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.