Bibhuti Das, Shreesha Maiya, Basha Khan, Vimal Raj, Shashi Raj
Pulmonary arterial hypertension in children remains a progressive disease marked by right ventricular failure and early mortality despite advances in targeted therapy. In resource-limited settings, access to parenteral prostacyclin is often constrained by cost, infrastructure, and challenges related to chronic administration. The atrial flow regulator is a transcatheter device that creates a controlled interatrial shunt to provide right-heart decompression. We describe our single-center experience with atrial flow regulator implantation in children with severe pulmonary arterial hypertension. We retrospectively reviewed six children with World Health Organization Group 1 pulmonary arterial hypertension who underwent device implantation between 2021 and 2024. Baseline assessment included echocardiography, cardiac magnetic resonance imaging, right-heart catheterization, N-terminal pro-B-type natriuretic peptide (NT-proBNP), and 6-minute walk distance. All patients had advanced pulmonary vascular disease despite combination oral therapy. Median indexed pulmonary vascular resistance was 17.0 Wood units·m² (10.8-28.5), median right ventricular ejection fraction was 31% (22-39), and median 6-minute walk distance was 250 m (190-280). Device implantation was successful in all patients, with no periprocedural mortality. During follow-up, NT-proBNP levels decreased and 6-minute walk distance improved. Friedman testing demonstrated significant changes over time for NT-proBNP (p = 0.002) and 6-minute walk distance (p = 0.003). In this small cohort, atrial flow regulator implantation was technically feasible and associated with favorable longitudinal trends in biomarker and functional measures. In resource-constrained settings, it may serve as an adjunctive strategy for carefully selected high-risk patients. Larger multicenter studies are needed to refine patient selection and long-term outcomes.