Unilateral Pulmonary Artery Banding for Critically ill Neonate with Absent Pulmonary Valve Syndrome: A Case Report

Abstract Backgound Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease that is challenging to manage when it presents with severe symptoms at an early age. We describe a critically ill neonate with tetralogy of Fallot (TOF) with APVS in whom main pulmonary artery (PA) banding failed, but alternative left PA banding and PA plication and open chest strategy effectively stabilized the patient’s condition and enabled elective cardiac repair 6 days later. Case presentation A 3.2-kg baby girl with prenatal diagnosis of TOF and APVS was electively delivered by cesarean section. She was intubated soon after birth, however her condition deteriorated with respiratory and metabolic acidosis even with maximum ventilator settings and inotropic support. On the second day of life, she developed bloody stool suggesting necrotizing enterocolitis, indicating the need for a palliative procedure without cardiopulmonary bypass. First, we attempted main PA banding but abandoned it due to suspected myocardial ischemia caused by left main coronary artery compression. As an alternative procedure, we performed left PA banding, which improved oxygen saturation and increased blood pressure; the chest was left open. Postoperatively, the patient’s condition improved dramatically within 48 hours. On the eighth day of life, we performed an elective intracardiac repair on her under stable preoperative condition. At the 5-year follow-up, she was in a stable condition at home with home oxygen therapy. Conclusion Unilateral PA banding and open chest strategy may be an alternative treatment of choice for critically ill neonates with TOF with APVS.