Abstract 13140: Cardiac Arrest and Detection of Residual Shunt Following Ligation of Anomalous Left Coronary Artery Arising From the Pulmonary Artery (ALCAPA)
Background: Coronary anomalies are rare, often associated with morbidity and can be potentially fatal. We present a case of an adult with sudden cardiac death in the setting of residual anomalous left coronary from the pulmonary artery (ALCAPA) following presumed ligation in infancy.
Case: A 20 year old female with known dilated cardiomyopathy (DCM) with stable left ventricular ejection fraction (LVEF) and no symptoms of heart failure following presumed ligation of ALCAPA in infancy, suddenly collapsed due to ventricular fibrillation. After successful resuscitation with CPR and hypothermia protocol treatment, cardiac catheterization revealed residual connection of the left main coronary artery to the right pulmonary artery. Cardiac MRI revealed a dilated left ventricle with moderately depressed LVEF of 35% and old near transmural infarct of the posterolateral wall. She underwent defibrillator implantation for secondary prevention and successful closure of the residual coronary connection with an Amplatzer vascular plug. She continues to demonstrate stable LVEF and no evidence of further ischemia or heart failure. Figures 1,2 and 3 demonstrate catheterization and cardiac CT images of large conus branch supplying collateral flow to the left sided circulation, residual connection of the LMCA to the right pulmonary artery, and balloon occlusion and placement of vascular plug.
Discussion: ALCAPA is a rare congenital anomaly and untreated can result in DCM and death within the first year. With the development of an extensive collateral network, patients may survive into adulthood. However risks of ischemia, heart failure, arrhythmias, and sudden death exist.
Conclusion: ALCAPA is a rare yet potentially fatal congenital anomaly. This case demonstrates the importance of ongoing follow-up, recognition of such a rare finding, and detailing the option of percutaneous coronary intervention as treatment which has resulted in excellent prognosis.
- Adult congenital heart disease
- Cardiac arrest
- Percutaneous coronary intervention
- Congenital heart disease
- Heart failure
- © 2013 by American Heart Association, Inc.