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.