PMID- 29517668
OWN - NLM
STAT- MEDLINE
DCOM- 20180326
LR  - 20231105
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Print)
IS  - 0025-7974 (Linking)
VI  - 97
IP  - 10
DP  - 2018 Mar
TI  - The efficiency of endothelin receptor antagonist bosentan for pulmonary arterial 
      hypertension associated with congenital heart disease: A systematic review and 
      meta-analysis.
PG  - e0075
LID - 10.1097/MD.0000000000010075 [doi]
LID - e0075
AB  - 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.
FAU - Kuang, Hong-Yu
AU  - Kuang HY
AD  - 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.
FAU - Wu, Yu-Hao
AU  - Wu YH
FAU - Yi, Qi-Jian
AU  - Yi QJ
FAU - Tian, Jie
AU  - Tian J
FAU - Wu, Chun
AU  - Wu C
FAU - Shou, We Nian
AU  - Shou WN
FAU - Lu, Tie-Wei
AU  - Lu TW
LA  - eng
PT  - Journal Article
PT  - Meta-Analysis
PT  - Review
PT  - Systematic Review
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Endothelin Receptor Antagonists)
RN  - 0 (Sulfonamides)
RN  - Q326023R30 (Bosentan)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Bosentan
MH  - Child
MH  - Child, Preschool
MH  - Endothelin Receptor Antagonists/*therapeutic use
MH  - Familial Primary Pulmonary Hypertension/complications/*drug therapy
MH  - Heart Defects, Congenital/*complications
MH  - Humans
MH  - Infant
MH  - Middle Aged
MH  - Sulfonamides/*therapeutic use
MH  - Treatment Outcome
MH  - Young Adult
PMC - PMC5882424
COIS- The authors have no conflicts of interest to disclose.
EDAT- 2018/03/09 06:00
MHDA- 2018/03/27 06:00
PMCR- 2018/03/09
CRDT- 2018/03/09 06:00
PHST- 2018/03/09 06:00 [entrez]
PHST- 2018/03/09 06:00 [pubmed]
PHST- 2018/03/27 06:00 [medline]
PHST- 2018/03/09 00:00 [pmc-release]
AID - 00005792-201803090-00013 [pii]
AID - MD-D-17-07225 [pii]
AID - 10.1097/MD.0000000000010075 [doi]
PST - ppublish
SO  - Medicine (Baltimore). 2018 Mar;97(10):e0075. doi: 10.1097/MD.0000000000010075.