Anomalous origin of the left coronary artery from the pulmonary artery with large anterior myocardial infarction and ischemia: successful tunnel repair and concomitant heterotopic heart transplantation as biological bridge to recovery

Sir: The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) causes myocardial ischemia, infarction, and cardiac failure, leading to a high mortality rate of 90 % in infancy [lo]. Early diagnosis and surgical repair may prevent further myocardial damage. Transposition of the anomalous coronary artery to the aorta and creation of a tunnel within the pulmonary artery to connect the ostium of the left coronary artery to the aorta has become a standard surgical technique with good results. The extent of myocardial ischemia significantly influences surgical mortalityrates [9]. A 4-month-old female infant was referred to our cardiac center with mild symptoms of cardiac failure, holosystolic heart murmur with propagation to the left axilla, and signs of myocardial infarction on the ECG. Echocardiography revealed severe hypokinesia of the anterolateral wall, an increased density of this part of the ventricular wall and the anterior papillary muscle, and severe mitral regurgitation. The origin of the left coronary artery could be visualized neither from the aorta nor from the pulmonary trunk. The diagnosis of ALCAPA was confirmed by cardiac catheterization, which showed that the left coronary artery was filled by collaterals from the right coronary artery. The left ventricular ejection fraction was calculated as 19 YO (Table 1). SPECT imaging using a T1-201 tracer and persantin showed a large area of diminished myocardial perfusion of the anterolateral wall indicative of scaring after myocardial infarction, but also a large area of tracer redistribution compatible with myocardial ischemia. Because of the severely impaired left ventricle, standard surgery for ALCAPA repair seemed to be too hazardous. We decided to transplant a heart heterotopically with the intention of removing the allograft after sufficient recovery of the native heart, since a pediatric left ventricular assist device was not available and our experience with extra-corporeal membrane oxygenation was rather limited at that time. On 4 September 1991, a suitable donor heart without a major HLA mismatch became available. For ALCAPA repair, direct implantation of the coronary artery into the aorta was impossible, and therefore tunnel repair was performed. Heterotopic heart transplantation followed, with the donor pulmonary artery connected to the patient’s right atrium to augment left ventricular function only.