PMID- 37945502
OWN - NLM
STAT- MEDLINE
DCOM- 20240101
LR  - 20240416
IS  - 1879-114X (Electronic)
IS  - 0149-2918 (Linking)
VI  - 46
IP  - 1
DP  - 2024 Jan
TI  - Selexipag for the Treatment of Pediatric Pulmonary Hypertension: A Systematic 
      Review.
PG  - 59-68
LID - S0149-2918(23)00391-0 [pii]
LID - 10.1016/j.clinthera.2023.09.026 [doi]
AB  - PURPOSE: To systematically evaluate the safety, dosing regimen, and efficacy of 
      selexipag for pediatric patients with pulmonary hypertension (PH). METHODS: A 
      literature search of the electronic databases of PubMed, Embase, Web of Science, 
      Cochrane Library, and Google Scholar was performed from inception through 
      February 28, 2023. Two reviewers independently searched and evaluated the quality 
      of the studies and pooled data when appropriate. Full-text articles of studies of 
      children diagnosed with PH and treated with selexipag were eligible. Pediatric 
      patients with PH were classified into 2 groups: the add-on therapy group, in 
      which selexipag was used as a third therapy in addition to the baseline 
      treatment, and the transition therapy group, in which patients were switched from 
      parenteral prostacyclin analogs to selexipag. FINDINGS: Fourteen studies 
      involving 58 pediatric patients with PH were included. All studies were either 
      case reports or case series. Overall, 30 and 28 patients were in the add-on and 
      transition therapy groups, respectively. In both groups, selexipag was initially 
      administered as 50-200 microg twice daily and titrated to a tolerated dosage of 
      200-1,600 microg twice daily. Prostacyclin analogs were simultaneously weaned for 
      patients in the transition group. In the add-on therapy group, 16 patients 
      (80.0%) were at low risk of the World Health Organization functional class (WHO 
      FC I/II), 12 (76.9%) were at low risk of the 6-minute walk distance (6MWD; >350 
      m), and 21 (95.5%) were at low risk of the pulmonary vascular resistance index 
      (PVRi; <20 WU/m(2)). Furthermore, N-terminal pro-brain natriuretic peptide and 
      mean pulmonary arterial pressure were significantly improved. More than 70% of 
      patients experienced common tolerable side effects, such as headache, nausea, and 
      diarrhea. In the transition therapy group, 5 patients (55.6%) were at low risk 
      according to WHO FC I/II, 6 (66.7%) were at low risk according to 6MWD, and 14 
      (87.5) were at low risk according to PVRi; however, selexipag had no significant 
      effect on their hemodynamic parameters. Additionally, more than 80% of patients 
      experienced no side effects. IMPLICATIONS: Selexipag as add-on therapy or for 
      transition from prostacyclin analogs may have a favorable safety profile and 
      potential efficacy for pediatric patients with PH. Further high-quality evidence 
      of the efficacy and safety of selexipag for the treatment of pediatric PH is 
      warranted.
CI  - Copyright (c) 2023 Elsevier Inc. All rights reserved.
FAU - Li, Meng
AU  - Li M
AD  - Department of Pharmacy, Shenzhen Children's Hospital, Shenzhen, China.
FAU - Liu, Lin
AU  - Liu L
AD  - Department of Cardiology, Shenzhen Children's Hospital, Shenzhen, China.
FAU - Liu, Cong
AU  - Liu C
AD  - Department of Cardiology, Shenzhen Children's Hospital, Shenzhen, China.
FAU - Chen, Zebin
AU  - Chen Z
AD  - Department of Cardiology, Shenzhen Children's Hospital, Shenzhen, China.
FAU - Li, Weibin
AU  - Li W
AD  - Department of Cardiology, Shenzhen Children's Hospital, Shenzhen, China.
FAU - Li, Xuejuan
AU  - Li X
AD  - Department of Cardiology, Shenzhen Children's Hospital, Shenzhen, China.
FAU - Ma, Xiaopeng
AU  - Ma X
AD  - Department of General Surgery, Shenzhen Children's Hospital, Shenzhen, China.
FAU - Zhang, Yumao
AU  - Zhang Y
AD  - Department of Pharmacy, the Eighth Affiliated Hospital, Sun Yat-sen University, 
      Shenzhen, China. Electronic address: y.zhangym@gmail.com.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PT  - Systematic Review
DEP - 20231107
PL  - United States
TA  - Clin Ther
JT  - Clinical therapeutics
JID - 7706726
RN  - 5EXC0E384L (selexipag)
RN  - 0 (Antihypertensive Agents)
RN  - 0 (Acetamides)
RN  - 0 (Prostaglandins I)
SB  - IM
MH  - Humans
MH  - Child
MH  - *Hypertension, Pulmonary/drug therapy
MH  - Antihypertensive Agents/adverse effects
MH  - Acetamides/adverse effects
MH  - Prostaglandins I/therapeutic use
OTO - NOTNLM
OT  - Dosing regimen
OT  - Efficacy
OT  - Pediatrics
OT  - Pulmonary hypertension
OT  - Safety
OT  - Selexipag
COIS- Declaration of Competing Interest None.
EDAT- 2023/11/10 00:44
MHDA- 2024/01/02 11:46
CRDT- 2023/11/09 21:57
PHST- 2023/04/27 00:00 [received]
PHST- 2023/09/27 00:00 [revised]
PHST- 2023/09/28 00:00 [accepted]
PHST- 2024/01/02 11:46 [medline]
PHST- 2023/11/10 00:44 [pubmed]
PHST- 2023/11/09 21:57 [entrez]
AID - S0149-2918(23)00391-0 [pii]
AID - 10.1016/j.clinthera.2023.09.026 [doi]
PST - ppublish
SO  - Clin Ther. 2024 Jan;46(1):59-68. doi: 10.1016/j.clinthera.2023.09.026. Epub 2023 
      Nov 7.