ABSTRACT

Valvar pulmonary atresia is a rare congenital anomaly usually associated with ventricular septal defects or intact ventricular septum. The patent ductus arteriosus (56%) and systemic pulmonary collaterals maintain pulmonary blood flow. Presentation beyond childhood is unusual, as there is usually high morbidity in the first year of life. The two index cases highlight the rarity of adolescents with valvar pulmonary atresia and focus on the transthoracic echocardiographic features, whose diagnoses provide definitive and curative options with adequate size pulmonary dimensions. Options available are transcatheter interventional radiofrequency pulmonary valvotomy, which is both diagnostic and therapeutic, and intraoperative valve replacement, thus eliminating the need for right ventricle pulmonary artery external valved conduit during corrective surgery. They are the first cases of adolescents with isolated cardiac anomaly of valvar pulmonary atresia, documented in Afro-Caribbeans.