Cardiac pathologies and clinical follow-up of patients with Williams syndrome

Canan AYABAKAN, Özlem SARISOY, Rıza TÜRKÖZ, Kürşad TOKEL, Emre ÖZKER, Can VURAN, Bülent SARITAŞ

Abstract

Between August 2008 and August 2011, nine patients (3 girls, 6 boys; mean age 19±29 months) were admitted to our clinic with Williams syndrome. The mean body weight of the patients was 9454±5880 g. Two patients had supravalvular aortic stenosis (sAS), one had periferal pulmonary stenosis (PPS), one had supravalvular PS (sPS), two had sAS and sPS, and three had sAS and bilateral PPS. Two patients were not operated. Four patients were operated for sAS and PPS, one patient for sAS, valvular and sPS, one patient for PPS and sPS, and one patient for sAS operation. The gradients were decreased and remained unchanged during follow-up.

Özet

Ağustos 2008 - Ağustos 2011 tarihleri arasında Williams sendromlu dokuz hasta (3 kız, 6 erkek; ort. yaş 19±29 ay) kliniğimize başvurdu. Hastaların vücut ağırlıkları 9454±5880 g idi. Hastaların ikisinde supravalvüler aortdarlığı (sAS), birinde periferik pulmoner darlık (PPS),birinde supravalvüler PS (sPS), ikisinde sAS ve sPS, üçünde sAS ve iki taraflı PPS saptandı. İki hasta ameliyatedilmedi. Dört hastaya sAS ve PPS, bir hastaya sAS ilevalvüler ve sPS, bir hastaya sPS ve PPS, bir hastaya sAS ameliyatı yapıldı. Gradyanlarda azalma olmakla birlikte,izlemde artış saptanmadı.