Long-term survival of shunt closure in atrial septal defects with pulmonary arterial hypertension.

OBJECTIVE

This study aims to refine closure indications and assess long-term outcomes in pulmonary arterial hypertension associated with atrial septal defect (PAH-ASD).

METHODS

We enrolled 197 PAH-ASD patients classified as unrepairable (n = 101) or repairable (n = 96), with the latter subdivided into direct repair (DR, n = 74) and treat-and-repair (TR, n = 22) groups. Closure criteria included pulmonary vascular resistance (PVR) 3-6 Wood units and Qp/Qs ≥1.5. The TR group received targeted therapy before closure.

RESULTS

Targeted therapy significantly improved TR group hemodynamics: PVR decreased from 8.0 ± 2.0 to 3.0 ± 1.0 WU (p < 0.001); Qp/Qs increased from 1.7 ± 0.4 to 2.3 ± 0.3 (p < 0.001). Post-closure, pulmonary artery systolic pressure significantly decreased in both TR (67 ± 15 to 38 ± 9 mmHg) and DR groups (66 ± 17 to 40 ± 10 mmHg) (both p < 0.001). Right ventricular remodeling improved significantly. During median follow-up (3.7-4.5 years), mortality was lower in repairable versus unrepairable groups (1 vs. 10 deaths). Univariate COX analysis identified ASD closure as a protective factor (HR 0.093, 95 %CI [0.01,0.98].

CONCLUSION

ASD closure improves long-term survival in select PAH-ASD patients. Targeted therapy enables closure in previously ineligible patients.