Sinus of Valsalva Aneurysm in a Preschooler With Schuurs-Hoeijmakers Syndrome.

Severe right ventricular outflow tract (RVOT) obstruction developed in a 4-year-old boy with Schuurs-Hoeijmakers syndrome and history of double-outlet right ventricle, tetralogy of Fallot type, status post repair with transannular patch augmentation of the RVOT. Echocardiography and computed tomography defined the presence of a 1 × 1-cm sinus of Valsalva aneurysm protruding into the RVOT, causing obstruction. Resection and repair of the aneurysm by a 2-patch technique as well as resection of RVOT muscle bundles and revision of the transannular patch were performed. The postoperative course was uneventful, with no evidence of persistent RVOT obstruction.