical technique of Fontan conversion with arrhythmia surgery, the medical challenges of managing adult CHD patients, and more.

This new edition includes:

• Contributions from over 65 world-renowned experts
• More beautiful illustrations, by renowned medical illustrator Rachid Idriss,  which have brought acclaim to previous editions
• Reviews of the embryology, physical findings, diagnostic criteria, and therapeutic choices for each disease entity and describes the latest in surgical techniques in each chapter
• All-new chapters that guide readers through new treatment options and other key developments since the publication of the third edition highlighting recent advances in congenital heart surgery.
• All-new new chapters that review advances in right ventricular to pulmonary artery conduits, arrhythmia surgery, double outlet ventricles, and adult congenital heart disease, among other key topics.

Leseprobe

Table of Contents

Cover

Title page

Copyright page

List of Contributors

Preface

CHAPTER 1 Development of the Heart and Great Vessels

Introduction

Formation of Cardiac Precursors

Formation of the Tube Heart

Looping, Convergence, and Septation – Key Landmarks

The Atrioventricular Junction Segment and Atrioventricular Valve Development

Development of the Conduction System

Morphogenesis of the Outflow Tract

Morphogenesis of the Great Vessels and Coronary Circulation

CHAPTER 2 Nomenclature and Classification of Pediatric and Congenital Heart Disease

Background

Segmental Anatomy

Classification of Cardiac Lesions

Description of Cardiac Lesions

Summary

CHAPTER 3 Physiology of the Fetal and Neonatal Circulations and Fetal Cardiac Surgery

Fetal Circulation

Circulatory Changes at Birth

Fetal Surgery

Fetal Cardiac Surgery

CHAPTER 4 Preoperative Diagnostic Evaluation

Introduction

History

Physical Examination

Chest Radiography

Electrocardiography

Preoperative Echocardiography

Cardiac Magnetic Resonance Imaging

Cardiac Catheterization

Angiography

CHAPTER 5 Hybrid Procedures for Congenital Heart Disease

Introduction

Elements of a Hybrid Program for Congenital Heart Disease

Design of a Hybrid Suite

Hybrid Therapy for Vascular Stenoses

Hybrid Closure of Atrial Septal Defects and Ventricular Septal Defects

Hybrid Approach for Hypoplastic Left Heart Syndrome

Completion Angiography in the Cardiac Operating Suite

Hybrid Approach to Valve Replacement

Summary

CHAPTER 6 Anesthesia for the Patient with Congenital Heart Disease

Demographics

Anesthetic Assessment

Anesthetic Management

Anesthesia and Cardiac Imaging

Pulmonary Hypertension

Cardiac Pharmacology

Postanesthesia Process

Conclusion

CHAPTER 7 Perioperative Care

Preoperative Preparation

Intraoperative Care

Routine Postoperative Care

Postoperative Complications

Conclusion

CHAPTER 8 The Nurse Practitioner’s Role in Patient Management

History of Nurse Practitioners in Cardiothoracic Surgery

Diagnosis

Preoperative Evaluation

Perioperative Process

Postoperative Assessment and Complications

Pain Management

Discharge Planning

Patient and Family Satisfaction

Conclusion

CHAPTER 9 Palliative Operations

Pulmonary Artery band

Aortopulmonary Shunts

Miscellaneous Palliative Procedures

Conclusion

CHAPTER 10 Management of Pediatric Cardiopulmonary Bypass

History

Cardiopulmonary Bypass in Infants versus Adults

Physiology of Cardiopulmonary Bypass

Neurological Injury and Protection

Myocardial Protection

Pulmonary Effects of Cardiopulmonary Bypass

Renal Function and Protection on Cardiopulmonary Bypass

Endocrine Response to CPB

Systemic Inflammation

Management of Cardiopulmonary Bypass

Conclusions

CHAPTER 11 Intraoperative Myocardial Protection

Physiologic Differences Between Immature and Mature Myocardium

Clinical Strategies for Myocardial Protection

Clinical Techniques

Conclusion

CHAPTER 12 Patent Ductus Arteriosus

Introduction

Anatomy

Etiologic Factors

Pathophysiology

Natural History

Clinical Features

Diagnosis

Management

Surgical Repair of PDA

Special Considerations in Premature Infants

Special Consideration in Infants and Children

Special Considerations in Adults

Surgical Outcomes

Percutaneous Closure of PDA

Conclusion

CHAPTER 13 Vascular Rings and Pulmonary Artery Sling

Classification

Embryology and Pathology

Clinical Presentation and Diagnosis

Surgical Management

Postoperative Care

Video-Assisted Thoracoscopic Surgery

Conclusion

CHAPTER 14 Coarctation of the Aorta

Embryology and Anatomy

Natural History and Pathophysiology

Diagnosis

Surgical Techniques

Conclusion

CHAPTER 15 Interrupted Aortic Arch

Introduction

Pathologic Anatomy of the Aortic Arch

Associated Anomalies

Pathophysiology and Clinical Features

Historical Perspective

Diagnosis of Interrupted Aortic Arch

Management of Interrupted Aortic Arch

Postoperative Management of Interrupted Aortic Arch

Results of Surgery

Complications of Surgery

Conclusion

CHAPTER 16 Atrial Septal Defect, Partial Anomalous Pulmonary Venous Connection, and Scimitar Syndrome

Anatomy and Pathology

Pathophysiology and Natural History

Diagnosis

Surgical Technique

Postoperative Care

Technical Pitfalls

Results of Operation

Scimitar Syndrome

Conclusion

CHAPTER 17 Ventricular Septal Defect

Definition and Prevalence

Historical Perspectives

Embryology and Pathologic Anatomy

New Concepts of Nomenclature

Pathophysiology

Natural History

Diagnosis

Medical Management

Patient Selection

Surgical Considerations

Surgical Management of Ventricular Septal Defects with Associated Lesions

Complications

Results

Conclusions

CHAPTER 18 Atrioventricular Canal Defects

Pathology and Anatomy

Hemodynamics/Natural History

Diagnosis

Indications and Timing of Operation

Operative Management and Results

Results

Conclusion

CHAPTER 19 Truncus Arteriosus

Embryologic and Anatomic Features

Physiology and Clinical Findings

Natural History

Management

Operative Technique

Repair of Truncus with Interrupted Aortic Arch

Truncal Valve Repair/Replacement

Results of Total Correction

CHAPTER 20 Aortopulmonary Window and Aortic Origin of a Pulmonary Artery

Introduction

Historical Aspects

Embryology and Anatomy

Clinical Features and Natural History

Diagnosis

Surgical Treatment

Postoperative Course

Outcome After Surgery

CHAPTER 21 Isolated Right Ventricular Outflow Tract Obstruction

Pulmonary Atresia with Intact Ventricular Septum

Valvar Pulmonary Stenosis

Other Forms of Isolated Right Ventricular Outflow Tract Obstruction

Conclusion

CHAPTER 22 Tetralogy of Fallot

Definition, Morphology, and Nomenclature

History of Surgical Management

Presentation and Diagnosis

Medical Management

Surgical Repair

Palliative Procedures

Complete Repair

Results

Long-term Complications Following Repair of Tetralogy of Fallot

Special Circumstances

Conclusion

CHAPTER 23 Surgical Treatment of Pulmonary Atresia with Ventricular Septal Defect

Introduction

Clinical Features

Diagnostic Evaluation

Surgical Management

Postoperative Management

Follow-Up Evaluation

Outcomes

CHAPTER 24 Ventricular to Pulmonary Artery Conduits

Polytetrafluoroethylene Monocusp Right Ventricular Outflow Tract Reconstruction

Allograft Conduits

Pulmonary Allografts in Ross Aortic Valve Replacement Patients

Bovine Jugular Venous Valved Conduit

Percutaneous Pulmonary Valves

Nonvalved Right Ventricular–Pulmonary Artery Conduits

Stented and Stentless Xenograft Valves in the Right Ventricular–Pulmonary Artery Position

Mechanical Valves in the Pulmonary Position

Bioengineered Valved Conduits for Right Ventricular Outflow Tract Reconstruction

When to Operate for Right Ventricular Outflow Tract Dysfunction

Conclusion

CHAPTER 25 Double-Outlet Ventricles

DOUBLE-OUTLET RIGHT VENTRICLE

Introduction

History

Embryology

Morphology

Classification

Pathophysiology

Diagnosis

Natural History

Treatment

DOUBLE-OUTLET LEFT VENTRICLE

Introduction

History

Embryology

Morphology

Pathophysiology

Diagnosis

Natural History

Treatment

CHAPTER 26 Transposition of the Great Arteries

Embryologic and Anatomic Considerations

Physiology

Clinical Presentation

Medical Management of the Neonate

Surgical Management

Results and Analysis

Arterial Switch and Ventricular Septal Defect Closure for Transposition of the Great Arteries and Ventricular Septal Defect

Current Approach

Staged Repair of Transposition of the Great Arteries with Intact Ventricular Septum

Arterial Switch Operation for Double-Outlet Right Ventricle and Subpulmonary Ventricular Septal Defect (Taussig-Bing Anomaly)

Staged Arterial Switch Operation for Right Ventricular Failure After the Atrial Baffle Procedures

Late Complications and Reoperations Following the Arterial Switch Operation

CHAPTER 27 Congenitally Corrected Transposition of the Great Arteries

History

Anatomy

Natural History and Physiology

Diagnosis

Surgical Management

Results

Conclusion

CHAPTER 28 The Functionally Univentricular Heart and Fontan’s Operation

Background

Nomenclature and Classification

Anatomy

Natural History

Evolution of Surgery for Functionally Univentricular Hearts

Surgical Methods

Results of Surgery

Late Considerations

Conclusion

CHAPTER 29 Ebstein Anomaly

Anatomy

Physiology

The Neonate

Children and Adults

Postoperative Management

Risk Factors and Outcome

CHAPTER 30 Left Ventricular Outflow Tract Obstruction

Introduction

Valvar Aortic Stenosis

Subvalvar Aortic Stenosis

Supravalvar Aortic Stenosis

Left Ventricular Outflow Tract Obstruction as Part of Multiple Obstructions in the Heart–Aorta Complex

CHAPTER 31 Hypoplastic Left Heart Syndrome

Pathology

Clinical Picture and Medical Treatment

Therapy

Staged Reconstruction

Neurodevelopmental Outcomes

Orthotopic Cardiac Transplantation

Conclusion

CHAPTER 32 Aortico-Left Ventricular Tunnel

Pathophysiology and Anatomy

Pathogenesis

Presentation and Diagnosis

Treatment

Conclusion

CHAPTER 33 Congenital Anomalies of the Mitral Valve

Historical Notes

Normal Mitral Valve Anatomy

Pathologic Anatomy, Classification, and Analysis

Pathophysiology

Principles of Treatment

Surgical Technique

Postoperative Care

Results

CHAPTER 34 Total Anomalous Pulmonary Venous Connection

Overview

Embryology

Classification

Cardiac Anatomy, Associated Lesions

Natural History

Diagnosis

Operative Management and Anesthetic Considerations

Results

CHAPTER 35 Cor Triatriatum Sinister, Pulmonary Vein Stenosis, Atresia of the Common Pulmonary Vein, and Cor Triatriatum Dexter

Embryology of Pulmonary Venous System

Pathologic Anatomy

Cor Triatriatum Sinister

Atresia of the Common Pulmonary Vein

Pulmonary Vein Stenosis

Cor Triatriatum Dexter

CHAPTER 36 Anomalous Systemic Venous Connections

Introduction

History

Incidence

Anatomy

Diagnosis

Pathophysiology and Natural History

Surgical Management of Systemic Venous Route Abnormalities

Surgical Management of Systemic Venous Destination Abnormalities

Results

CHAPTER 37 Sinus of Valsalva Aneurysm

Introduction

Epidemiology

Anatomy

Natural History

Diagnosis

Treatment

Results

CHAPTER 38 Coronary Artery Anomalies

Introduction

Anomalous Left Coronary Artery from Pulmonary Artery

Anomalous Aortic Origins of Coronary Arteries

Anomalous Pulmonary Origins of Coronary Arteries

Single Coronary Artery

Congenital Atresia/Stenosis of the Left Main Coronary Artery

Coronary Arteriovenous Fistulas

Intramyocardial Course of Coronary Arteries (Bridging)

Coronary Aneurysms

Coronary Aneurysms in Kawasaki Disease

CHAPTER 39 Cardiac Tumors

Historical Background

Nomenclature

Incidence

Clinical Appearance

Diagnostic Modalities

General Principles of Surgical Resection

General Considerations of Treatment Strategies

Primary Benign Cardiac Tumors

Primary Malignant Cardiac Tumors

Secondary Cardiac Tumors

Acknowledgments

CHAPTER 40 Diseases of the Pericardium

Historical Perspectives

Embryology and Anatomy

Diseases of the Pericardium

CHAPTER 41 Surgical Therapy of Cardiac Arrhythmias

Evolving Role of Surgical Therapy

Accessory Connection-Mediated Atrial Tachycardia

Macro Re-Entrant Atrial Tachycardia (Atrial Re-Entry Tachycardia, Atrial Flutter)

Atrioventricular Nodal Re-Entry Tachycardia

Focal (Automatic) Atrial Tachycardia

Atrial Fibrillation

Ventricular Tachycardia

Ventricular Tachycardia Associated with Ion Channelopathies

Prophylactic Arrhythmia Surgery

Pacemaker and Device Therapy

Pacemakers

Management of Acute Perioperative Arrhythmias

CHAPTER 42 Heart Transplantation

History of Heart Transplantation

Etiology and Indication for Heart Transplantation in Children

Management of Chronic Heart Failure

Management of Acute Heart Failure

Transplantation Evaluation

Surgical Technique

Postoperative Recovery

Long-Term Outcomes and Follow-Up

CHAPTER 43 Pediatric Lung and Heart-Lung Transplantation

Introduction

Historical Background

Indications for Transplantation

Contraindications to Transplantation

Timing of Referral

Allocation of Organs

Donor Selection

Anesthetic and Operative Considerations

Operative Technique

Postoperative Management

Outcome

Summary

CHAPTER 44 Infective Endocarditis

Definition

Diagnosis

Antimicrobial Therapy

Indications for Surgery

Timing of Surgery

Surgical Procedures

Results

Summary

CHAPTER 45 Pediatric Mechanical Circulatory Support

Introduction

Available Devices for Pediatric Mechanical Circulatory Support

Decision Matrix to Guide Device Selection for Pediatric Circulatory Support

The National Heart, Lung, and Blood Institute Pediatric Circulatory Support Program

Future Directions

Acknowledgment

CHAPTER 46 Adult Congenital Heart Disease

General Considerations

Surgical Considerations in Patients with Previous Operations

Anesthesia Considerations

Postoperative Care

Specific Congenital Heart Defects

Supplemental Images

Index

This edition first published 2013, © 2013 by Blackwell Publishing Ltd

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Library of Congress Cataloging-in-Publication Data

Pediatric cardiac surgery. – 4th ed. / edited by Constantine Mavroudis and Carl L. Backer.

p. ; cm.

 Includes bibliographical references and index.

 ISBN-13: 978-1-4051-9652-9 (hard cover : alk. paper)

 ISBN-10: 1-4051-9652-1

 I. Mavroudis, Constantine. II. Backer, Carl.

 [DNLM: 1. Cardiac Surgical Procedures. 2. Heart Defects, Congenital–surgery. 3. Child. 4. Infant. WS 290]

 LC classification not assigned

 617.4'12059–dc23

2011030347

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

Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books.

Cover images: From left to right: courtesy of Rachid F. Idriss, courtesy of C. Mavroudis, iStock © John Barnett, courtesy of C. Backer, courtesy of C. Backer.

Cover design by Fortiori Design

List of Contributors

Robert H. Anderson BSc, MD, FRCPathEmeritus ProfessorUniversity CollegeLondon, UKVisiting ProfessorUniversity of NewcastleNewcastle, UKVisiting Professor of PediatricsMedical University of South CarolinaCharleston, SC, USA

Janine Arruda, MDStaff, Pediatric CardiologyCenter for Pediatric and Congenital Heart DiseaseCleveland Clinic Children’s HospitalCleveland, OH, USA

Carl L. Backer, MDDivision Head, Cardiovascular-Thoracic SurgeryAnn & Robert H. Lurie Children’s Hospital of Chicago, formerly Children’s Memorial HospitalProfessor of SurgeryDepartment of SurgeryNorthwestern University Feinberg School of MedicineChicago, IL, USA

Nancy Benson, MSNCenter for Congenital Heart SurgeryCleveland Clinic Children’s HospitalCleveland, OH, USA

Darryl H. Berkowitz, MDAttending PhysicianSociety Hill Anesthesia ConsultantsPennsylvania HospitalPhiladelphia, PA, USA

Pierre-Luc Bernier, MD, FRCSCAttending SurgeonDivision of Cardiovascular SurgeryThe Montreal Children’s HospitalMcGill UniversityMontreal, QC, Canada

Edward L. Bove, MDHelen and Marvin Kirsh Professor of SurgeryChair, Department of Cardiac SurgeryC.S. Mott Children’s Hospital at the University of MichiganAnn Arbor, MI, USA

John W. Brown, MDHarris B. Shumacker Professor Emeritus of SurgeryIndiana University School of MedicineIndianapolis, IN, USA

Morgan L. Brown, MDResearch FellowCardiovascular SurgeryMayo ClinicRochester, MN, USA

Christopher A. Caldarone, MDProfessor and Chair, Division of Cardiac SurgeryUniversity of TorontoStaff Cardiovascular SurgeonLabatt Family Heart CenterThe Hospital for Sick ChildrenToronto, ON, Canada

Paul J. Chai, MDAssociate Medical DirectorAll Children’s Hospital Heart CenterThoracic and Cardiovascular SurgeonJohns Hopkins Children’s Heart Surgery, All Children’s HospitalSaint Petersburg, FL, USA

John P. Cheatham, MD, FAAP, FACC, FSCAIGeorge H. Dunlap Endowed Chair in Interventional CardiologyDirector, Cardiac Catheterization & Interventional TherapyCo-Director, The Heart CenterNationwide Children’s HospitalProfessor, Pediatrics & Internal Medicine, Cardiology DivisionThe Ohio State UniversityColumbus, OH, USA

David R. Clarke, MDClinical Professor of Cardiothoracic SurgerySchool of MedicineUniversity of Colorado DenverAurora, CO, USA

John M. Costello, MD, MPHMedical Director, Regenstein Cardiac Care UnitAnn & Robert H. Lurie Children’s Hospital of Chicago, formerly Children’s Memorial HospitalAssociate Professor of PediatricsNorthwestern University Feinberg School of MedicineChicago, IL, USA

Lara Danziger-Isakov, MD, MPHStaff, Center for Pediatric Infectious DiseasesDirector for Pediatric Clinical ResearchCleveland Clinic Children’s HospitalAssistant Professor of PediatricsCleveland Clinic Lerner College of Medicine of Case Western Reserve UniversityCleveland, OH, USA

Jeffrey R. Darst, MDAssistant Professor, PediatricsSection of CardiologyChildren’s Hospital ColoradoAurora, CO, USA

Denise Davis, RN, MSN, CPNP-AC/PCCenter for Congenital Heart SurgeryCleveland Clinic Children’s HospitalCleveland, OH, USA

Barbara J. Deal, MDDivision Head, CardiologyGetz Professor of CardiologyAnn & Robert H. Lurie Children’s Hospital of Chicago, formerly Children’s Memorial HospitalProfessor of PediatricsNorthwestern University Feinberg School of MedicineChicago, IL, USA

Joseph A. Dearani, MDProfessor of SurgeryCardiovascular SurgeryMayo ClinicRochester, MN, USA

Danny Del Duca, MDResident, Cardiac SurgeryMcGill UniversityMontreal, QC, Canada

Ralph E. Delius, MDVice Chief, Department of Cardiovascular SurgeryChildren’s Hospital of MichiganAssociate Professor of SurgeryWayne State University School of MedicineDetroit, MI, USA

Eric J. Devaney, MDAssociate Professor of SurgeryUniversity of California, San DiegoRady Children’s Hospital at the University of CaliforniaSan Diego, CA, USA

Konstantinos Dimopoulos, MD, MSc, PhD, FESCConsultant CardiologistAdult Congenital Heart Centre and Centre for Pulmonary HypertensionRoyal Brompton HospitalNational Heart & Lung InstituteImperial College LondonLondon, UK

Ali Dodge-Khatami, MD, PhDChief of Pediatric Cardiac SurgeryProgram Head for Congenital Heart DiseaseUniversity Heart CenterProfessor of Cardiac SurgeryUniversity of Hamburg-Eppendorf School of MedicineHamburg, Germany

Brian W. Duncan, MD, MBABioEnterpriseArboretum VenturesCleveland, OH, USA

Osama Eltayeb, MDFellow in Pediatric Cardiothoracic SurgeryIndiana University School of MedicineIndianapolis, IN, USA

Charles D. Fraser Jr, MDChief of Congenital Heart Surgery and Cardiac Surgeon In-ChargeTexas Children’s HospitalBaylor College of MedicineHouston, TX, USA

Stephanie Fuller, MDAssistant ProfessorDivision of Cardiothoracic SurgeryThe Children’s Hospital of PhiladelphiaUniversity of Pennsylvania School of MedicinePhiladelphia, PA, USA

Mark Galantowicz, MDChief, Department of Cardiothoracic SurgeryMurray D. Lincoln Chair of Cardiothoracic SurgeryCo-Director, The Heart CenterNationwide Children’s HospitalProfessor of SurgeryThe Ohio State UniversityColumbus, OH, USA

Michael A. Gatzoulis, MD, PhD, FACC, FESCProfessor of Cardiology, Congenital Heart Disease and Consultant CardiologistAdult Congenital Heart Centre and Centre for Pulmonary HypertensionRoyal Brompton HospitalNational Heart & Lung InstituteImperial College LondonLondon, UK

J. William Gaynor, MDAssociate Professor of SurgeryThe Children’s Hospital of PhiladelphiaPhiladelphia, PA, USA

Peter J. Gruber, MD, PhDD. Rees and Eleanor T. Jenson Presidential Chair in SurgeryInvestigator, University of UtahMolecular Medicine (U2M2) ProgramEccles Institute of Human GeneticsChief, Section of Pediatric Cardiothoracic SurgeryPrimary Children’s Medical CenterSalt Lake City, UT, USA

Thomas Günther, MDDepartment of Cardiovascular SurgeryGerman Heart CenterTechnische Universität MünchenMunich, Germany

Frank L. Hanley, MDProfessor of Cardiothoracic Surgery – Pediatric Cardiac SurgeryDirector, Children’s Heart CenterLucile Packard Children’s HospitalStanford University Medical CenterFalk CV Research CenterStanford, CA, USA

Jeffrey S. Heinle, MDSurgical Director, Heart and Lung Transplant ProgramTexas Children’s HospitalAssociate Professor of Surgery and PediatricsBaylor College of MedicineHouston, TX, USA

Samantha Hill, MD, MSc, FRCSCResident, Cardiac SurgeryMcGill UniversityMontreal, QC, Canada

Sharon L. Hill, MSN, ACNP-BCInterventional Nurse PractitionerThe Heart Center, Nationwide Children’s HospitalCollege of NursingThe Ohio State UniversityColumbus, OH, USA

Jennifer C. Hirsch, MD, MSAssistant Professor, Department of Surgery and PediatricsSurgical Director, Pediatric Cardiac Intensive Care UnitDivision of Pediatric Cardiovascular SurgeryC.S. Mott Children’s Hospital at the University of Michigan School of MedicineAnn Arbor, MI, USA

Ralf J. Holzer, MD, MSc, FSCAIAssistant Director, Cardiac Catheterization & Interventional TherapyThe Heart Center, Nationwide Children’s HospitalAssistant Professor of PediatricsThe Ohio State UniversityColumbus, OH, USA

Fejeania Hunter, RN, BSN, CPNPCenter for Congenital Heart SurgeryCleveland Clinic Children’s HospitalCleveland, OH, USA

Jeffrey P. Jacobs, MD, FACS, FACC, FCCPSurgical Director of Heart Transplantation and Extracorporeal Life Support ProgramsAll Children’s Hospital Heart CenterThoracic and Cardiovascular SurgeonJohns Hopkins Children’s Heart Surgery, All Children’s HospitalClinical ProfessorDepartment of SurgeryUniversity of South Florida (USF)Saint Petersburg, FL, USA

Marshall L. Jacobs, MDCenter for Congenital Heart SurgeryCleveland Clinic Children’s HospitalCleveland, OH, USA

Richard A. Jonas, MDChief, Cardiac SurgeryCodirector, Children’s National Heart InstituteCohen Funger Professor of Cardiac SurgeryChildren’s National Medical CenterWashington, DC, USA

Jason M. Kane, MD, MSAssociate Professor of PediatricsNorthwestern University Feinberg School of MedicineDirector, Regenstein Cardiac Care UnitDivision of CardiologyAnn & Robert H. Lurie Children’s Hospital of Chicago, formerly Children’s Memorial HospitalChicago, IL, USA

John M. Karamichalis, MDClinical Instructor in SurgeryDivision of Pediatric Cardiothoracic SurgeryUniversity of California, San FranciscoSan Francisco, CA, USA

Sunjay Kaushal, PhD, MDAttending SurgeonUniversity of MarylandBaltimore, MD, USA

Ergin Kocyildirim, MDAssistant ProfessorDepartment of Cardiothoracic SurgeryMcGowan Institute for Regenerative MedicineUniversity of PittsburghPittsburgh, PA, USA

Steven W. Kubalak, PhDAssociate ProfessorDepartment of Regenerative Medicine and Cell BiologyCardiovascular Developmental Biology CenterMedical University of South CarolinaCharleston, SC, USA

John J. Lamberti, MDProfessor of SurgeryUniversity of California San DiegoEugene and Joyce Klein Director of the Heart InstituteChildren’s Hospital of San DiegoRady Children’s HospitalSan Diego, CA, USA

Rüdiger Lange, MD, PhDDirector Department of Cardiovascular SurgeryGerman Heart CenterTechnische Universität MünchenMunich, Germany

Richard Lorber, MDStaff, Pediatric CardiologyCenter for Pediatric and Congenital Heart DiseaseCleveland Clinic Children’s HospitalCleveland, OH, USA

Richard D. Mainwaring, MDClinical Associate Professor of SurgeryCardiothoracic Surgery–Pediatric Cardiac SurgeryStanford University School of MedicineStanford, CA, USA

George B. Mallory Jr, MDPediatric PulmonologistDirector, Pediatric Lung Transplantation ProgramTexas Children’s HospitalProfessor of PediatricsBaylor College of MedicineHouston, TX, USA

Constantine Mavroudis, MDDirector, Congenital Heart Institute, Florida Hospital for ChildrenSite Director, Johns Hopkins Children’s Heart Surgery, Florida Hospital for ChildrenOrlando, FL, USA

Max B. Mitchell, MDProfessor of SurgerySchool of MedicineUniversity of Colorado Denver Health Sciences CenterChildren’s Hospital Colorado Heart InstituteAurora, CO, USA

David L.S. Morales, MDCongenital Heart SurgeryDirector of Mechanical Circulatory SupportAssociate Professor of Surgery and PediatricsTexas Children’s HospitalBaylor College of MedicineHouston, TX, USA

Victor O. Morell, MDCodirector, University of Pittsburgh Medical Center Heart and Vascular InstituteVice Chair and Director of Cardiovascular ServicesDepartment of Cardiothoracic SurgeryMcGowan Institute for Regenerative MedicineChief, Pediatric Cardiothoracic SurgeryChildren’s Hospital of PittsburghAssociate Professor of SurgeryPittsburgh, PA, USA

Muhammad Ali Mumtaz, MDMedical Director and Chief, Children’s Cardiothoracic SurgeryChildren’s Hospital of the King’s DaughtersNorfolk, VA, USA

Ashok Muralidaran, MDClinical InstructorCardiothoracic Surgery–Pediatric Cardiac SurgeryStanford University School of MedicineStanford, CA, USA

Richard G. Ohye, MDAssociate Professor, Department of SurgeryDivision of Pediatric Cardiovascular SurgeryC.S. Mott Children’s Hospital at the University of Michigan School of MedicineAnn Arbor, MI, USA

Vincent F. Olshove, BS, CCP, CCT, FPPDirector, Cardiovascular PerfusionThe Heart Center, Nationwide Children’s HospitalClinical Instructor, Circulation TechnologyThe Ohio State UniversityColumbus, OH, USA

Noritaka Ota, MDCardiac SurgeonDepartment of Cardiovascular SurgeryMt. Fuji Shizuoka Children’s HospitalShizuoka, Japan

Mario Petrou, PhD, FRCSConsultant Cardiothoracic SurgeonDepartments of Thoracic Surgery and Adult Cardiac SurgeryRoyal Brompton HospitalLondon, UK

Alistair Phillips, MD, FACC, FACSSurgical Director, Pediatric Heart Transplantation and Mechanical Circulatory Support Surgical Director, Adult Congenital Heart Disease Associate Professor, Division of Pediatric SurgeryCincinnati Children’s Hospital Medical CenterCincinnati, OH, USA

Stamatia Prapa, MDClinical Research FellowImperial College LondonRoyal Brompton HospitalLondon, UK

Tamar J. Preminger, MDStaff, Pediatric CardiologyCenter for Pediatric and Congenital Heart DiseaseCleveland Clinic Children’s HospitalCleveland, OH, USA

Lourdes R. Prieto, MDDirector, Cardiac CatheterizationCenter for Pediatric and Congenital Heart DiseaseCleveland Clinic Children’s HospitalAssociate ProfessorCleveland Clinic Lerner College of Medicine of Case Western Reserve UniversityCleveland, OH, USA

H. Jay Przybylo, MDAttending AnesthesiologistDepartment of AnesthesiaAnn & Robert H. Lurie Children’s Hospital of Chicago, formerly Children’s Memorial HospitalAssociate Professor of AnesthesiologyNorthwestern University Feinberg School of MedicineChicago, IL, USA

Athar Qureshi, MDAssistant ProfessorCleveland Clinic Lerner College of Medicine of Case Western Reserve UniversityStaff, Center for Pediatric and Congenital Heart DiseaseCleveland Clinic Children’s HospitalCleveland, OH, USA

Vadiyala Mohan Reddy, MDAssociate Professor of Cardiothoracic Surgery and PediatricsChief, Division of Pediatric Cardiac SurgeryLucile Packard Children’s HospitalStanford UniversityStanford, CA, USA

Dewei Ren, MDCongenital Heart SurgeryTexas Children’s HospitalHouston, TX, USA

W. Steves Ring, MDProfessor, Department of Cardiovascular and Thoracic SurgeryUniversity of Texas Southwestern Medical CenterChildren’s Medical Center of DallasDallas, TX, USA

Mark D. Rodefeld, MDAssociate Professor of SurgeryIndiana University School of MedicineIndianapolis, IN, USA

Joseph W. Rossano, MDAssistant Professor of PediatricsTexas Children’s HospitalBaylor College of MedicineHouston, TX, USA

Mark Ruzmetov, MDFellow in Pediatric Cardiothoracic SurgeryIndiana University School of MedicineIndianapolis, IN, USA

Marcy L. Schwartz, MDStaff, Pediatric CardiologyCenter for Pediatric and Congenital Heart DiseaseCleveland Clinic Children’s HospitalCleveland, OH, USA

Darryl F. Shore, MD, FRCSConsultant Cardiac SurgeonAdult Congenital Heart CenterRoyal Brompton HospitalNational Heart & Lung InstituteImperial College LondonLondon, UK

Thomas L. Spray, MDChief, Division of Cardiothoracic SurgeryThe Children’s Hospital of PhiladelphiaProfessor of SurgeryUniversity of Pennsylvania School of MedicinePhiladelphia, PA, USA

Richard Sterba, MDStaff, Pediatric CardiologyCenter for Pediatric and Congenital Heart DiseaseCleveland Clinic Children’s HospitalCleveland, OH, USA

Robert D. Stewart, MD, MPHInterim ChairCenter for Congenital Heart SurgeryCleveland Clinic Children’s HospitalCleveland, OH, USA

Christo I. Tchervenkov, MD, FRCSC, FACSDirectorDivision of Cardiovascular SurgeryThe Montreal Children’s HospitalTony Dobell Professor of Pediatric SurgeryMcGill UniversityMontreal, QC, Canada

Kim Teknipp, MSNCenter for Congenital Heart SurgeryCleveland Clinic Children’s HospitalCleveland, OH, USA

Jamie Thomas, RN, MSN, CPNP-AC/PCPediatric Nurse PractitionerPediatric Intensive Care UnitCleveland Clinic Children’s HospitalCleveland, OH, USA

Mark W. Turrentine, MDProfessor of SurgeryIndiana University School of MedicineIndianapolis, IN, USA

Nicola Viola, MDConsultant Cardiac SurgeonSpecialist Services DivisionCardiovascular and Thoracic Care GroupSouthampton General HospitalSouthampton, UK

Henry L. Walters III, MDChief, Department of Cardiovascular SurgeryChildren’s Hospital of MichiganProfessor of SurgeryWayne State University School of MedicineDetroit, MI, USA

Andy Wessels, PhDProfessorCardiovascular Developmental Biology CenterRegenerative Medicine and Cell BiologyMedical University of South CarolinaCharleston, SC, USA

Preface

This is the 4th Edition of Pediatric Cardiac Surgery. The first edition was published by Arciniegas in 1985, was followed by the 2nd Edition in 1994, and the 3rd Edition in 2003 by the present editors. Timely updates are important for any textbook as scientific intellectual curiosity, sentinel discoveries, and technological improvements have progressed at lightning speed. Even cardiac embryology, a field thought to be constant and thoroughly studied, has emerged with new findings of a second heart field, detailed results of syndromic genomes, and the promise of new paradigms and ontologies. Our readership from around the world has included numerous colleagues comprised of surgeons, cardiologists, intensivists, anes­thesiologists, residents, students, perfusionists, and nurses. They have found the book to be well organized, easy to read, and to the point. We have preserved this format for the 4th Edition and added several chapters that have mirrored the directions and practice of pediatric and congenital heart surgery in the twenty-first century.

Several new chapters by new authors have highlighted the advances of congenital heart surgery. While this textbook emphasizes the pediatric nature of the specialty, it is clear that there are more adults with congenital heart disease living today than there are children with congenital heart disease. This is a testimony to the years of scientific and clinical research that have combined to improve the lot of these patients who now present with medical and surgical problems of their own. The new chapter on adult congenital heart disease reviews these very important issues and serves as an important contribution to the textbook. Several new and updated chapters, written by experts in their field, review advances that have been made in congenital heart surgery such as right ventricular to pulmonary artery conduits, arrhythmia surgery, double-outlet ventricles, and cardiac transplantation, among many others. In some cases, the same authors have updated their previous chapters. In others, new authors have been selected because of their demonstrated expertise.

The 4th Edition maintains its comprehensive coverage of the breadth of congenital heart surgery and related fields. Each chapter reviews the embryology, physical findings, diagnostic criteria, and therapeutic choices associated with each disease entity. State-of-the art technology and the latest in surgical techniques are discussed.

As in previous editions of Pediatric Cardiac Surgery, the figures have predominantly been illustrated by Rachid Idriss. His drawing techniques are legendary not only because of his artistic talents, but more so for his ability to see an operation in his mind’s eye and demonstrate with a few lines the important parts of the relative anatomy and reparative operation. Sutures are clear, pledgets are well placed, and structures are anatomically correct. Hidden intracardiac anatomy is displayed by ghost techniques that transform the image into a three-dimensional living characterization of reality. His contribution to this 4th Edition cannot be overstated.

This textbook is reflective of the cooperation, expertise, and altruism that the contributing authors have so generously shared with the readership. Simply stated, these chapters are a delight to read. We are sure that the reader will have the same experience. All royalties from the sale of this book will be contributed to the Thoracic Surgery Foundation for Research and Education for the purpose of supporting congenital heart surgery initiatives. We are greatly indebted to the editorial staff at Wiley-Blackwell for their support, in particular, Kate Newell. Important editorial contributions were made by Patricia Heraty and, in particular, Melanie Gevitz who was so instrumental in organizing the successful efforts associated with the 2nd and 3rd Editions. In the Orlando Office, we found a jewel, Allison Siegel, who took on this project with an impress­ive zeal, expertise, and commitment that are rarely found anywhere.

The concluding paragraph in this Preface is quoted from the Preface of the 3rd Edition because it stands as a timeless dedication to our loved ones and family members who have shown their devotion, calmed the children, and explained our absences on countless occasions without rancor, excuses, or disappointment. It reads, “Finally, as with all surgeons and physicians, our accomplishments are facilitated by the sacrifices made by our families. Every author who wrote a chapter for this textbook, no doubt, has loved ones who have contributed in one way or another to their creativity, stability, and industry. We thank each and every one of these wives, husbands, children, parents, and friends, including our own, which have had the patience, perseverance, and equanimity to stand by.”

Constantine Mavroudis, MDCarl L. Backer, MD

CHAPTER 1

Development of the Heart and Great Vessels

Peter J. Gruber,1 Andy Wessels,2 and Steven W. Kubalak2

1Primary Children’s Medical Center, Salt Lake City, UT, USA

2Medical University of South Carolina, Charleston, SC, USA

Introduction

Modern cardiac embryology combines molecular and cellular biologic techniques with traditional embryologic morphologic approaches during development. The limited descriptions of human cardiac development are necessarily supplemented by nonhuman models of cardiac development. Avian embryos have traditionally been favored experimental models because of the ease with which they can be observed and manipulated. More recently, the developing mouse has become the preferred model for studying cardiac development because of the strength of genetic and molecular investigative tools available in this species. Where possible, this chapter discusses how results in experimental animal models relate to human cardiac development. provides a simplified comparison of two widely utilized developmental schemes for developmental staging in chick and mouse embryos [1–7]. The comparison of multiple species provides an important platform for understanding the development of the human heart and the pathogenesis of human disease.