Diagnosis and Management of Hypertrophic Cardiomyopathy

Diagnosis and Management of Hypertrophic Cardiomyopathy is a unique, multi-authored compendium of information regarding the complexities of clinical and genetic diagnosis, natural history, and management of hypertrophic cardiomyopathy (HCM)—the most common and important of the genetic cardiovascular diseases—as well as related issues impacting the health of trained athletes. Edited by Dr. Barry J. Maron, a world authority on HCM, and with major contributions from all of the international experts in this field, this book provides a single comprehensive source of information concerning HCM. Recent advances in the field are discussed, including the importance of left ventricular outflow tract obstruction, the use of implantable defibrillators for the prevention of sudden death in young people, definition of the genetic basis for HCM and its role in clinical diagnosis and risk stratification, the development of more precise strategies for assessing the level of risk for sudden death among all patients with HCM, and the evolution of invasive interventions for heart failure symptoms, such as surgical management and its alternatives (alcohol septal ablation and dual-chamber pacing). Key Features: Contributions from all experts in the field, representing diverse viewpoints regarding this heterogeneous disease and related issues in athletes Information to dispel misunderstandings regarding issues associated with HCM and cardiovascular disease in athletes The only comprehensive source of information available on the topic

Foreword, ix Pravin M. Shah, MD Dedication and Acknowledgments, xi List of Contributors, xiii 1 Phenotypic Expression and Clinical Course of Hypertrophic Cardiomyopathy, 1 Barry J. Maron, MD 2 Genetic Mutations that Remodel the Heart in Hypertrophic Cardiomyopathy, 37 Carolyn Y. Ho, MD and Christine E. Seidman, MD 3 Genetic Basis and Genotype–Phenotype Relationships in Familial Hypertrophic Cardiomyopathy, 67 Albert A. Hagège, MD, PhD, Ketty Schwartz, PhD, Michel Desnos, MD, and Lucie Carrier, PhD 4 Historical Perspective, Mechanism, and Clinical Significance of Left Ventricular Outflow Tract Obstruction in Hypertrophic Cardiomyopathy, 81 Martin S. Maron, MD, Iacopo Olivotto, MD, and Barry J. Maron, MD 5 Hypertrophic Cardiomyopathy with Latent (Provocable) Obstruction: Pathophysiology and Management, 95 E. Douglas Wigle, MD, Maria Eriksson, MD, PhD, Paul Rakowski, David Focsaneanu, Cairrine Sloggett, RN, Anna Woo, MD, and Harry Rakowski, MD 6 Pathophysiology and Clinical Consequences of Atrial Fibrillation in Hypertrophic Cardiomyopathy, 105 Iacopo Olivotto, MD, Barry J. Maron, MD, and Franco Cecchi, MD 7 Other Modes of Disability or Death Including Stroke, and Treatment Strategies, in Hypertrophic Cardiomyopathy, 121 Franco Cecchi, MD, Iacopo Olivotto, MD, and Barry J. Maron, MD 8 Disturbed Vascular Control in Hypertrophic Cardiomyopathy: Mechanisms and Clinical Significance, 132 Ross Campbell, BSc, Jayne A. Morris-Thurgood, PhD, and Michael P. Frenneaux, FRCP, FRACP 9 Clinical Significance of Diastolic Dysfunction and the Effect of Therapeutic Interventions, 147 Sandro Betocchi, MD and Raffaella Lombardi, MD 10 Value of Exercise Testing in Assessing Clinical State and Prognosis in Hypertrophic Cardiomyopathy, 158 Sanjay Sharma, MRCP, MD 11 Pathophysiology and Significance of Myocardial Ischemia in Hypertrophic Cardiomyopathy, 172 Rajesh Thaman, MBBS, MRCP, Bhavesh Sachdev, MBBS, MRCP, and Perry M. Elliott, MBBS, MD, MRCP 12 Hypertrophic Cardiomyopathy in Japan: Clinical, Morphologic and Genetic Expression, 185 Yoshinori Doi, MD, PhD, Hiroaki Kitaoka, MD, Nobuhiko Hitomi, MD, Naohito Yamasaki, MD, Yoshihisa Matsumura, MD, Takashi Furuno, MD, and Barry J. Maron, MD 13 Prevalence, Prevention and Treatment of Infective Endocarditis in Hypertrophic Cardiomyopathy, 195 Paolo Spirito, MD, Marco Piccininno, MD, and Camillo Autore, MD 14 Pharmacologic Treatment of Symptomatic Hypertrophic Cardiomyopathy, 200 Mark V. Sherrid, MD and Ivan Barac, MD 15 Obstructive Hypertrophic Cardiomyopathy: Results of Septal Myectomy, 220 Joseph A. Dearani, MD and Gordon K. Danielson, MD 16 United States Perspectives on the Role of Dual-Chamber Pacing in Patients with Hypertrophic Cardiomyopathy, 236 Paul Sorajja, MD, Steve R. Ommen, MD, and Rick A. Nishimura, MD 17 Dual-Chamber Pacing for Hypertrophic Obstructive Cardiomyopathy, 246 Xavier Jeanrenaud, MD and Lukas Kappenberger, MD 18 Alcohol Septal Ablation, 259 Hubert Seggewiss, MD, Angelos Rigopoulos, MD, Lothar Faber, MD, and Peer Ziemssen, MD 19 Alcohol Septal Ablation in the Treatment of Hypertrophic Obstructive Cardiomyopathy: A Seven-Year Experience, 279 Horst Kuhn, MD, Thorsten Lawrenz, MD, Frank Lieder, MD, Frank H. Gietzen, MD, Ludger Obergassel, MD, Claudia Strunk-Muller, MD, Berit Stolle, MD, and Christian H. Leuner, MD 20 Role of Septal Ablation in a Surgical Center, 297 Harry M. Lever, MD 21 Molecular and Clinical Tools for Sudden Death Risk Assessment in Hypertrophic Cardiomyopathy, 307 Asifa Quraishi, MB, MRCP, Mohammad S. Hamid, MB, MRCP, and William J. McKenna, MD, FRCP 22 Risk Stratification For Sudden Death in Hypertrophic Cardiomyopathy: Extreme Left Ventricular Hyptertrophy as a New Indicator of Risk, 319 Paolo Spirito, MD and Barry J. Maron, MD 23 Implantable Defibrillator for Prevention of Sudden Death in Hypertrophic Cardiomyopathy, 327 Barry J. Maron, MD, Win-Kuang Shen, MD, and Paolo Spirito, MD 24 Hypertrophic Cardiomyopathy and Other Causes of Sudden Death in the Trained Athlete: An Electrophysiologist Perspective on the Management of Benign and Not So Benign Arrhythmias, 345 N. A. Mark Estes III, MD, Paul J. Wang, MD, Munther K. Homoud, MD, and Mark S. Link, MD 25 The Athlete's Heart, ECG, and Differential Diagnosis with Hypertrophic Cardiomyopathy and Other Cardiomyopathies, 367 Antonio Pelliccia, MD and Barry J. Maron, MD 26 Importance of Congenital Coronary Artery Anomalies, 382 Cristina Basso, MD, PhD, Domenico Corrado, MD, and Gaetano Thiene, MD 27 Arrhythmogenic Right Ventricular Cardiomyopathy and Hypertrophic Cardiomyopathy: Identification with the Italian Preparticipation Athlete Screening Program, 393 Domenico Corrado, MD, Cristina Basso, MD, PhD, Maurizio Schiavon, MD, and Gaetano Thiene, MD 28 Cardiovascular Causes of Sudden Death, Preparticipation Screening, and Criteria for Disqualification in Young Athletes, 404 Barry J. Maron, MD 29 Sudden Death Due to Chest Blows (Commotio Cordis), 432 Mark S. Link, MD, N. A. Mark Estes III, MD, and Barry J. Maron, MD 30 Naturally Occurring Animal Models of Cardiovascular Disease Causing Premature Death, 448 Philip R. Fox, DVM 31 The Role of the Internet and Patient Support Groups for Those Living with Hypertrophic Cardiomyopathy, 473 Lisa Salberg Afterword, 481 Robert Jon Pensack, MD Index, 487