Recognition of Hypertrophic Cardiomyopathy as a Contemporary, Relatively Common, and Treatable Disease (from the International Summit V)

From September 27 to 29, 2013, the Fifth International Summit on Hypertrophic Cardiomyopathy was held in Minneapolis, Minnesota, sponsored by the Minneapolis Heart Institute Foundation, with the relevant title, “ Hypertrophic Cardiomyopathy: A Contemporary and Treatable Disease .” The theme of the conference was intentionally selected to recognize the maturation of this complex disease within the cardiovascular community, particularly the evolution of diagnostic and management strategies. An international faculty of 31 was assembled representing the most experienced, respected, and dedicated experts in hypertrophic cardiomyopathy (HCM), all of whom have played major roles in defining this disease. The faculty presented and discussed the emerging principles of HCM for 250 attendees from 17 countries and 27 states over 2.5 days ( Table 1 ; Figures 1 to 3 ).

Table 1

Hypertrophic Cardiomyopathy Summit V

Director: Barry J. Maron, MD
Coordinator: Hayley Matthews-Jones

Summit faculty Location
Barry J. Maron, MD Minneapolis, Minnesota
Eugene Braunwald, MD Boston, Massachusetts
Martin S. Maron, MD Boston, Massachusetts
Berglind Adalsteinsdottir, MD Akureyri, Iceland
Michael J. Ackerman, MD, PhD Rochester, Minnesota
Cristina Basso, MD Padua, Italy
Franco Cecchi, MD Florence, Italy
Joseph A. Dearani, MD Rochester, Minnesota
Robert J. Desnick, MD, PhD New York, New York
N.A. Mark Estes, III, MD Boston, Massachusetts
David Feldman, MD, PhD Minneapolis, Minnesota
Philip R. Fox, DVM New York, New York
Gunnar Thor Gunnarsson, MD, PhD Akureyri, Iceland
Robert G. Hauser, MD Minneapolis, Minnesota
Carolyn Y. Ho, MD Boston, Massachusetts
Carey D. Kimmelstiel, MD Boston, Massachusetts
Harry Lever, MD Cleveland, Ohio
Mark S. Link, MD Boston, Massachusetts
Iacopo Olivotto, MD Florence, Italy
Steve R. Ommen, MD Rochester, Minnesota
Antonio Pelliccia, MD Rome, Italy
Lisa Salberg Rockaway, New Jersey
Christine E. Seidman, MD Boston, Massachusetts
Christopher Semsarian, MD, PhD Sydney, Australia
Mark V. Sherrid, MD New York, New York
Paolo Spirito, MD Genoa, Italy
Folkert J. ten Cate, MD, PhD Rotterdam, the Netherlands
Gaetano Thiene, MD Padua, Italy
Jeffrey A. Towbin, MD Cincinnati, Ohio
Sami Viskin, MD Tel Aviv, Israel
Anna Woo, MD Toronto, Ontario, Canada
Notable guests
Sue Casey, RN Minneapolis, Minnesota
Jackie Dosmann Minneapolis, Minnesota
Tammy Haas, RN Minneapolis, Minnesota
Vicki Pink, RN Minneapolis, Minnesota
Joseph Murgo, MD San Antonio, Texas
Charles McIntosh, MD, PhD Washington, DC
Jodie Ingles, PhD Sydney, Australia
Donna Maron, MSW Minneapolis, Minnesota

Figure 1

Some of the Summit faculty. (A) Barry Maron, course director: Basic principles, clinical course, and reduction in mortality with prevention of sudden death. (B) Eugene Braunwald: How to “discover” a new disease. (C) Martin Maron: Outflow obstruction; CMR for defining morphology and predicting sudden death risk. (D) Iacopo Olivotto: Atrial fibrillation; end-stage heart failure. (E) Robert Hauser: The ICD leads … and recalls. (F) Chris Semsarian: Clinical implications and limitations of genetic testing. (G) Gaetano Thiene: Pathology of hypertrophic cardiomyopathy. (H) Folkert ten Cate: Myectomy and alcohol ablation in Europe. (I) Franco Cecchi: Clinical implications of myocardial ischemia (Fondazione Menarini).

Figure 2

Eugene Braunwald at the International Hypertrophic Cardiomyopathy Summit V. (A) Luncheon talk describing his experiences in the “early years” of HCM. (B) With Barry Maron and Martin Maron. (C) Engaged in conversation with Barry Maron. (D) With Iacopo Olivotto (far right) and his large group of colleagues from the HCM Center in Florence, Italy (Fondazione Menarini).

Figure 3

Minneapolis at night: from the observation deck of the IDS Center, site of the dinner reception for registrants and faculty at Windows on Minnesota. (A) The Minneapolis skyline. (B) View of Target Field during a major league baseball game.

The priority was education and achieving an understanding of HCM necessary to benefit the patient population, while at the same time avoiding unnecessary controversy for a disease too often fraught with misunderstanding and confusion. The Summit was the place to hear the whole “hypertrophic cardiomyopathy story” in an intense but interactive format.

Furthermore, it was very appropriate for Dr. Eugene Braunwald to be present at the Summit and to lecture and interact ( Figure 2 ), for he, more than any other investigator, is responsible for the initial seminal description of HCM in the 1960s at the National Institutes of Health (Bethesda, Maryland).

This editorial serves as a primer for underscoring the many important advances in HCM and its changing epidemiology and clinical profile, particularly in the years since the last Summit in 2009. It was also notable and quite sad to recognize the passing of Dr. Douglas Wigle at age 85 years after a long illness. Dr. Wigle was a pioneer, scholar, and innovator in HCM, as well as a true gentleman.


HCM is a global disease known to affect patients in well over 50 countries but very similar in presentation throughout the world, and likely the most common genetic heart disease. Several diverse population studies report a frequency in the general population of about 1:500 ( Figure 4 ; Table 2 ). Because these data are based on probands presenting with phenotypically expressed disease (i.e., left ventricular hypertrophy), the overall population burden of HCM is probably substantially underestimated, given the large numbers of clinically unrecognized patients and family members who are genetically affected without overt evidence of the disease.

Dec 5, 2016 | Posted by in CARDIOLOGY | Comments Off on Recognition of Hypertrophic Cardiomyopathy as a Contemporary, Relatively Common, and Treatable Disease (from the International Summit V)

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