Centennial Collection: Hypertrophic Cardiomyopathy

In 1958, pathologist Donald Teare described 8 sudden cardiac death victims in whom post-mortem examination showed asymmetric hypertrophy of the intraventricular septum with "bizarre" arrangement of muscle bundles. 1 Over the next 6 years, hemodynamic studies described the physiology of left ventricular outflow tract obstruction and its exacerbation by decreasing preload or afterload, or increasing contractility.These observations led Drs.Braunwald, Lambrew and Morrow to name the condition "Idiopathic Hypertrophic Subaortic Stenosis or IHSS" in a case series of 64 patients published in 1964, followed closely by a report of the first 10 septal myectomies performed by Dr. Morrow at NIH.The next major milestone came in 1980 when one of the first 3 patients in the world to receive an automatic implantable defibrillator by Drs.Mirowski, Weisfeldt and colleagues at Johns Hopkins was a young man with "IHSS" who had survived 2 cardiac arrests (Figure 1A).Although the name went through various iterations over the ensuing decades before arriving at hypertrophy cardiomyopathy (HCM), these two groundbreaking interventions remain a cornerstone of care for patients with HCM who have medically refractory left ventricular outflow tract obstruction or who are at high risk for sudden cardiac death respectively.