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[Hypertrophic obstructive cardiomyopathy: surgical treatment]
H D Schulte1, H Gramsch-Zabel, B Schwartzkopff
1Chirurgische Klinik der Heinrich-Heine-Universität, Düsseldorf.
Schweizerische Medizinische Wochenschrift
|October 14, 1995
Summary
Hypertrophic obstructive cardiomyopathy (HOCM) is an inherited heart muscle disease causing thickening and outflow obstruction. Surgical myectomy is a restrictive option for severe cases, with early mortality around 4.9%.
Area of Science:
- Cardiology
- Genetics
- Pathology
Context:
- Hypertrophic obstructive cardiomyopathy (HOCM) is an autosomal, dominant inherited myocardial disease.
- Characterized by left ventricular hypertrophy, outflow tract obstruction, and impaired compliance.
- Genetic defects are localized on various chromosomes, with histological evidence of myocyte disarray and interstitial fibrosis.
Purpose:
- To describe the pathological-anatomical and histological characteristics of HOCM.
- To investigate potential microcirculatory disturbances and their consequences.
- To review surgical indications and outcomes for HOCM.
Summary:
- Pathological-anatomical findings include asymmetric septal hypertrophy and left ventricular outflow tract obstruction.
- Histology reveals hypertrophied myocytes, disarray, and increased interstitial connective tissue.
- Dysplastic intramural arteries suggest microcirculatory disturbances contributing to arrhythmias, syncope, and sudden death.
Impact:
- Microcirculatory disturbances and scar development may underlie arrhythmias, syncope, and anginal pain.
- Surgical myectomy is reserved for NYHA class III patients refractory to medical treatment.
- Early mortality for surgical myectomy was 4.9% in 466 patients operated between 1963 and 1994.