C11. Aorto-Pulmonary Window in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome : A Rare Case Report

Abstract

Abstract Background Aorto-pulmonary window and Tetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) are very rare entities. The combination of the two defects in adolescence patient made the diagnosis based on standard imaging modalities challenging. Case Illustration 14-year-old boy was referred from Borneo Hospital with failure to thrive and shortness of breath symptoms. He was underwent multiple modalities procedure and it shows the presences of features of tetralogy of Fallot : right ventricular hypertrophy, sub-aortic ventricular septal defect and pulmonary infundibular stenosis. Pulmonary valve leaflets are not seen with mild dilatation of the main and left pulmonary artery (Absent PV syndrome) and a connection from Pulmonary Artery and Ascending Aorta (Aorta-Pulmonary Window) Discussion TOF-APVS is a relatively rare variant of TOF. Its co-existence with Aorto-Pulmonary Window make this case was very exceptional. There are only a few reported cases diagnosed with TOF-APVS in adult age in the literature as most of them are diagnosed at younger ages. The unique physiology due to the large connection of Ascending aorta and the main pulmonary artery resulting in L-R Shunt. The large connection from Ascending Aorta to Main Pulmonary artery (L-R Shunt) prompt this patient to pulmonary hypertension (before and after surgery) and heart failure, hence initial treatment of ACE-I and sildenafil was very necessary.