AI Article Synopsis

  • This study investigated the effectiveness of combining PAH-specific medications with transcatheter closure in patients with atrial septal defects (ASD) and severe pulmonary arterial hypertension (PAH).
  • It involved 42 patients who were treated with this "treat-and-repair" strategy and measured outcomes such as cardiovascular death and hospitalizations due to heart failure.
  • Results showed that after treatment, many patients experienced improved blood flow and reduced pulmonary artery pressure, with a low rate of complications and mortalities during a median follow-up period of 33 months.

Article Abstract

Objective: Therapeutic strategies for atrial septal defect (ASD) with severe pulmonary arterial hypertension (PAH) are controversial. This study aimed to evaluate the efficacy of PAH-specific medications and subsequent transcatheter closure (ie, treat-and-repair strategy) on clinical outcomes.

Methods: We enrolled 42 patients who were referred to 13 institutions for consideration of ASD closure with concomitant PAH and underwent the treat-and-repair strategy. The endpoint was cardiovascular death or hospitalisation due to heart failure or exacerbated PAH.

Results: At baseline prior to PAH-specific medications, pulmonary to systemic blood flow ratio (Qp:Qs), pulmonary vascular resistance (PVR), and mean pulmonary artery pressure (PAP) were 1.9±0.8, 6.9±3.2 Wood units and 45±15 mm Hg. Qp:Qs was increased to 2.4±1.2, and PVR and mean PAP were decreased to 4.0±1.5 Wood units and 35±9 mm Hg at the time of transcatheter ASD closure after PAH-specific medications. Transcatheter ASD closure was performed without any complications. During a median follow-up period of 33 months (1-126 months) after transcatheter ASD closure, one older patient died and one patient was hospitalised due to heart failure, but the other patients survived with an improvement in WHO functional class. PAP was further decreased after transcatheter ASD closure.

Conclusions: The treat-and-repair strategy results in low complication and mortality rates with a reduction in PAP in selected patients with ASD complicated with PAH who have a favourable response of medical therapy.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8862039PMC
http://dx.doi.org/10.1136/heartjnl-2021-319096DOI Listing

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Article Synopsis
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Surgical indications for patients with pulmonary arterial hypertension (PAH) and congenital heart defects are controversial. The treat and repair strategy has demonstrated efficacy in adult populations, but there have been no studies on pediatric patients. This study included pediatric patients with PAH and simple congenital heart defects who underwent corrective repair between 2012 and 2021.

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