Pulmonary Atresia with Intact Ventricular Septum, also known as pulmonary atresia-intact ventricular septum syndrome, is related to tricuspid valve insufficiency and patent ductus arteriosus 1. An important gene associated with Pulmonary Atresia with Intact Ventricular Septum is TPM1 (Tropomyosin 1). Affiliated tissues include heart and liver, and related phenotypes are abnormal tricuspid valve morphology and maternal diabetes