Abstract Objective: Compare various catheter based interventions for critical pulmonary stenosis (PS) and pulmonary atresia with intact ventricular septum without right ventricular dependent coronary circulation (PA) regarding outcomes, predictors and survival. Results: Sixty two neonates underwent intervention at 4 days (weight: 3.3 kg). Fifty one had PS and 11 had PA with one procedural mortality (1.6%). Post intervention, 11 patients (18%) required a surgical BT shunt of which six eventually required univentricular palliation. Multivariate analysis predicted the initial tricuspid valve Z score was an independent predictor for BT shunt placement (mean -3.1; CI: -2.2 to -3.9 vs -1.9; CI: -1.3 to -2.5, p < 0.017). Initial pulmonary valve Z score was an independent predictor for both BT shunt placement (mean -1.6; CI: -2.1 to -1.1 vs -0.4; CI: -0.6 to -0.2, p < 0.001) and univentricular palliation (mean -1.4; CI: -2.2 to -0.67 vs -0.5; CI: -0.8 to -0.3, p = 0.029). Mean follow up is 10 years with a 93% survival. 25-year freedom from valve re-intervention was similar in patients with a biventricular repair requiring a BT shunt vs not. Four patients (7%) eventually required surgical pulmonary valve replacement (mean: 11 years; range 0.81- 19.82 years). Conclusion: A smaller tricuspid valve annulus diameter was an independent variable for BT shunt placement. A smaller pulmonary valve annulus was an independent predictor for BT shunt placement and subsequent univentricular palliation. 25-year survival is excellent with a low incidence of valve re-intervention or replacement in those patients with a biventricular circulation.
Keywords: critical pulmonary valve stenosis; pulmonary valve atresia; pulmonary valvuloplasty; laser perforation