Pulmonary atresia with intact ventricular septum (PA/IVS) is a spectrum of diseases with varying severity of right ventricle hypoplasia and potential for biventricular, univentricular, or hybrid repairs. Pessimistic outcome measures for PA/IVS may give way to optimism with the refinement of early diagnosis and early intervention to encourage right ventricle flow and optimize growth. To this end, PA/IVS has become a focus of innovative interventions (eg,fetal surgery) and new catheter-based and surgical techniques.