日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Postnatal pulmonary circulation depends on the ductus in neonates with pulmonary atresia with an intact ventricular septum (PAIVS), requiring intravenous administration of prostaglandin. However, life-threatening events, such as necrotizing enterocolitis, can occur due to pulmonary overcirculation through the ductus. To address this issue, prostaglandin therapy may be discontinued or hypoxic inhalation treatment may be attempted. This paper reports the case of a 2.2-kg male infant with PAIVS who underwent successful staged surgery. The patient exhibited heart failure due to pulmonary overcirculation soon after birth despite the discontinuation of prostaglandin therapy and the initiation of hypoxic gas treatment. At the age of 3 days, he underwent bilateral pulmonary arterial banding to regulate the pulmonary blood flow, resulting in stable hemodynamics. Subsequently, we performed transcatheter pulmonary valvuloplasty and surgical right ventricular outflow plasty at 21 and 55 days of age, respectively. Bilateral pulmonary arterial banding could serve as an alternative bridge therapy to definitive corrective surgery to achieve stable hemodynamics during the neonatal period.