Congenital heart disease - Marfan syndrome
Description
- AD connective tissue disorder of fibrillin-1
- Triad of systems: skeletal + ocular + cardiovascular
- Cardiovascular disease determines survival; everything else determines the diagnosis
The lesions that kill
- Aortic root dilatation -> dissection/rupture (sinuses of Valsalva, effacing the sinotubular junction)
- Aortic regurgitation (annular dilatation)
- Mitral valve prolapse +/- MR (commonest valve lesion)
- Main pulmonary artery dilatation, descending aorta aneurysm
6 more sections, plus exam facts
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