title: Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients.
abstract: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo-8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.
authors.forename: Moises
authors.initials: M
authors.lastname: Rodriguez-Gonzalez
authors.forename: Antonio Moruno
authors.initials: AM
authors.lastname: Tirado
authors.forename: Reza
authors.initials: R
authors.lastname: Hosseinpour
authors.forename: Jose Santos
authors.initials: JS
authors.lastname: de Soto
mesh_terms: D001011:Aorta / Q000601:surgery; D063748:Bland White Garland Syndrome / Q000150:complications / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery*; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D002311:Cardiomyopathy, Dilated / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D017023:Coronary Angiography; D003331:Coronary Vessels / Q000000981:diagnostic imaging / Q000601:surgery*; D018618:Echocardiography, Doppler, Color; D004562:Electrocardiography; D005260:Female; D016027:Heart Transplantation; D006353:Heart-Assist Devices; D006801:Humans; D007223:Infant; D008297:Male; D008944:Mitral Valve Insufficiency / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D017202:Myocardial Ischemia / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D011237:Predictive Value of Tests; D012092:Replantation; D012189:Retrospective Studies; D016896:Treatment Outcome; D018754:Ventricular Dysfunction / Q000175:diagnosis / Q000209:etiology / Q000601:surgery
keywords: Abnormalities, multiple/diagnosis; cardiac surgical procedures/methods; cardiomyopathy, dilated/etiology/physiopathology; collateral circulation/physiology; coronary vessel anomalies/diagnosis/pathology/radiography/surgery; echocardiography, Doppler, color/methods; heart arrest/etiology; pulmonary artery/abnormalities/pathology; time factors; treatment outcome