Late outcomes for the surgical management of absent pulmonary valve syndrome in infants

Abstract

Absent pulmonary valve syndrome (APVS) is a rare cardiac malformation that is usually associated with aneurysmal dilatation of pulmonary arteries and respiratory distress. The surgical mortality of neonates and infants with APVS has decreased tremendously, from 60% in 1980s to 10-20% recently. This study retrospectively reviews surgical outcomes of our 10-year experience in patients with APVS. From 2002 to 2012, 42 patients with APVS underwent surgical correction. Thirty-seven patients had APVS as a variant of tetralogy of Fallot, 4 with double outlet right ventricle and 1 with ventricular septal defect. Respiratory distress was present in 12 infants. Four patients needed continuous positive airway pressure and 5 required intubation with mechanical ventilation before surgery. There was no hospital death and 3 late deaths. The mean follow-up time was 62.71 ± 34.31 months. Significant differences were found in the duration of postoperative ventilation between patients with or without respiratory distress (P = 0.009) and patients with left or right aortic arch (P = 0.012). The Kaplan-Meier curve indicated that overall survival at 5 and 10 years was 92.4%. The survival rates between patients with or without respiratory distress were 72.7 and 100%, respectively (P = 0.003). Overall mortality was associated with longer cardiopulmonary bypass time (P = 0.004) and lower weight at operation (P = 0.042). There were no significant differences in survival and postoperative data such as the duration of ventilation or intensive care unit stay and New York Heart Association class among the three methods of right ventricular outflow tract (RVOT) reconstruction. Surgical treatment of APVS has got favourable outcomes in terms of mortality and reoperation rate. Different methods of RVOT reconstruction do not affect the surgical outcome. Patients required long-term follow-up for postoperative respiratory complications secondary to persistent bronchomalacia.