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Published on: August 8, 2022
Hypertrophic cardiomyopathy: the early years
1Harvard Medical School, Boston, MA, USA. ebraunwald@partners.org
Insights
Hypertrophic obstructive cardiomyopathy (HOCM) is characterized by severe left ventricular hypertrophy and potential sudden cardiac death. This review highlights the key features of HOCM, including its genetic basis and hemodynamic obstruction.
Area of Science:
- Cardiology
- Genetics
- Pathophysiology
Background:
- Hypertrophic obstructive cardiomyopathy (HOCM) is a complex cardiac condition.
- Understanding its defining features is crucial for diagnosis and management.
Observation:
- HOCM presents with severe left ventricular hypertrophy, often in the basal interventricular septum.
- Familial occurrence with autosomal dominant transmission is common.
- Sudden cardiac death, typically due to ventricular fibrillation, is a significant risk.
Findings:
- The review examines key papers recognizing HOCM's major features.
- Particular focus is placed on the labile nature of intraventricular obstruction.
- The identification of labile obstruction in 1959 completed the description of HOCM's major characteristics.
Implications:
- This comprehensive understanding aids in diagnosing and managing HOCM.
- Recognizing the lability of obstruction is key for clinical assessment.
- The golden anniversary of HOCM's description marks a milestone in cardiovascular research.
Abstract:
Hypertrophic obstructive cardiomyopathy (HOCM) has four major features: (1) severe left ventricular hypertrophy, often most prominent in the basal interventricular septum; (2) frequent familial occurrence with autosomal dominant transmission; (3) occurrence of sudden cardiac death that is usually considered to be due to ventricular fibrillation; and (4) presence of hemodynamic evidence of labile intraventricular obstruction. The key papers describing the recognition of each of these features, as well as of various combinations of them, are reviewed in this paper. Particular attention is focused on the very frequent finding of marked lability of intraventricular obstruction. The recognition of this fourth and last major feature in 1959 makes 2009 the golden anniversary year marking completion of the description of the major features of HOCM.
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