Case report
A 41-day-old female infant was admitted for sustained fever. The patient was diagnosed with bacterial meningitis by analysis of the cerebrospinal fluid and treated with intravenous antibiotics. During the hospital stay, the patient showed desaturation accompanied by cyanosis and cardiomegaly (cardiothoracic ratio, 0.77) on chest X-ray (Fig. 1A). Due to a constant increase in oxygen demand, the patient was intubated and transferred to the pediatric intensive care unit. Echocardiography showed coarctation of the aorta with decreased left ventricular function, atrial septal defect (ASD), and mitral valve regurgitation (Fig. 1B). Three-dimensional computed tomography (CT) angiography showed PFAA with aortic coarctation (Fig. 1C). Because the patient showed progressive oliguria and metabolic acidosis, we decided to perform emergency surgery.
The intraoperative findings revealed that the fourth aortic arch was connected to the descending aorta through a stenotic isthmic portion, and a stenotic area was also observed between the PFAA and the descending aorta (Fig. 2A). Cardiopulmonary bypass (CPB) was established by arterial cannulation of the innominate artery and bicaval venous cannulations. After cardiac arrest was induced, the connection between the fourth aortic arch and the descending aorta was divided. Antegrade cerebral perfusion was started under 25 ℃ of body temperature, and the CPB flow rate was 50 ml/min/kg. The PFAA was also divided, and the ductal tissue was resected completely from the descending aorta (Fig. 2B). The repair was performed in an end-to-side fashion between the proximal stump of the fifth aortic arch and the trimmed descending aorta (Fig. 2C). The ASD was closed primarily, and the CPB was weaned successfully. The perioperative period was uneventful. The patient was extubated three days after the operation. Postoperative echocardiography showed a wide aortic arch with improved left ventricular function and no mitral regurgitation. The postoperative CT showed a wide aortic arch as well (Fig. 3). The patient was discharged from the hospital 18 days after the operation and followed up 12 months without complications.