Unmasking the Late Presentation of a Rare Coronary Artery Anomaly in Children

Introduction Anomalous left coronary artery from pulmonary artery (ALCAPA) is a rare congenital heart anomaly, making up to 0.46% of all cases of congenital heart disease [1]. In this condition, the left coronary artery (LCA) arises from the pulmonary artery (PA) instead of the aorta, leading to compromised myocardial perfusion. Clinical presentations range from asymptomatic to heart failure symptoms or sudden cardiac death. We present two cases of ALCAPA in pediatric patients. Case Presentation Case 1: An 11-year-old boy presented with fever, somnolence, and loss of appetite. Medical history included mitral valve replacement at 8 months due to congenital severe mitral regurgitation (MR). Echocardiography (Echo) demonstrated retrograde coronary flow and abnormal accessory blood flow at the PA, suggesting ALCAPA. Multislice computed tomographic angiography (MSCTA) confirmed the anomalous origin of the LCA from the proximal pulmonary trunk. Following treatment with antibiotics for an underlying infection, he was referred to a pediatric heart surgery center (PHSC). Case 2: An 8-year-old boy without prior complaints was referred to a pediatric cardiologist for suspected multiple ventricular septal defects. Echo revealed marked dilation of the LCA and the right coronary artery, with suspicions that the LCA originated from the PA. MSCTA confirmed ALCAPA. The surgery was planned in a PHSC. Discussion Since the LCA originates from the PA instead of the aorta, the blood supplied to the myocardium is poorly oxygenated, leading to myocardial ischemia (MI). If left untreated, up to 90% of infants die within the first year [2]. However, clinical presentation depends particularly on the number and size of collateral vessels [3]. Due to good collateral blood flow in the presented cases, there were no signs of MI in early infancy as described in previous reports [1].In other cases, findings such as isolated severe MR might mask the presence of the anomaly. Although Echo can suggest ALCAPA, CTA or MRI are used to confirm the diagnosis. Surgery is the treatment of choice for pediatric patients, with survival rates ranging up to 95% over 30 years [4]. Conclusions We presented 2 cases of ALCAPA, which is usually fatal in the first year of life. However, on rare occasions, it may only be diagnosed later in life due to masking or absence of symptoms.