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[Clinical aspects and course of endomyocardial fibrosis]
Insights
Endomyocardial fibrosis primarily affects women, causing severe heart failure with preserved systolic function. Mitral valve replacement significantly improves patient outcomes in cases of severe mitral insufficiency.
Area of Science:
- Cardiology
- Pathology
Background:
- Endomyocardial fibrosis (EMF) is a rare condition characterized by fibrosis of the endocardium.
- This study investigates the clinical course and outcomes of EMF in a cohort of female patients.
Observation:
- Six female patients with confirmed endomyocardial fibrosis were followed for 30 months.
- All patients exhibited partial obliteration of one or both heart chambers.
- Left ventricular pressure-volume relations indicated reduced ventricular distensibility.
Findings:
- Congestive heart failure was the predominant clinical presentation, with only slight cardiac enlargement.
- Systolic performance remained normal or moderately depressed despite restrictive filling and/or atrioventricular valve insufficiency.
- Echocardiography detected partial obliteration of ventricles; rheumatic fever was an occasional cause.
- Mitral valve replacement led to significant functional improvement in patients with severe mitral insufficiency.
Implications:
- Endomyocardial fibrosis predominantly affects women and presents with severe heart failure.
- Early detection via echocardiography and timely surgical intervention, such as mitral valve replacement, can improve patient prognosis.
- Further research into the etiology and management of endomyocardial fibrosis is warranted.
Abstract:
In 6 women with the hemodynamic, angiographic and histological findings of endomyocardial fibrosis the clinical course was followed for 30 months. All six cases showed the typical partial obliteration of one (4 patients) or both (2 patients) heart chambers. Left ventricular pressure-volume relations elicited decreased distensibility of the left ventricle. The functional state in 2 patients with severe mitral insufficiency was improved following successful mitral valve replacement. One patient with left ventricular, and 2 patients with biventricular, obliteration were given drug therapy and in one case recompensation lasting 2 years was obtained. One patient with partial obliteration of the left ventricle and severe biventricular failure died after an observation period of 4 years. In summary it can be said that (1) endomyocardial fibrosis in Switzerland is observed mainly in females; (2) the clinical picture is dominated by severe congestive heart failure in which the heart size is only slightly enlarged; (3) systolic performance is normal or only moderately depressed despite severe restriction to filling and/or av-valve insufficiency; (4) partial obliteration of the right or left ventricle may be detected by echocardiography; (5) rheumatic fever may occasionally be the cause of endomyocardial fibrosis; (6) mitral valve replacement is accompanied by appreciable improvement in the patient's condition.