Adult Type Anomalous Origin of the Left Coronary Artery from the Main Pulmonary Artery: Report of One Case

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare disease. Most of the cases suffer from myocardial infarction, congestive heart failure or even death during the early infantile period, and thus are called “infantile type”. We present one five-year-old girl with ALCAPA syndrome, adult type, who was initially suspected to be a case of atypical Kawasaki disease (KD) complicated with coronary arterial aneurysms. Due to persistent dilatation of the coronary artery after medical treatment for one year and a turbulent flow noticed between the left coronary artery (LCA) and the main pulmonary artery (MPA), cardiac catheterization was arranged. The diagnosis was established from the coronary cineangiography in spite of a direct connection between the aorta and LCA revealed from the echocardiogram. Thereafter open heart surgery was undertaken. Diagnosis of ALCAPA syndrome can be made by echocardiogram, but this is not fully satisfactory. Selective coronary cineangiography remains the definitive diagnosis. Although the patients of adult type are more asymptomatic, surgical correction should be undertaken as soon as the diagnosis is established since a substantial risk is still present.