Unidirectional valved patch closure of VSD is a promising technique for patients with a large VSD and severe PAH. It had a favorable effect on the immediate, early, and midterm clinical outcomes and hemodynamic parameters.

Surgical correction of patients with tetralogy of Fallot with pulmonary atresia is now one of the routine procedures performed by pediatric cardiac surgeons. In one variant, the pulmonary arterial supply is derived from a fistulous communication from the coronary arteries. This rare and interesting situation poses a diagnostic and therapeutic dilemma, as well as providing specific management challenges to the surgical team. Here, we discuss important aspects of this rare variant, specifically its morphology, presentation, evaluation and management.

Management of long standing left to right shunt lesion resulting in elevated pulmonary vascular resistance (PVR) is challenging. Limited surgical options are further complicated by an unpredictable postoperative period. Unidirectional valve patch (UVP) closure has shown to be useful in cases of the large ventricular septal defect (VSD) who present late. We report a case of large aortopulmonary window coexisting with a large VSD with severe pulmonary artery hypertension and significantly elevated PVR that was managed surgically by closure of the window by sandwich technique and closure of the septal defect with a UVP. This report emphasizes the importance of UVP in the management of such patients.