Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience

Abstract

Background Absent pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary arteries and compression of the tracheobronchial tree and may lead to significant respiratory compromise. We describe the outcomes of surgical correction of absent pulmonary valve syndrome and risk factors for mortality and reoperation. Methods A review of 52 patients with absent pulmonary valve syndrome who underwent surgical correction between 1975 and 2013 was conducted. The median age and weight at repair were 9 months (range, 4 days to 24.2 years) and 6.9 kg (range, 1.8 to 56 kg). Preoperative intubation was required in 15 patients (29%), and 21 patients (40%) underwent urgent repair. Th..