Anomalous origin of the left coronary artery (ALCAPA) is a rare congenital defect with an incidence of 1:300,000. These patients often present in infancy with anterior myocardial infarction, CHF or death. Adult type ALCAPA patients often present with symptomatic lesions, and therapy is aimed to restore a two coronary system. We present a 52 year old female who presented with severe mitral insufficiency who underwent preoperative cardiac catheterization and was found to have an anomalous left coronary artery arising from the main pulmonary artery. The patient underwent a mitral valve replacement along with re-establishment of a dual coronary system with bypass grafting and ligation. The patient recovered without incident. A review of the literature was undertaken to determine the optimum management of these asymptomatic lesions in adults.
We conducted a Medline review of the English literature from 1966 through September of 2004. Fifteen articles containing 41 patients >11 years old were identified. The patient data was tabulated and analyzed.
The mean age at diagnosis was 34 (16-72). The male to female ratio was 1:2. 86% of the patients underwent surgical therapy. Of these patients, 69% had a dual coronary system re-established either by direct reimplantation of the left coronary artery or by grafting with either the IMA or SVG. There were 2 perioperative deaths (5.7%). In the five patients reported with nonoperative therapy, there was a 40% incidence of cardiac arrest in follow up.
Adult survival appears to be improved with surgical therapy for ALCAPA. Re-establishment of a dual coronary system with closure of the fistula is the most common surgical therapy for adult type ALCAPA. Interestingly, the male to female ratio (1:2) in adults is reversed to that found in infants and children (2:1); suggesting men are more susceptible to death as a result of this disorder if left untreated.
Asymptomatic adult patient with ALCAPA are at increased risk for myocardial infarction and sudden death.
Frank Manetta, None.