"Treat-to-close": Non-repairable ASD-PAH in the adult: Results from the North American ASD-PAH (NAAP) Multicenter Registry

Adults presenting with an unrepaired atrial septal defect and pulmonary arterial hypertension (ASD-PAH) are typically classified as "correctable" or "non-correctable". The use of directed PAH medical therapy in non-correctable ASD-PAH leading to favorable closure candidacy, repai...

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Bibliographic Details
Published in:International journal of cardiology 2019-09, Vol.291, p.127-133
Main Authors: Bradley, Elisa A, Ammash, Naser, Martinez, Sara C, Chin, Kelly, Hebson, Camden, Singh, Harsimran S, Aboulhosn, Jamil, Grewal, Jasmine, Billadello, Joseph, Chakinala, Murali M, Daniels, Curt J, Zaidi, Ali N
Format: Article
Language:English
Subjects:
Adult
Female
Heart Septal Defects, Atrial - diagnostic imaging
Heart Septal Defects, Atrial - epidemiology
Heart Septal Defects, Atrial - surgery
Humans
Male
Middle Aged
North America - epidemiology
Pulmonary Arterial Hypertension - diagnostic imaging
Pulmonary Arterial Hypertension - epidemiology
Pulmonary Arterial Hypertension - surgery
Registries
Retrospective Studies
Treatment Outcome
Walk Test - methods
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Summary:Adults presenting with an unrepaired atrial septal defect and pulmonary arterial hypertension (ASD-PAH) are typically classified as "correctable" or "non-correctable". The use of directed PAH medical therapy in non-correctable ASD-PAH leading to favorable closure candidacy, repair status and long-term follow-up is not well studied. We therefore sought to characterize response to PAH targeted therapy in 'non-correctable' ASD-PAH. Nine North American tertiary care centers submitted retrospective data from adults with unrepaired ASD-PAH that did not meet recommendations for repair at initial presentation (1996-2017). Sixty-nine patients (women 51(74%), 40 ± 15 years, mean pulmonary artery pressure (mPA) 51 ± 13 mm Hg, pulmonary vascular resistance (PVR) 8.7 ± 4.9 Wood units, Qp:Qs 1.6 ± 0.4) were enrolled. All patients were prescribed PAH targeted therapy and late shunt repair occurred in 19(28%) (Women 15(29%) vs. Men 4(22%), p = 0.6). At late follow-up (4.4 ± 2.9 years) 6-minute walk test distance (6MWTD) was significantly better in the group that underwent repair (486 ± 89 m vs. 375 ± 139 m, p 
ISSN:1874-1754
DOI:10.1016/j.ijcard.2019.03.056