[Noonan syndrome associated with atrial septal defect, pulmonary stenosis, and completely unroofed coronary sinus without LSVC: a case report].

: An eight-year-old boy, Noonan syndrome associated with ASD and PS, was referred to our department for surgical repair. During operation, the coronary sinus ostium was not found. Farther more exploration revealed completely unroofed coronary sinus without LSVC. The large ASD (confluent with coronary sinus ASD) was closed with a ePTFE patch. The pulmonary valve was thickened moderately and each commissure was adhesive, but not dysplastic. PS was released with commissurotomy and subpulmonary muscle resection. The postoperative course was uneventful and the patient discharged at 14 postoperative day. At present, he has been followed at outpatient without PS and any sign and symptom of myocardial hypertrophy.