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The efficiency of endothelin receptor antagonist bosentan for pulmonary arterial hypertension associated with congenital heart disease: A systematic review and meta-analysis. | LitMetric

The efficiency of endothelin receptor antagonist bosentan for pulmonary arterial hypertension associated with congenital heart disease: A systematic review and meta-analysis.

Medicine (Baltimore)

Department of Cardiology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics Riley Heart Center, Herman B Wells Center for Pediatric Research, Indiana University School of Medicine, Indianapolis, IN.

Published: March 2018

AI Article Synopsis

  • Oral bosentan has been evaluated for its effectiveness in treating pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) through a systematic review and meta-analysis involving adult and pediatric patients.
  • The analysis covered 17 studies with a total of 456 PAH-CHD patients, finding significant improvements in exercise capacity, particularly in the 6-minute walk distance (6MWD) and functional classification within six months of treatment, although some measures showed no change.
  • Long-term bosentan therapy led to further enhancements in exercise performance and changes in vital parameters such as resting oxygen saturation and heart rate, indicating its effectiveness in managing PAH-CHD.

Article Abstract

Background: Oral bosentan has been widely applied in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD). A systemic review and meta-analysis was conducted for a therapeutic evaluation of oral bosentan in both adult and pediatric patients with PAH-CHD. The acute responses and a long-term effect were respectively assessed in a comparison with baseline characteristics, and the improvement of exercise tolerance was analyzed.

Methods: PubMed, Medline, Embase, and Cochrane Central Register of clinical controlled trails or observational studies have been searched for a recording of bosentan effects on the PAH-CHD participants. For mortality and rate of adverse events (AEs), it was described in detail. Randomized-effects model or fixed-effects model was used to calculate different effective values with a sensitivity analysis.

Results: Seventeen studies were pooled in this review, and 3 studies enrolled the pediatric patients. Among all studies, 456 patients were diagnosed with PAH-CHD, and 91.7% were treated with oral bosentan. With a term less than 6 months of bosentan therapy, there existed a significant improvement in 6-minute walk distance (6MWD) and the World Health Organization functional class (WHO-FC), but no such differences in Borg dyspnea index scores (BDIs) and the resting oxygen saturation (SpO2). Although with a prolonged treatment, not only 6MWD and FC, but also the resting SpO2 and heart rate were changed for a better exercise capability. Additionally, compared with the basic cardiopulmonary hemodynamics, it showed a statistically significant difference in mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance index (PVRi). Although a limitation of pooled studies with comparative outcomes of different terms, outcomes presented a lower WHO-FC which contributes to a success in a prolonged treatment.

Conclusions: Bosentan in PAH-CHD is well established and still requires clinical trials for an identification of its efficiency on CHD patients for an optimized period lessening a serious complication and the common AEs.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5882424PMC
http://dx.doi.org/10.1097/MD.0000000000010075DOI Listing

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