Gatzoulis: Cases in Adult Congenital Heart Disease (Expert Consult Series)

Training opportunities for congenital heart disease—the most common inborn defect—and in particular its adult aspect of care, remain scarce. This, sadly, is mirrored by major gaps in care provision globally. In the meantime, the number of adults with congenital heart disease (ACHD) continues to increase. Despite advances in our understanding and management of this growing patient cohort, much of the practice is still based on personal experiences and limited or uncontrolled data. Furthermore, the complexity of the various lesions and the multitude of challenges that ACHD patients face call for a multidisciplinary approach to care. This is indeed the essence of good ACHD practice and the very focus of this training/educational book. For cardiology trainees and those who are interested in the ACHD field who desire additional opportunities to expose themselves to this fascinating and evolving field, our book is a forum for such exposure.

We submit that each ACHD patient is very different from another because of the range of anatomy and physiology, the continuously evolving palliative or reparative procedures available, and the different life circumstances that adults encounter. While the list of cases herein is not exhaustive, we have chosen the case mix in such a way to give the reader a good balance and flavor on current topics and areas of debate in ACHD.

The reader will find that the cases demonstrate the realities of the ACHD field, including incomplete medical histories, challenging or even suboptimal imaging, difficult decision making, and even, at times, poor outcome. Each case represents real experiences from real patients. At times, we have supplemented or editorialized the cases to maximize their educational value. This includes the addition of imaging studies, sometimes from similar patients, that, although not necessary from a case management standpoint, were included to demonstrate or clarify teaching points made.

The book can be read as a stand-alone text. There is also a web-based electronic version to facilitate accessibility and add value from cine imaging that cannot be printed. The latter, we hope, will enhance its training and educational value; individual imaging modalities can be played alone, thus facilitating a teaching session on any topic, for example ECG interpretation or CMR.

The format and table of contents follows closely the table of contents of our original textbook, Diagnosis and Management of Adult Congenital Heart Disease. It was our intention to provide a companion book that would stimulate further reading and welcome newcomers in one of the most exciting and rewarding areas of cardiology and indeed of medicine.

-- Michael A. Gatzoulis, Gary D. Webb, Craig S. Broberg, Hideki Uemura

Key Features

• Features 85 cases encompassing a full range of congenital heart disease problems—from the simple to the complex—that provide a better understanding of these conditions from a real-life, clinical perspective.
• Presents examples of multiple imaging modalities (including chest radiography, echocardiography, CT, MR, and angiography) clearly depict the clinical manifestations of congenital defects and provide you with the best views available of these conditions.
• Offers guidance on the assessment of congenital heart disease during pregnancy equips you with essential knowledge in addressing the needs of this growing patient population.

Website Features

• Consult the book from any computer at home, in your office, or at any practice location.
• Instantly locate the answers to your clinical questions via a simple search query.
• Quickly find out more about any bibliographical citation by linking to its MEDLINE abstract.
• Videos: View video clips illustrating a variety of conditions.

Contents

SECTION ONE - Shunts

Part I - Atrial Septal Defects/Abnormal Pulmonary Venous Return

• Case 1 - Right Heart Enlargement of Uncertain Cause (Sinus Venosus Atrial Septal Defect)
• Case 2 - Rationale for Septal Defect Closure in the Elderly
• Case 3 - Patent Foramen Ovale with Transient Ischemic Attack
• Case 4 - Atrial Septal Defect with Cyanosis: The Hypoplastic Right Ventricle
• Case 5 - Partial Anomalous Pulmonary Venous Return
• Case 6 - Scimitar Syndrome

Part II - Ventricular Septal Defects

• Case 7 - Endocarditis in a Young Man
• Case 8 - Ventricular Septal Defect and the Aortic Valve
• Case 9 - Catheter Closure of Ventricular Septal Defects
• Case 10 - Straddling of the Tricuspid Valve: Long-Term Outcome

Part III - Atrioventricular Septal Defects

• Case 11 - Long-Term Follow-Up of Atrioventricular Septal Defect
• Case 12 - Outflow Tract Obstruction after Atrioventricular Septal Defect Repair
• Case 13 - Left Atrioventricular Valve Regurgitation: Criteria for Intervention
• Case 14 - Anticoagulation in a Pregnant Patient with a Mechanical Valve
• Case 15 - Atrial Septal Defect Associated with Pulmonary Hypertension—Cause and Effect

Part IV - Patent Ductus Arteriosus

• Case 16 - Catheter Closure of a Patent Ductus Arteriosus
• Case 17 - Erythrocytosis with Normal Oxygen Saturation

SECTION TWO - Left-Sided Lesions

Part I - Left Ventricular Inflow

• Case 18 - Cor Triatriatum

Part II - Left Ventricular Outflow

• Case 19 - Considerations for the Ross Procedure
• Case 20 - Aortic Stenosis and Endocarditis during Pregnancy
• Case 21 - Subaortic Stenosis: Indications for Surgery
• Case 22 - Pericardial Constriction after Relief of Subaortic Stenosis
• Case 23 - Ventricular Arrhythmia Following a Ross Procedure

Part III - Coarctation of the Aorta

• Case 24 - Acute Presentation with Cerebral Hemorrhage
• Case 25 - Recoarctation: Criteria for Intervention
• Case 26 - Pregnancy-Related Complications in Coarctation
• Case 27 - Extraanatomic Bypass Graft Repair of Coarctation
• Case 28 - Interrupted Aortic Arch in a Patient with DiGeorge Syndrome

SECTION THREE - Right-Sided Lesions

Part I - Right Ventricular Inflow

• Case 29 - Ebstein Anomaly and Wolff-Parkinson-White Syndrome
• Case 30 - Ebstein Anomaly and Sudden Cardiac Death
• Case 31 - Successful Tricuspid Valve Repair of Ebstein Anomaly
• Case 32 - Ebstein Anomaly and Prepregnancy Counseling
• Case 33 - Pregnancy and Fetal Death in a Patient with Ebstein Anomaly
• Case 34 - Cyanosis in Ebstein Anomaly and Catheter Closure of Atrial Septal Defect

Part II - Right Ventricular Outflow

• Case 35 - Congenital Pulmonary Stenosis Turned to Pulmonary Regurgitation
• Case 36 - Syncope in a Patient with Noonan Syndrome
• Case 37 - Dyspnea in a Patient with a Loud Murmur
• Case 38 - Arrhythmia and Syncope in a Patient with a Childhood Murmur
• Case 39 - Right Ventricular Outflow Obstruction in a Pregnant Woman

Part III - Tetralogy of Fallot

• Case 40 - Late Repair of Tetralogy of Fallot
• Case 41 - Timing of Pulmonary Valve Replacement
• Case 42 - Catheter Implantation of Stented Pulmonary Valve
• Case 43 - Management of Tachyarrhythmia in Tetralogy: Ablation versus Surgery
• Case 44 - Consideration for Automatic Implantable Cardioverter-Defibrillator in Tetralogy
• Case 45 - Aortopathy in Tetralogy of Fallot

Part IV - Pulmonary Atresia

• Case 46 - Pulmonary Atresia with Intact Ventricular Septum
• Case 47 - Multiple Aortopulmonary Collaterals: Too Many or Too Few
• Case 48 - Pacemaker Infection in a Cyanotic Patient

SECTION FOUR - Complex Congenital Heart Conditions

Part I - Transposition of Great Arteries

• Case 49 - Catheter Intervention for Baffle Leak or Venous Obstruction
• Case 50 - Transcatheter Options for Atrial Arrhythmia after the Mustard Procedure
• Case 51 - Considerations for Tricuspid Valve Replacement in Patients with a Systemic Right Ventricle
• Case 52 - Late Complications after the Arterial Switch Operation
• Case 53 - Long-Term Outcome after Rastelli Repair
• Case 54 - Pregnancy and the Systemic Right Ventricle
• Case 55 - Palliative Mustard for Transposition and Ventricular Septal Defect

Part II - Congenitally Corrected Transposition

• Case 56 - Assessment of Systemic Right Ventricle Function
• Case 57 - Management of Systemic Ventricular Failure
• Case 58 - Late Outcome Following Systemic Tricuspid Valve Replacement
• Case 59 - The Criss-Cross Heart
• Case 60 - Congenitally Corrected Transposition of the Great Arteries with Pulmonary Stenosis and Ventricular Septal Defect: When to Intervene?

Part III - The Fontan Circulation

• Case 61 - Intracardiac Thrombus in the Fontan Circulation
• Case 62 - Protein-Losing Enteropathy
• Case 63 - Fontan and Pregnancy
• Case 64 - Catheter Ablation for Atrial Arrhythmia
• Case 65 - Total Cavopulmonary Conversion: When and How
• Case 66 - Arrhythmia Intervention at the Time of Total Cavopulmonary Conversion
• Case 67 - Timing and Merits of Transplantation in a Fontan Patient
• Case 68 - Early Appraisal of Adults with Norwood Correction

Part IV - Other Single Ventricle Physiology

• Case 69 - Complications of Ventricular Septation in a Patient with a Single Ventricle
• Case 70 - Fever in a Patient with a Single Ventricle

Part V - Eisenmenger Syndrome

• Case 71 - Long-Term Survival in Eisenmenger Syndrome
• Case 72 - Iron Deficiency in Cyanotic Heart Disease
• Case 73 - Management of Acute Hemoptysis
• Case 74 - Pulmonary Artery Thrombosis and Recurrent Hemoptysis in Eisenmenger Syndrome
• Case 75 - Pregnant Patient with Unoperated Truncus Arteriosus

SECTION FIVE - Special Topics

Part I - Coronary Anomalies

• Case 76 - Left Coronary Artery Arising from the Right Coronary Sinus
• Case 77 - Anomalous Left Coronary Artery from the Pulmonary Artery
• Case 78 - Coronary Artery Fistulae and Their Significance
• Case 79 - Long-Term Management of Kawasaki Disease

Part II - Marfan

• Case 80 - Recurrent Aortic Dissection in Marfan Syndrome

Part III - Pulmonary Arterial Hypertension

• Case 81 - The Role of Pulmonary Vasodilators in Pulmonary Hypertension
• Case 82 - Pulmonary Hypertension after Repair of Congenital Diaphragmatic Hernia
• Case 83 - Contraception Counseling

Part IV - Isomerism

• Case 84 - Left Atrial Isomerism

Part V - Cardiac and Yet Noncardiac

• Case 85 - Liver Dysfunction after the Mustard Procedure

Appendix: Notes on Quantification

• Oxygen Saturation
• Ventricular Volumes And Normal Range
• Flow Quantification From Hemodynamic Studies

References

About the Authors

• Michael A. Gatzoulis, MD, PhD, FACC, FESC, Professor of Cardiology and Congenital Heart Disease; Consultant Cardiologist, Head, Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, National Heart and Lung Institute, Imperial College, London, United Kingdom.
• Gary D. Webb, MD, FRCPC, FACC, Professor of Medicine; Director of the Philadelphia Adult Congenital Heart Center, University of Pennsylvania School of Medicine, The Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania.
• Craig S. Broberg, MD, Assistant Professor, School of Medicine; Director, Adult Congenital Heart Disease Program, Oregon Health & Science University, Portland, Oregon.
• Hideki Uemura, MD, Consultant Cardiac Surgeon, Royal Brompton Hospital, London, United Kingdom.

Book Review

"Dr. Gatzoulis, Webb, Broberg and Uemura have been at the forefront of advances in care of adult congenital heart disease for many years and have edited or contributed to numerous textbooks for medical professionals. This new book, however, is the first to focus on specific cases. It includes 85 cases from first presentation to the latest follow-up, and discusses the specific challenges in each patient. By choosing to focus on individual cases, the editors fully explore all aspects of the congenital heart picture and illustrate the array of trajectories that comprise life with congenital heart disease. By creating Cases in Adult Congenital Heart Disease, the editors have created a unique and essential tool to help ensure that these patients find the care they seek. They guide the medical professional to a much richer understanding of the complexity one must master to help ensure that each of us, patient by patient, gets the life and health care we deserve."

-- Amy Verstappen, President and CEO, Adult Congenital Heart Association

Product Details

• Hardcover: 560 pages
• Publisher: Churchill Livingstone; 1 Har/Psc edition (2010)
• Language: English
• ISBN-10: 0443067120
• ISBN-13: 978-0443067129
• Product Dimensions: 11.1 x 8.7 x 1 inches

List Price: $186.00