Outcomes of atrial septostomy and effect on long-term survival in patients with idiopathic pulmonary arterial hypertension: A single-center cohort.

Objective: Pulmonary arterial hypertension (PAH) is a chronic progressive disease that may lead to right heart failure (RHF) and early death. Balloon atrial septostomy (BAS) may be used for the palliative treatment of RHF from PAH. We present our contemporary institutional experience of utilizing BAS in idiopathic PAH (IPAH) patients with refractory RHF to investigate the effect on the safety, efficacy and long-term survival.

Methods: This retrospective analysis included 12 IPAH patients with severe RHF from March 2017 to May 2019 who were assessed as high risk. All patients received standard treatment including combination of PAH-specific drugs. Graded BAS was performed on these patients due to unsatisfactory clinical response. Clinical, functional and hemodynamic variables before and immediately after the procedure were collected. 1-year follow-up outcomes and 3-year survival rate were further analyzed.

Results: Successful septostomy was achieved in cases with no procedure-related complications. All patients obtained hemodynamic improvement immediately after the procedure. The WHO functional class and exercise endurance improved at 1-year follow-up, 7 of 12 patients achieved intermediate-low risk status, while the rest remained at intermediate-high risk. 2 patients died at 18 and 20 months due to malignant arrhythmia and advanced heart failure, respectively. Survival at 1 year and 3 years was 100% and 83.3%.

Conclusions: In selected IPAH patients with refractory RHF, BAS is an additional therapeutic strategy, especially when PAH-specific drugs could not achieve the treatment target. BAS can improve hemodynamic variables, bring clinical and cardiac functional benefits and increase the 3-year survival.