Abstract
Aortic hypoplasia, aortic root, and valvular involvement associate with homozygous form of Familial Hypercholesterolem (FH) less commonly. We describe a 32 year old female patient with a previous dacron patch aortoplasty. The echocardiographic supra-aortic gradient was maximally 120 mmHg. Angiographicy revealed 40% left and 50% right ostial stenosis. Annular and supra annular stenosis was revealed by using the Manouguian type aortic root enlargement procedure with a unique oval shaped dacron patch. Before closing the patch completely, a supracoronary positioned mechanical aortic valve was implanted. Two saphenous bypasses for left anterior descending and right coronary arteries were added because of the ostial stenosis. The postoperative period was uneventfull. We suggest that all patients with FH should be evaluated in detail with CT or MRI for possible hypoplastic aortic root. Therapy of this kind of FH is still under debate. We evaluated the therapy modalities and small aortic root problems in this report.
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