Management of Rare Combination of Cardiac Anomalies in A Patient with Noonan Syndrome

Mustafa Yılmaz, Şafak Alpat, İlker Ertuğrul, Tevfik Karagöz, Metin Demi̇rci̇n

Keywords: Noonan syndrome, atrial fibrillation, atrial septal defect, noncompaction, sick sinus syndrome

Abstract

Noonan syndrome is a rare disorder in which different cardiac abnormalities can exist. This report describes the combination of cardiac abnormalities in a patient with Noonan syndrome, which has not been reported previously. Coexisting cardiac abnormalities were: atrial septal defect, atrial fibrillation and noncompaction of the ventricular myocardium. Surgical fenestrated closure of atrial septal defect and concomitant maze procedure were performed. Surgery of congenital heart defects in syndromic patients has different difficulties than those in nonsyndromic patients. Thus, specific approach should be taken in these patients.