Hypoplastic left heart syndrome: medical management and practical surgical palliation1

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A new surgical procedure has allowed successful palliation of hypoplastic left heart syndrome in 2 full-term infants. This procedure establishes continuity between the proximal pulmonary artery and the descending aorta. Pulmonary flow is controlled by an aortopulmonary shunt to the isolated confluence of the pulmonary arteries. The technique obviates the need for manipulation of the diminutive ascending aorta and, in addition, corrects coarctation of the aorta which is present in 70% of patients with hypoplastic left heart syndrome. The wide availability of prostaglandin E1 offers a means of medical resuscitation for these moribund infants and makes surgical palliation a viable option for many infants whose condition had previously been considered hopeless.



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