Evolution of staged surgical management

Among the earliest reported attempts at palliation of hypoplastic left heart syndrome were those described by Cayler et al. in 1970,1 and Freedom et al. in 1977.2 Each case involved separate banding of the right and left pulmonary arteries, together with the construction of a direct aortopulmonary shunt. These two babies, one with critical aortic stenosis, and one with aortic atresia and ventricular septal defect, survived initial palliation but died without more definitive surgery. The separate banding of the pulmonary arteries had resulted in distortion of the distal pulmonary arterial branches and abnormal distribution of flow. Litwin et al.,3 in 1972, performed an operation for interrupted aortic arch in which a non-valved conduit was placed from the pulmonary trunk proximally to the descending thoracic aorta, and the pulmonary trunk was banded distal to the conduit. The one survivor again manifested severe distortion of the pulmonary arteries, and developed pulmonary vascular obstructive disease. An operation fundamentally similar to this, nonetheless, was subsequently used by several surgeons in an attempt to treat neonates with hypoplastic left heart syndrome. These included Albert et al. in 1978, and Mohri in 1979.