Hypertrophic cardiomyopathy: The first century 1869–1969

Brigham and Women’s Hospital, Harvard University, Boston MA, USA *Email: [email protected] Thank you so much, Professor Yacoub. It’s a great honor for me to participate in this conference. I thank you for your vision and your tremendous energy and for everything that you have and are and will accomplish in cardiovascular surgery and science. I’m going to talk about the first century of hypertrophic cardiomyopathy (HCM) and will emphasize my experience during the last decades of this century. Few people realize that the first patient with HCM was described in 1869, during the reign of Queen Victoria. The great physician, William Osler, was four years old. Winston Churchill had not yet been born. The electrocardiogram and cardiac catheterization were 35 and 60 years in the future, respectively. But, in the Medical Gazette of Paris, a paper appeared, which was entitled Cardiac Sub-Aortic Stenosis by Henri Liouville [1] and which described a 75-year-old woman who developed worsening dyspnea over several days. On physical examination, she had a systolic heart murmur. She died shortly after presentation. An autopsy revealed the following: ‘‘The left ventricle is enlarged and very thick. It has considerable concentric hypertrophy’’—which means that Liouville understood the difference between concentric and eccentric hypertrophy— ‘‘measuring 3.5 to 4 centimeters in width. When I insert my index finger from the ventricle toward the aortic outflow tract, my finger becomes tightly pinched in the myocardium, one centimeter below the aortic valve. The aortic valve itself does not appear to be stenosed or calcified. When I try to insert my thumb backward through the aortic valve toward the ventricle, it cannot reach my index finger that I have inserted from the opposite direction. This is due to the obstruction that is caused by the myocardial thickening that is situated below the level of the aortic valve. There is no evidence of aortic insufficiency by water testing.’’ I think that this case report is incredible for the time. This physician examined the patient, recognized and timed the murmur, and after her death described carefully the pathology that all of us now recognize as HCM. Now, I call your attention to a paper published in the Deutsche Med. Wochenschrift in 1907 [2]. The paper is about left-sided muscular outflow tract obstruction by a pathologist, Dr. A. Schmincke. He described two hearts, both of which came from women in their mid fifties. They both had left ventricular hypertrophy. Decades before the development of left heart catheterization, Schmincke described a vicious circle as follows: ‘‘Diffuse muscular hypertrophy of the left ventricle outflow tract causes an obstruction. The left ventricle has to work harder to overcome the obstruction. So, the primary hypertrophy will be accompanied by a secondary hypertrophy causing an incremental (further) narrowing of the outflow tract.’’ He clearly understood that hypertrophy caused obstruction to left ventricular outflow which in turn caused more hypertrophy. Now that I have mentioned these two remarkable very early papers, I want to turn to the more recent observations which have led to our current understanding of the clinical and hemodynamic features of HCM. There are six major components.