ABSTRACT
The case is an infant referred to the mother after a fetal ultrasound at 22 gestational weeks revealed a left renal cyst and congenital heart disease. He was born at 36 weeks and five days, weighing 2,282 g, with Apgar 8/8 points, and diagnosed with tetralogy of Fallot, absent pulmonary valve, pulmonary regurgitation, persistent left superior vena cava, right aortic arch, and cervical arch. He was placed on ventilatory management immediately after birth, but was extubated at age nine days. On approximately day 26, since the SpO2 markedly decreased and bronchoscopy showed only mild stenosis, we concluded that decreased pulmonary blood flow mainly caused cyanosis. Because of the low body weight and presence of a single coronary artery, we decided to proceed with a palliative surgery. However, since the patient had a cervical arch and the usual Blalock-Taussig shunt was anatomically difficult, we performed a central shunt＋main pulmonary artery ligation on day 49. Cyanosis improved and his respiratory condition was stable, but contrast-enhanced computed tomography showed a tendency for left pulmonary artery enlargement and left main bronchus compression. On day 87, the Rastelli procedure＋bilateral pulmonary artery plication was performed because worsening airway symptoms were anticipated. The patient’s respiratory condition stabilized postoperatively, and he was extubated 10 days postoperatively. On day 136, the patient was discharged with a home high-flow nasal cannula.
