PubMed · 6814624
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O Feizi. 1982-11-20. Myocardial disarray revisited.. https://doi.org/10.1136/bmj.285.6353.1506
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BACKGROUND AND OBJECTIVE: The purpose of this study was to characterize adult patients with hypertrophic cardiomyopathy (HCM), to compare their mortality with that of the general population and to establish a prognosis based on clinical and noninvasive techniques. PATIENTS AND METHOD: One hundred nineteen consecutive patients (60 women, mean: 52 [12] years) with HCM were prospectively studied by ECG, Holter, echo-Doppler, exercise testing, myocardial perfusion SPET and radionuclide ventriculography. Prognostic variables included clinical data and parameters derived from these noninvasive techniques. RESULTS: During a mean follow-up of 10 [6.7] years, 7 patients (5.8%) died of cardiovascular causes (4 cardiac failure and 3 sudden death). The annual mortality rate was 0.6% and the actuarial survival curve for patients with HCM was significantly worse compared with the expected survival curve derived from the general population after adjustment for age and sex (p = 0.008). The presence of atrial fibrillation (p = 0.04), moderate or severe mitral regurgitation (p = 0.02), dynamic gradient > 50 mmHg (p = 0.02), left atrial diameter > 45 mm (p = 0.02), and interventricular septal thickness > 25 mm (p = 0.04) were all predictive of mortality. CONCLUSIONS: The mortality rate of adult patients with HCM is significantly higher than that expected for the general population and heart failure and sudden death are almost evenly distributed as a cause of death in these patients. Atrial fibrillation, magnitude of mitral regurgitation, dynamic gradient, left atrial dilatation and interventricular septal thickness are the main predictors of death.
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Hypertrophic cardiomyopathy is a common genetically transmitted disease, defined clinically by the presence of unexplained left ventricular hypertrophy. The disease has a varied clinical course and outcome; many patients have little or no discernible cardiovascular symptoms, whereas others have profound exercise limitation and recurrent arrhythmias. The overall risk of disease-related complications such as sudden death, endstage heart failure, and fatal stroke is roughly 1-2% per year, but the absolute risk in individuals varies as a function of underlying genetic abnormality, age, myocardial pathology, and other pathophysiological abnormalities such as impaired peripheral vascular responses. Genetic counselling and clinical risk stratification are relevant to all patients, but many therapeutic interventions, including septal alcohol ablation, septal myectomy, and implantable cardioverter defibrillators, are appropriate only in particular patient subsets. We review the management of patients with unexplained myocardial hypertrophy, considering the influence of underlying genetic and pathophysiological substrates on clinical decision-making.