Background: Absent pulmonary valve syndrome (APVS) is a relatively rare anomaly that is usually associated with a ventricular septal defect and a restrictive pulmonary annulus with severe pulmonary regurgitation. The to-and-fro flow across the right ventricular outflow tract produces enormously dilated and pulsatile pulmonary arteries (PAs) that cause severe respiratory distress and tracheomalacia by compression of the trachea and primary bronchi. This retrospective study summarizes our 20-year experience of surgical treatment in patients with APVS.
Methods: Between 1984 and 2005, 20 patients underwent repair of APVS using a valved conduit (n = 9), transannular patch (TAP) insertion alone (n = 5), or TAP with monocusp valve (n = 6) with PA reduction arterioplasty. Median age was 7 months (range, 6 days to 9 years).
Results: There were one perioperative and two late deaths. All deaths were related to airway complications. Survival was 90% at 1 year and 85% at 10 and 15 years. In a multivariate analysis only preoperative ventilator dependency was associated with a worse outcome (p = 0.02). Follow-up was available in 18 operative survivors (mean follow-up, 7.3 +/- 5.2 years). Six patients (33%) underwent reoperation for pulmonary valve incompetence and right ventricular dysfunction. Three patients (17%) had episodic bronchospasm of mild to moderate severity that were responsive to sympathomimetic bronchodilators.
Conclusions: Morbidity associated with perioperative respiratory complications and ventilator dependency due to underlying tracheobronchomalacia is an important problem in patients with APVS. These infants may require multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Complete repair with a valved conduit and reduction pulmonary arterioplasty at the onset of symptoms and a definitive diagnosis is our procedure of choice for infants with APVS. With this approach, the airway can be optimized to give the best patient outcome.