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

To identify a large group of patients operated for absent pulmonary valve syndrome (APVS), and describe the outcomes of various subgroups and surgical strategies.

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 pulmonary valve (47%). All patients underwent ventricular septal defect (VSD) patching. Seventy-four percent underwent right ventricular outflow tract resection and 91% underwent transannular patching. Pulmonary artery reduction plasty was performed in 86%.

The follow-up was 94% complete. Nineteen percent had died. The median follow-up time was 9.2 years (1 day-20.5 years). Survival after repair was 82% at 1 year and 79% at 5, 10, and 15 years. Postoperative survival was strongly associated to preoperative ventilator dependency (p=0.002). The current New York Heart Association (NYHA) classification was established in 93% of survivors. Eighty-nine percent were in NYHA 1 or 2, 11% were in NYHA 3. No patient was in NYHA 4. Freedom from reoperation 1, 5, and 10 years after repair was 78, 65, and 55%, respectively.

The surgical outcome of APVS was closely related to preoperative ventilator dependency. Efforts to improve the surgical outcome should be focused on identifying and correcting this.