Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up

Abstract

Objective: To identify a large group of patients operated for absent pulmonary valve syndrome (APVS), and describe the outcomes of various subgroups and surgical strategies. Methods: Thirty-six consecutive patients operated during 1979-2004 were included. Thirty-one percent furthermore had other vascular abnormalities. Eighty-three percent had airway obstruction symptoms before surgery. Twenty-eight percent were ventilator dependent, while 72% underwent repair electively. The median age and weight at repair were 0.8 years (4 days-24 years) and 6.7 kg (1.8-56 kg). The surgical approach was modified several times, including the following: homograft conduit (14%), monocusp valve (39%), and no p..