Management of Anomalous Left Coronary Artery from the Pulmonary Artery

Twenty-five patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) are reported. Six unoperated infants, ages 2-9 months, died within 3 months after the onset of symptoms and emphasize the poor prognosis of medically treated patients with this defect. Nineteen patients were treated surgically at a mean age of 13.7 months after the onset of symptoms by one of the following methods: simple ligature, four patients; aortic reimplantation, one patient; aortocoronary bypass graft (ACBG) with autogenous saphenous vein, seven patients; ACBG with retroaortic free segment of the autogenous left subclavian artery (LSCA), five patients; and ACBG with a transpulmonary arterial free segment of the LSCA, two patients. Two patients died during operation among those managed by ligature alone. All other surgically treated patients survived and are alive and well at late follow-up. Postoperatively the electrocardiographic ischemic pattern, cardiac size and angiographic and echocardiographic measures of left ventricular function have improved in most patients. Improvement has been greater in patients with patent ACBGs who underwent early operation. Prompt surgery is recommended in all infants and children with ALCAPA, regardless of symptomatic status. Reestablishment of a two-coronary-artery system is the procedure of choice, especially in infancy, because simple ligature has a high mortality. A new method of transpulmonary arterial ACBG using a free segment of the LSCA is also described.