Abstract
BACKGROUND: Though a rare clinical entity, anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) a common cause of myocardial infarction in children. Unrecognized and untreated it leads to progressive left ventricular dilatation and systolic dysfunction. In settings of high infectious burden, ALCAPA may erroneously be diagnosed as myocarditis, dilated cardiomyopathy or other common childhood disorders. CLINICAL CASE: We present the case of a 10 weeks old male infant who presented to the inpatient unit with marked restlessness and irritability. He was inconsolable, had marked respiratory distress, cool extremities, central and peripheral cyanosis oxygen. The radial and brachial pulses were absent. The mean arterial pressure was 65mmHg, Heart rate of 160 beats per minute with a third heart sound. The liver was enlarged 4cm below the costal margin and tender, with a splenomegaly. He had an elevated Creatinine Kinase-MB of 112.5 u/L. ECG revealed deep Q waves in leads I, aVL, V5, V6 with ST elevation in the anterolateral leads. Echo showed a dilated left ventricle LVEDd of 40mm, with paradoxical interventricular septal motion, severe LV systolic dysfunction (FS=15%, EF=28%), LV anterolateral wall echo brightness and flow reversal in the Left coronary artery with its origin from the pulmonary trunk. He was admitted to the coronary care unit as a case of acute myocardial infarction with cardiovascular collapse. He received fluid resuscitation, inotropic support and standard management of heart failure. Six days later he was discharged home with a plan to refer abroad. He died at home after one week. CONCLUSION: A combination of a high index of suspicion, typical ECG and echocardiographic findings in a young infant presenting with LV dysfunction could lead to an earlier diagnosis of ALCAPA.