Staged palliation of single ventricle with Levo-transposition of the great arteries

Abstract

Patients born with single ventricle and L-transposition of the great arteries present challenging medical and surgical management problems. This defect is characterized by a wide variation in cardiac morphology and physiology. The clinical spectrum can range from cyanosis due to pulmonary outflow obstruction and venoarterial shunting to congestive heart failure from pulmonary over-circulation and systemic outflow tract obstruction. To avoid major operations in ill neonates and infants, earlier surgical treatment strategies concentrated on less invasive procedures that did not require cardiopulmonary bypass. Because of relatively poor initial results and a small number of patients presenting later as good candidates for the Fontan procedure, more elaborate initial palliative operations have been used. Despite their increasing complexity, these procedures more completely address the underlying anatomic and physiologic problems and have accounted for steadily improved patient survival.