Abstract
BackgroundAnomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially fatal congenital coronary anomaly associated with early infant mortality and sudden adult death. By the development or lack of coronary collateral, it can be classified as infantile or adult type. However, even with the compensatory mechanism in adult patients, there is an estimated 80 to 90% incidence of sudden death at the mean age of 35 years.MethodsWe enrolled 9 patients with ALCAPA within the age group 5 to 16 years.ResultsOnly one patient developed symptoms (apsychia), whereas other patients were asymptomatic, and there was no evident left ventricular dysfunction found in any of the cases.ConclusionWith the development of imaging techniques, asymptomatic adult-type ALCAPA patients could be identified and diagnosed in childhood or adolescence. As a potential cause of sudden death, ALCAPA should be surgically repaired soon after the diagnosis.
Highlights
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially fatal congenital coronary anomaly associated with early infant mortality and sudden adult death
There is little or no coronary collateral development in the infantile type of circulation; and after the closure of patent ductus arteriosus, it could lead to severe myocardial ischemia, left ventricle (LV) dysfunction, dilatation, and mitral regurgitation (MR) when the pressure of pulmonary artery (PA) falls [5,6,7]
Seven patients (77.8%) had cardiomegaly on Chest X-ray (CXR); one patient (11.1%) appeared to be normal and another did not go through CXR
Summary
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially fatal congenital coronary anomaly associated with early infant mortality and sudden adult death. By the development or lack of coronary collateral, it can be classified as infantile or adult type. Even with the compensatory mechanism in adult patients, there is an estimated 80 to 90% incidence of sudden death at the mean age of 35 years. There is little or no coronary collateral development in the infantile type of circulation; and after the closure of patent ductus arteriosus, it could lead to severe myocardial ischemia, left ventricle (LV) dysfunction, dilatation, and mitral regurgitation (MR) when the pressure of pulmonary artery (PA) falls [5,6,7].
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
