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Published on: December 16, 2021
Evidence for Marfan cardiomyopathy.
Francisco Alpendurada1, Joyce Wong, Anatoli Kiotsekoglou
1Royal Brompton & Harefield NHS Foundation Trust, London, UK.
European Journal of Heart Failure
|September 24, 2010
Summary
This study suggests Marfan syndrome (MFS) can cause a primary cardiomyopathy. Biventricular dysfunction in MFS patients is often mild and asymptomatic, requiring tailored cardiovascular monitoring.
Area of Science:
- Cardiology
- Genetics
- Cardiovascular Imaging
Background:
- Marfan syndrome (MFS) is an inherited connective tissue disorder with frequent cardiovascular involvement.
- While valvular regurgitation is common, a primary Marfan-related cardiomyopathy remains debated.
- Cardiovascular magnetic resonance (CMR) can assess cardiac structure and function.
Purpose of the Study:
- To investigate the existence of a primary cardiomyopathy in Marfan syndrome patients using CMR.
- To evaluate biventricular volumes, ejection fraction, and mass in MFS patients.
Main Methods:
- Retrospective evaluation of 68 adult MFS patients without prior cardiac surgery or significant valvular regurgitation.
- Comparison of CMR-derived ventricular parameters with healthy control data.
- Assessment for heart failure, aortic dimensions, and valve disease.
Main Results:
- 25% of MFS patients exhibited reduced left ventricular ejection fraction (LVEF).
- Increased left and right ventricular volumes were observed in a significant subgroup.
- No association found between reduced LVEF and age, gender, aortic dimensions, or valve disease.
Conclusions:
- Findings support a primary cardiomyopathy in a subset of Marfan syndrome patients.
- Biventricular enlargement and dysfunction are typically mild, asymptomatic, and independent of other MFS cardiovascular issues.
- Further research is needed; MFS management should consider myocardial function alongside aortic root surveillance.
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