Device Closure of a Large Atrial Septal Defect in a Patient with Severe Pulmonary Arterial Hypertension after 1 Year Use of an Oral Endothelin Receptor Antagonist

Abstract

The presence of severe pulmonary arterial hypertension (PAH) in patients with atrial septal defect (ASD) is still thought to preclude shunt closure, although there are several reports of good clinical outcomes after vasodilator therapy. We report the case of a young woman with ASD and severe PAH who was able to successfully undergo percutaneous shunt closure following 1 year use of the oral endothelin receptor antagonist, bosentan.

Large Atrial Septal Defect Closure in a Patient with Severe Pulmonary Arterial Hypertension

“…A decrease in mPAP or PVR that is less than 20% after a pulmonary vasodilator test indicates irreversible PAH [ 3 ]. Patients with severe or irreversible PAH are considered ineligible for defect closure, as the procedure is associated with a decrease in cardiac output, an increase in right-sided heart failure, and death in such patients [ 4 , 5 ].…”

“…The use of nonselective vasodilators that specifically target pulmonary vasodilation was reported to have a beneficial effect in patients with secondary PAH related to cardiac shunts [ 6 , 7 ]. Schwerzmann et al [ 4 ] and Jung et al [ 5 ] used 1-year intravenous prostacyclin and oral bosentan therapy, respectively, to treat ASD patients with less severe PAH. These patients showed clinical improvement after shunt closure following long-term vasodilator therapy.…”

“Treat and repair” strategy for shunt lesions: a critical review

“…42 In about half of the reports of pre-tricuspid shunts, catheterisation was not repeated after surgery and authors relied on Doppler echocardiography alone for the estimation of PA pressure, thereby underestimating to an extent and also making it difficult to assess an average change in PVRI due to TDT in this population. 28,29,31,36,[42][43][44] In the retrospective series by Bradley et al, 12 patients with ASD and severe PAH with PVR >6 WU and PVR/ SVR >0.3 were treated with TDT for a duration of 6-12 months. Five of them responded with >30% reduction in PVR with final PVR <6 WU and underwent ASD closure.…”

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

“…In addition, percutaneous ASD occlusion devices have become available that are expected to repair defects less invasively. 15,27,28) Therefore, the indication for defect closure may be different between percutaneous and surgical procedures. Thus, randomized controlled studies and/or world-scale surveys on national registries, such as the REVEAL Registry, 29) are required.…”

“…13,14) In addition, several recent reports have shown that targeted therapy with certified PAH drugs allows adult patients with uncorrected ASD-PAH to undergo successful ASD closure. [15][16][17] Thus, at present, the therapeutic strategy for patients with ASD-PAH needs to be revised.…”