Long-term outcomes of Transcatheter Potts shunt in children with suprasystemic pulmonary arterial hypertension

Abstract

Transcatheter Potts shunt (TPS) is a palliation alternative for children with severe pulmonary arterial hypertension (PAH). Debates on the long-term outcomes remain unsolved. Single-center retrospective data review of children with severe PAH who had TPS between 2009 and 2018. Patients who died per-operatively and early post-procedure were excluded. Long-term outcomes of survivors were evaluated. Out of 13 identified patients (53.8% males), 7 had endovascular stenting of probe/patent arterial ducts, while 6 individuals had aorta-to-pulmonary radiofrequency perforation and covered stent placement. Compared to baseline, the overall clinical condition significantly improved at discharge ( P < 0.001) and stayed better at the last visit ( P < 0.05) despite frequent clinical worsening events across follow-up. Improvement in functional status across follow-up was significant ( P < 0.001). There was no significant improvement in other disease markers (TPASE, 6MWD z-scores, and NT-proBNP levels) or reduction in PAH medications. The Median follow-up was 62.7 months (IQR, 32.2–96.5). Survival was 100% at 1 year and 92.3% at 5 years. Freedom from reinterventions was 77% at 1 year and 17% at 5 years. Eight (61.5%) patients had stent reinterventions at a median of 22 months (IQR, 8.8–49.8) postoperative. Balloon dilatation and restenting were performed in 46.1% and 38.5% of patients, respectively. Stent malfunctioning was present in 46.2% of patients at the last follow-up. One patient is listed for heart-lung transplantation. Survivors of TPS procedures experience significant improvement in functional class that can be durable. Clinical worsening and stent malfunctioning are frequent morbid events indicating recurrent transcatheter reinterventions throughout follow-up. Five-year survival is however satisfactory.