Case Presentation
A 14-week-old female infant, who had respiratory distress worsened after bronchiolitis, was admitted to the intensive care unit. She was intubated in another hospital before the admission. On the first evaluation, the patient was cyanotic and had a cutis marmorata appearance on her skin. Capillary refill time was 4 seconds, and her pulse was week. She had tachycardia and bilaterally crepitant rales. According to the echocardiographic examination, the left atrium and left ventricle were greatly enlarged and left ventricular contractions were markedly decreased (Shortening Fraction:13%), thus indicating a dilated cardiomyopathy. Figure 1 represents the right ventricle (RV) stuck between the sternum and the left ventricle (LV). Septum movements were better than the posterior LV wall. A moderate degree of insufficiency was observed in the mitral and tricuspid valve. Pulmonary arterial pressure was measured to be approximately 60 mmHg through the tricuspid valve insufficiency. There was also a mild degree of insufficiency in the aortic valve. Pulmonary and aortic flows were decreased due to low cardiac output. Since she had a cardiogenic shock, IVIG, hydrocortisone, and inotropic support drugs were started with the preliminary diagnosis of myocarditis. The patient was placed on extracorporeal membrane oxygenation (ECMO) due to the low cardiac output leading to multiorgan failure using neck vessels. After two weeks on ECMO support, the patient was successfully weaned and separated from ECMO support. Both Coronary Multi-detector Computed Tomography (MDCT) and heart catheterization were performed with suspicion of ALCAPA syndrome. Figure 2 shows the coronary arteries anatomy.
The shadow indicated by the black arrow may appear as the pulmonary cusp, which the left coronary artery drains, in Figure 3. Although origin of the left coronary artery from aorta or pulmonary artery, could not be visualized, the patient underwent operation for left coronary artery revascularization, with the preoperative possible diagnosis of ALCAPA. After a midline sternotomy, the thymus was resected, and the pericardium was opened. After heparinization, aortic and bicaval cannulation was performed to initiate cardio-pulmonary bypass (CPB). The pulmonary artery and aorta were dissected from each other. Diastolic cardiac arrest was provided with 30°C systemic hypothermia and antegrade tepid blood cardioplegia after cross-clamping. Tepid blood cardioplegia was repeated every 20 minutes until releasing cross-clamp. The left side of the heart was vented. Pulmonary arteriotomy and aortotomy were performed. There were no left coronary ostia neither in the pulmonary artery nor in the aorta.
The Circumflex artery (Cx) and left anterior descending artery (LAD) were visible, but small in diameter. They were merged into a small blind pouch, 2 cm away from the left coronary sinus of the aorta. There was no possibility for preparation and anastomosis to the aorta, due to very small diameter and fragile vessel. Left internal mammary artery (LIMA) was harvested. LAD, near to the pouch was opened, LIMA-LAD anastomosis was performed with 8-0 prolene. Pulmonary artery and aortotomy were closed. Cross clamp was removed after de-airing. The patient was weaned from CPB with high dose s of inotropic support including, adrenaline, noradrenaline, dopamine, and milrinone. The sternum was left open due to hemodynamic instability. Cardiopulmonary bypass time was 141 minutes, and cross-clamp time was 101 minutes.
The patient needed ECMO support due to the low cardiac output on the second postoperative day. The patient died of massive cranial hemorrhage on ECMO, despite some improving ventricular function in the 10th postoperative day.