Management is both medical and surgical. Prostaglandin E1 is given to prevent closure of the ductus arteriosus thus reducing hypoxaemia and acidosis prior to surgery.
At surgery pulmonary valvotomy is performed to relieve right ventricular outflow obstruction and a systemic- pulmonary arterial anastomosis performed to ensure adequate pulmonary blood flow despite a small right heart and subsequent closure of the ductus arteriosus.
The aim of surgery is to allow right ventricular growth so that at a later date a more extensive valvotomy can be carried out and the systemic-pulmonary anastomosis closed. If at operation the right ventricle is minute then a Fontan procedure may be performed which allows blood flow from the right atrium directly into the pulmonary artery.
Last reviewed 01/2018