Optimal Therapeutic Strategy for Ventricular Septal Defect Closure in Extremely Preterm Infants with Chronic Lung Disease and Severe Pulmonary Hypertension

In preterm infants with large ventricular septal defects (VSD), severe chronic lung disease (CLD), and pulmonary hypertension (PH), hemodynamic instability may occur after open-heart surgery. The selection of first pulmonary artery banding or one stage VSD closure is a clinical issue. At present, no guidelines exist to support decision-making in this unique population. This study reports the case of a preterm infant with a gestational age of 23 weeks and 4 days, a birth weight of 540 g, and elevated immunoglobulin-M levels, who was successfully treated with pulmonary artery banding and subsequently VSD closure. Before pulmonary artery banding, the pulmonary-to-systemic blood flow ratio was 2.5 and pulmonary vascular resistance was 2.6 U·m2 under general anesthesia with FiO2 = 0.6. However, pulmonary artery banding preceded considering the severe CLD and the risk of PH crisis after cardiopulmonary bypass. In a review of the literature on preterm infants with VSD with CLD and PH, only five infants, including the one in this case, were found. One infant underwent pulmonary artery banding first and survived, and three infants underwent one-stage VSD closure, after which one died (trisomy 21) due to a postoperative PH crisis. Three of the five cases underwent tracheostomy. The selection of first pulmonary artery banding or one-stage VSD closure needs to be carefully considered on a case-by-case basis, taking into consideration the severity of the VSD with CLD and PH. A future registry study will be needed to review these cases and clarify the outcome of the two strategies.