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Circulation, Vol 65, 1388-1394, Copyright © 1982 by American Heart Association
BJ Maron, WC Roberts and SE Epstein
The clinical profile of 78 patients with hypertrophic cardiomyopathy who
died suddenly (or experienced cardiac arrest and survived) was analyzed. At
the time of cardiac catastrophe, 71% of the patients were younger than 30
years of age, 54% were without functional limitation and 61% were
performing sedentary or minimal physical activity. Nineteen of the 78
patients (24%) were taking propranolol in apparently adequate dosages,
indicating that this drug does not provide absolute protection against
sudden death. No clinical or morphologic variable was particularly reliable
in identifying patients at risk for sudden death. Forty-eight of 62
patients (77%) who died suddenly had a markedly increased ventricular
septal thickness of 20 mm or more; however, mean septal thickness was
similar in patients who died suddenly (25.2 +/- 0.9 mm) and in age- and
sex-matched control patients with hypertrophic cardiomyopathy who have
survived (23.6 +/- 0.8 mm). An abnormal ECG was present as often in
patients who died suddenly as in control patients who have survived, (51 or
53, 96%). In addition, no particular cardiac symptom or hemodynamic
variable (such as the magnitude of left ventricular outflow tract
obstruction under basal conditions or left ventricular end-diastolic
pressure) was characteristic of the patients with hypertrophic
cardiomyopathy who died suddenly.
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Sudden death in hypertrophic cardiomyopathy: a profile of 78 patients
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