Targeted Therapy Is Required for Management of Pulmonary Arterial Hypertension After Defect Closure in Adult Patients With Atrial Septal Defect and Associated Pulmonary Arterial Hypertension

Abstract

SummaryBackground: Therapeutic strategies for pulmonary arterial hypertension (PAH) associated with atrial septal defect (ASD) remain a matter of debate.Methods and Results: We identified 5 outpatients who had been diagnosed with ASD-PAH and undergone ASD closure in combination with targeted therapy with certified PAH drugs. We assessed changes in hemodynamic parameters and exercise capacity. The combination of ASD closure and targeted therapy significantly increased systemic blood flow (Qs) from the baseline (from 3.3 ± 0.6 L/minute to 4.2 ± 1.0 L/minute, P < 0.05) with a significant improvement in the World Health Organization Functional Class (WHO-FC; from 2.8 ± 0.4 to 1.6 ± 0.5, P < 0.05). The hemodynamic data before and after ASD closure without targeted therapy showed further elevation of pulmonary vascular resistance shortly after ASD closure (678 dyne·s/cm 5 to 926 dyne·s/cm

PPARγ Alleviates Right Ventricular Failure Secondary to Pulmonary Arterial Hypertension in Rats

“…33,34) However, the RV is the major determinant of functional state and prognosis in PAH. 35) Thus, PPARγ's advantage lies in offering the possibility of therapies that treat the RV and pulmonary artery as a whole.…”

Comparative Effectiveness of Oral Medications for Pulmonary Arterial Hypertension

“…PAH therapy has been substantially improved by prostacyclin formulations, endothelin receptor antagonists, PDE5 inhibitors, and other medications. 27,28) Prognostic improvements are also associated with increasing total medical costs. The total cost of oral medications for PAH in Japan is approximately JPY 48 billion per year ( © 2014 IMS Health, created based on the JPM Aug 2014 MAT, Unauthorized copying prohibited).…”

Treat and Repair Strategy in Patients With Atrial Septal Defect and Significant Pulmonary Arterial Hypertension

“…Treat and Repair for ASD and PAH describe, in a small number of serial patients, that treating patients with ASD and significant PAH using "certified" medications resulted in improvement of hemodynamics. 25 In their report, they also demonstrate that ASD closure alone could provoke an elevation of PVR and thus advocated the importance of "certified" PAH medications for these patients. Those results, with the results from the present study, suggest that PAH-specific medications have the potential to extend the consideration of interatrial left-to-right shunt closure for patients with severe PAH from the hemodynamic standpoint.…”