Ahmad Pandu Pratama
Universitas Indonesia
Title: Multimodality Approach to Determine Anomalous Left Coronary Artery Arising from The Pulmonary Artery (ALCAPA) Diagnosis
Biography
Biography: Ahmad Pandu Pratama
Abstract
Introduction. Anomalous left coronary artery arising from pulmonary artery (ALCAPA) is a very rare congenital anomaly. The incidence is about 1:300.000 live births and accounts for 0.25%–0.5% of all congenital heart defects. ALCAPA is one of the most common causes of myocardial ischemia and infarction in children. If left untreated, up to 90% of patients with ALCAPA syndrome will die within the first year of life. In patients who live to adulthood, ALCAPA syndrome may cause myocardial infarction, left ventricular dysfunction and mitral regurgitation, or silent myocardial ischemia, which can lead to sudden cardiac death. Early diagnosis and prompt surgical intervention with an aim to restore two-coronary-arteries circulatory system have shown an excellent results and lead to gradual myocardial recovery.
Case Report. Here we have 3 babies with ALCAPA came with signs and symptoms of heart failure. First and third case patients developed symptoms during 2-3 month of age, while second case patient at the 6 month of age. Diagnosis of ALCAPA confirmed by echocardiography in the first case patients. The second case beside echocardiography examination, also underwent multi-slice computed tomography (MSCT) scan examination. The third case patient have to underwent coronary angiography because coronary sinus was not well visualized by echocardiography. First and third case patient underwent ALCAPA repair with LCA implantation into Aortic root technic, showing favorable result, while second case patient scheduled to receive ALCAPA repair.
Conclusion. Diagnosing ALCAPA is challenging, but features like episodes of irritability during feeding that associated with sweating or pallor should alert us to suspect ALCAPA. Echocardiography is the most important non-invasive diagnostic tool and has replaced coronary angiography as the standard method of diagnosis. However, if it fails to visualized coronary sinus, other modalities such as MSCT Scan and magnetic resonance imaging (MRI) could be useful in order to make an early and accurate diagnosis. ALCAPA repair with left coronary artery (LCA) implantation was chosen due to favorable results found following re-establishment of the dual coronary system.