Difficult weaning from cardiopulmonary bypass after surgical
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The connection of the left coronary artery to the pulmonary artery may be asymptomatic due to high pulmonary vascular resistance in the context of left-to-right shunts. Before the repair of the mentioned anomalies, coronary anatomy must be defined.

A 10-month-old male infant was referred with the impression of a large subaortic VSD, a moderate-sized PDA, and pulmonary hypertension. Poor feeding and failure to thrive were noted. The patient was on oral medication (digoxin, furosemide, and enalapril).

On physical examination, he was 6200 g in weight and 67 cm in height with normal blood pressure, pulse rate, and body temperature. Additionally, tachypnea was detected. On cardiac examination, a precordial holosystolic murmur was auscultated.

Transthoracic echocardiography revealed mild left atrial enlargement, mild mitral regurgitation, moderate tricuspid regurgitation, right ventricular enlargement, tricuspid annular plane systolic excursion (TAPSE) of 10 mm, a large subaortic VSD (9 mm) with a bidirectional shunt, no evidence of aortic coarctation, and a left ventricular ejection fraction of 55%. Cardiac computed tomography angiography (CCTA) confirmed the same anatomical findings.

In surgery, the PDA was suture-ligated and the VSD was closed via the right atrial approach with autologous pericardium in a continuous running manner. At the end of the operation, the patient was weaned from cardiopulmonary bypass in sinus rhythm but with difficulty with the aid of a high dose of inotropes.

Postoperative transthoracic echocardiography revealed severe left ventricular dysfunction, moderate mitral regurgitation, and severe pulmonary hypertension; however, there were no right ventricular dysfunction and residual VSD.

The patient was transferred to the pediatric intensive care unit (PICU) with a stable hemodynamic status, and his sternum was kept open. In the next 2 postoperative days, his clinical conditions and echocardiographic findings showed no change. Therefore, CCTA was performed to seek the cause of the left ventricular dysfunction, and it revealed ALCAPA: the left main coronary artery originated from the pulmonary artery adjacent to the left sinus of Valsalva.

Source: https://onlinelibrary.wiley.com/doi/full/10.1002/ccr3.3486?af=R