Predicting the Future in Hypertrophic Cardiomyopathy
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Article, see p 1015
It’s tough to make predictions, especially about the future.
A 22-year-old man was initially diagnosed with hypertrophic cardiomyopathy (HCM) at 19 years of age during longitudinal family screening. He had been fit, active, and without cardiovascular symptoms or limitations. There was no family history of sudden death. He completed 18 minutes on a standard Bruce exercise test with normal heart rate and blood pressure response. Maximal left ventricular wall thickness was 17 mm; there was no resting or exercise-provoked obstruction; the left atrial size was normal; and no ventricular ectopy was identified on ambulatory monitoring. Three years after the initial diagnosis, without change in any clinical features, he had a cardiac arrest while walking to the subway.
Sudden death events in patients with HCM are rare, stochastic, and typically dissociated from premonitory changes in cardiac symptomatology, function, or morphology. Traditional risk predictors have low individual sensitivity and specificity. As a result, identifying patients who are at an increased risk for sudden cardiac death (SCD) remains a major clinical challenge. The stakes are high because, although implantable cardioverter-defibrillators (ICDs) can largely prevent SCD, they do not improve quality of life, and the long-term risks are real. The cumulative burden of infections, lead fractures, inappropriate shocks, and other complications will be particularly experienced by patients with HCM, who are usually younger than are other patients receiving ICDs and thus will potentially live with their devices for decades.
Large cohorts and iterative randomized controlled trials …