Long term follow-up post catheter intervention for critical pulmonary valve stenosis and atresia with intact ventricular septum: A 25-year single institutional experience utilizing various techniques

Abstract

IntroductionCritical pulmonary stenosis (PS) and pulmonary valve atresia with intact ventricular septum and non-right ventricular dependent coronary circulation (PA) are rare congenital heart defects. They are characterized by systemic hypoxemia with spontaneous ductus arteriosus closure after birth and maintaining patency of the ductus arteriosus is essential for survival. Typical associated conditions are significant tricuspid insufficiency, variable hypoplasia of the right heart structures, right ventricular hypertrophy, right ventricular dysfunction and obligatory right to left shunting at the atrial level. Prior studies have demonstrated that a bipartite right ventricle (RV) and a tricuspid valve (TV) annulus Z score  -3 are associated with the need for supplemental blood flow to the pulmonary arteries via a surgical Blalock Taussig (BT shunt) and a trend toward subsequent univentricular palliation [1,2] as the RV is unable to support systemic cardiac output. Univentricular palliation includes a second stage bidirectional Glenn anastomosis and finally an intra or extracardiac Fontan palliation procedure. Patients who do not require univentricular palliation achieve a biventricular circulation. Journal of Clinical and Interventional CardiologyOriginal research Nair A et al., J Clin Intervent Cardiol. 2016 AbstractObjective: 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 valvulopla...