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[Course and prognosis of primary hypertrophic cardiomyopathies]
Insights
Hypertrophic cardiomyopathy patients face a significant mortality risk, especially those with familial forms. Early identification of high-risk families with specific ECG and echocardiogram findings is crucial for prognosis.
Area of Science:
- Cardiology
- Genetics
Context:
- Hypertrophic cardiomyopathy (HCM) is a primary cardiac condition.
- Long-term outcomes and prognostic factors in HCM require further elucidation.
Purpose:
- To investigate the long-term clinical course and mortality in a cohort of hypertrophic cardiomyopathy patients.
- To identify prognostic factors, particularly familial aspects, influencing patient outcomes.
Summary:
- This study followed 33 hypertrophic cardiomyopathy patients for a mean of 48 months, observing a 21% 5-year mortality rate.
- Familial forms of HCM, especially in high-risk families with specific electrocardiogram (ECG) and echocardiogram findings, indicated a poor prognosis.
- Left ventricular diastolic diameter and septal thickness increased on echocardiography, irrespective of clinical course, while sudden death was not observed.
Impact:
- Findings highlight the critical role of family history and specific cardiac imaging/ECG markers in predicting hypertrophic cardiomyopathy prognosis.
- Identifies high-risk familial HCM as a distinct entity with a severe outlook, guiding clinical management and genetic counseling.
Abstract:
33 patients with hypertrophic cardiomyopathy were followed for a mean duration of 48 months. 29 of them were reviewed; 6 had died, including 4 with a familial form belonging to two different families. No cases of sudden death were observed. The mortality rate was 4 p. cent at 1 year, 11 p. cent at two years and 21 p. cent at 5 years. The clinical course was marked by a functional deterioration in one-third of cases and, one echocardiography, by an increase in the diastolic diameter of the left ventricle and in the thickness of the septum, independent of the clinical course. Subjects from "high risk" families have a very poor prognosis (4 deaths out of 7 patients at an average age of 25). These families present major conductive disturbances on the electrocardiogram and a very marked parietal hypertrophy on the echocardiogram. No other prognosis factor independent of the familial aspect was revealed.