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[Cardiovascular abnormalities in Marfan syndrome].
J H Fast1, G H Boers, S Meijers-Jacobs
1Afd. Cardiologie, De Wever-Ziekenhuis, Heerlen.
Nederlands Tijdschrift Voor Geneeskunde
|June 27, 1992
Summary
Marfan syndrome diagnosis is crucial for preventing aortic dissection. All diagnosed patients exhibited cardiac abnormalities, including mitral insufficiency and aortic dilatation, highlighting the need for early intervention and specialized clinics.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Marfan syndrome is a heritable connective tissue disorder.
- Early diagnosis is vital for preventing life-threatening complications like aortic dissection.
Observation:
- 52 adult patients were evaluated for Marfan syndrome between February and December 1990.
- Marfan syndrome was diagnosed in 24 out of 52 patients (46%).
Findings:
- All diagnosed patients presented with cardiac abnormalities.
- Common findings included mitral insufficiency (83%), aortic dilatation (67%), and aortic insufficiency (38%).
- Three patients had an aneurysm of the ascending aorta.
Implications:
- Early recognition of Marfan syndrome enables timely intervention to prevent aortic dissection.
- Endocarditis prophylaxis is recommended for patients with valve abnormalities.
- Establishing Marfan outpatient clinics can optimize diagnostic and management strategies.