Individualised interventional strategies and clinical outcomes in paediatric patients with pulmonary arterial hypertension.

OBJECTIVE

This study evaluated the safety and efficacy of individualised interventional strategies in paediatric pulmonary arterial hypertension, focusing on the outcomes of atrial septostomy, reverse Potts shunt, and partial ASD closure in patients unresponsive to medical therapy or with rapid right ventricular deterioration.

METHODS

A retrospective analysis included 12 paediatric patients who underwent 18 interventional procedures between 2019 and 2024. Pulmonary arterial hypertension was confirmed by right heart catheterisation, and pre- and post-procedural clinical, echocardiographic, and haemodynamic data were compared.

RESULTS

Interventions included graded balloon atrial septostomy (n = 10), partial ASD closure (n = 2), and reverse Potts shunt via ductal stenting (n = 1). All surviving patients showed clinical improvement with significant reductions in heart rate (p < 0.01), NT-proBNP (p = 0.008), mean right atrial pressure (p < 0.001), and RV/LV end-diastolic diameter ratio (p = 0.014), along with improvements in six-minute walk distance (p = 0.002), WHO functional class (p < 0.001), and TAPSE (p = 0.028). One patient (8.3%) died within days following atrial septostomy due to nonadherence to medical therapy. Three patients required repeat atrial septostomy because of shunt restriction or spontaneous narrowing.

CONCLUSION

Individualised interventional strategies guided by comprehensive haemodynamic assessment can slow disease progression and improve quality of life in paediatric pulmonary arterial hypertension. Partial ASD closure, as a novel approach in this cohort, reduces excessive left-to-right volume load while preserving a controlled right-to-left shunt, thereby enhancing haemodynamic stability and optimising outcomes. Timely implementation of interventional strategies before disease progression may help reduce mortality.