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Diagnosis and Management of Hypertrophic Cardiomyopathy

Diagnosis and Management of Hypertrophic Cardiomyopathy

Barry J. Maron (Editor)

ISBN: 978-0-470-98746-9

Oct 2007, Wiley-Blackwell

528 pages


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
ForewordPravin Shah, MD.

Dedication and Acknowledgments.

List of Contributors.

1. Phenotypic Expression and Clinical Course of Hypertrophic Cardiomyopathy.

Barry J. Maron, MD2. Genetic Mutations that Remodel the Heart Carolyn Y. Ho, MD and Christine E. Seidman, MD.

3. Genetic Basis and Genotype-Phenotype Relationships in Familial Hypertrophic Cardiomyopathy.

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 CardiomyopathyMartin S. Maron, MD, Iacopo Olivotto, MD, and Barry J. Maron, MD.

5. Hypertrophic Cardiomyopathy with Latent (Provocable) Obstruction: Pathophysiology and Management.

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.

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 CardiomyopathyFranco Cecchi, MD, Iacopo Olivotto, MD, and Barry J. Maron, MD.

8. Disturbed Vascular Control in Hypertrophic Cardiomyopathy: Mechanisms and Clinical Significance.

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.

Sandro Betocchi, MD and Raffaella Lombardi, MD.

10. Value of Exercise Testing in Assessing Clinical State and Prognosis in Hypertrophic Cardiomyopathy.

Sanjay Sharma, MRCP, MD.

11. Pathophysiology and Significance of Myocardial Ischemia in Hypertrophic Cardiomyopathy.

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.

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 CardiomyopathyPaolo Spirito, MD, Marco Piccininno, MD, and Camillo Autore, MD.

14. Pharmacologic Treatment of Symptomatic Hypertrophic CardiomyopathyMark V. Sherrid, MD and Ivan Barac, MD.

15. Obstructive Hypertrophic Cardiomyopathy: Results of Septal Myectomy Gordon K. Danielson, MD and Joseph A. Dearani, MD.

16. US Perspectives on the Role of Dual-Chamber Pacing in Patients with Hypertrophic CardiomyopathyPaul Sorajja, MD, Steve R. Ommen, MD, and Rick A. Nishimura, MD.

17. Dual-Chamber Pacing for Hypertrophic Obstructive Cardiomyopathy Xavier Jeanrenaud, MD, and Lukas Kappenberger, MD.

18. Alcohol Septal Ablation Hubert Seggewiss, MD Prof. 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.

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 CenterHarry M. Lever, MD.

21. Molecular and Clinical Tools for Sudden Death Risk Assessment in Hypertrophic Cardiomyopathy.

Asifa Quraishi MB, MRCP, Mohammad S. Hamid MB, MRCP, and William J. McKenna MD, FRCP.

21A. In Memorium: Asifa Quraishi.

22. Risk Stratification For Sudden Death in Hypertrophic Cardiomyopathy: Extreme Left Ventricular Hyptertrophy as a New Indicator of Risk.

Paolo Spirito, MD and Barry J. Maron, MD.

23. Implantable Defibrillator for Prevention of Sudden Death in Hypertrophic Cardiomyopathy.

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.

N.A. Mark Estes III, MD, Paul J. Wang, MD, Munther K. Homoud, MD, and Mark S. Link, MD.

25. The Athlete’s Heart, the Athlete’s ECG, and Differential Diagnosis with HCM and Other Cardiomyopathies.

Antonio Pelliccia, MD and Barry J. Maron, MD.

26. Importance of Congenital Coronary Artery AnomaliesCristina 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.

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 AthletesBarry J. Maron, MD.

29.Sudden Death Due to Chest Blows (Commotio Cordis).

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.

Philip R. Fox, DVM.

31. The Role of the Internet and Patient Support Groups for Those Living with Hypertrophic Cardiomyopathy.

Lisa Salberg.

AfterwordRobert Jon Pensack, MD.


  • Provides a comprehensive presentation including clinical and genetic aspects of the disease, natural history, and new information regarding risk stratification for and prevention of sudden death
  • An outstanding state of the art clinical monograph has been compiled by the major international authorities to provide a practical summary of the ever-changing disease spectrum of hypertrophic cardiomyopathy
  • This book will serve as an important reference document for many years to come