- Discussion:
- cardiovascular manifestations cause 90% of the deaths;
- major abnormality is dilatation of the ascending aorta, which usually
begins in the intrapericardial portion and extends into or beyond
aortic arch;
- dilatation at level of valve may result in
aortic regurgitation;
- dilatation is progressive & precedes aortic dissection & rupture;
-
mitral regurgitation secondary to mitral valve prolapse may also occur,
and cardiac arrhythmias have been implicated as a cause of death;
