PMID- 37284521
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230919
IS  - 2045-8932 (Print)
IS  - 2045-8940 (Electronic)
IS  - 2045-8932 (Linking)
VI  - 13
IP  - 2
DP  - 2023 Apr
TI  - Muscle strength is reduced in children with pulmonary arterial hypertension.
PG  - e12246
LID - 10.1002/pul2.12246 [doi]
LID - e12246
AB  - Muscle strength is decreased in adults with pulmonary arterial hypertension 
      (PAH). We aim to investigate muscle strength in children with PAH in relation to 
      a cohort of healthy children, and investigate correlations with disease severity 
      markers. This prospective study included children with PAH aged 4-18 years, who 
      visited the Dutch National Referral Center for Pulmonary Hypertension in 
      Childhood between October 2015 and March 2016. Muscle strength was assessed using 
      handgrip strength and maximum voluntary isometric contractility (MVIC) of four 
      peripheral muscles. Dynamic muscle function was evaluated with the 
      Bruininks-Oseretsky test of motor proficiency (BOT-2). These measurements were 
      compared with those in two cohorts of healthy children and correlated with 
      6-minute walk distance (6MWD), World Health Organization functional class 
      (WHO-FC), N-terminal pro-brain natriuretic peptide (NT-proBNP), and time since 
      diagnosis. Eighteen children with PAH aged 14.0 [interquartile range: 9.9-16.0] 
      years showed reduced muscle strength. Handgrip strength z-score -2.4 +/- 1.2, 
      p < 0.001, total MVIC z-score -2.9 +/- 1.2, p < 0.001, and BOT-2 z-score 
      -1.0 +/- 0.9, p < 0.001. 6MWD (67 +/- 11% predicted) correlated with most muscle 
      measurements (r = 0.49-0.71, p = 0.001). Dynamic muscle function (BOT-2) differed 
      between WHO-FC, whereas handgrip strength and MVIC did not. NT-proBNP and time 
      since diagnosis did not show significant correlations with muscle strength 
      measurements. Muscle strength was significantly reduced in children with PAH and 
      correlated with 6MWD, but not with disease severity markers WHO-FC and 
      NT-pro-BNP. The nature of this reduced muscle strength is yet unclear, but its 
      occurrence in children with seemingly mild or well-controlled PAH supports the 
      concept of PAH being a systemic syndrome involving peripheral skeletal muscles.
CI  - (c) 2023 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on 
      behalf of Pulmonary Vascular Research Institute.
FAU - Pepplinkhuizen, Shari
AU  - Pepplinkhuizen S
AD  - Department of Pediatric Cardiology, Center for Congenital Heart Disease, Beatrix 
      Children's Hospital, University Medical Center Groningen University of Groningen 
      Groningen The Netherlands.
FAU - Eshuis, Graziella
AU  - Eshuis G
AUID- ORCID: 0000-0001-6469-1566
AD  - Department of Pediatric Cardiology, Center for Congenital Heart Disease, Beatrix 
      Children's Hospital, University Medical Center Groningen University of Groningen 
      Groningen The Netherlands.
FAU - Zijlstra, Willemijn M H
AU  - Zijlstra WMH
AD  - Department of Pediatric Cardiology, Center for Congenital Heart Disease, Beatrix 
      Children's Hospital, University Medical Center Groningen University of Groningen 
      Groningen The Netherlands.
FAU - Timmer, Carola Y
AU  - Timmer CY
AD  - Department of Rehabilitation, University Medical Center Groningen University of 
      Groningen Groningen The Netherlands.
FAU - Ploegstra, Mark Jan
AU  - Ploegstra MJ
AD  - Department of Pediatric Cardiology, Center for Congenital Heart Disease, Beatrix 
      Children's Hospital, University Medical Center Groningen University of Groningen 
      Groningen The Netherlands.
FAU - Lelieveld, Otto T H M
AU  - Lelieveld OTHM
AD  - Department of Rehabilitation, University Medical Center Groningen University of 
      Groningen Groningen The Netherlands.
FAU - Hepping, Ann Marjolein
AU  - Hepping AM
AD  - Department of Orthopedics, University Medical Center Groningen University of 
      Groningen, Groningen The Netherlands.
AD  - Roessingh Center for Rehabilitation Enschede The Netherlands.
FAU - Beenakker, Ernesto A C
AU  - Beenakker EAC
AUID- ORCID: 0000-0001-7786-0285
AD  - Department of Neurology Medical Center Leeuwarden Groningen The Netherlands.
FAU - Berger, Rolf M F
AU  - Berger RMF
AD  - Department of Pediatric Cardiology, Center for Congenital Heart Disease, Beatrix 
      Children's Hospital, University Medical Center Groningen University of Groningen 
      Groningen The Netherlands.
LA  - eng
PT  - Journal Article
DEP - 20230605
PL  - United States
TA  - Pulm Circ
JT  - Pulmonary circulation
JID - 101557243
PMC - PMC10241349
OTO - NOTNLM
OT  - cardiopulmonary physiology and pathophysiology
OT  - muscle
OT  - pulmonary arterial hypertension
COIS- The authors declare no conflict of interest.
EDAT- 2023/06/07 13:10
MHDA- 2023/06/07 13:11
PMCR- 2023/06/05
CRDT- 2023/06/07 09:50
PHST- 2022/09/08 00:00 [received]
PHST- 2023/05/19 00:00 [revised]
PHST- 2023/05/23 00:00 [accepted]
PHST- 2023/06/07 13:11 [medline]
PHST- 2023/06/07 13:10 [pubmed]
PHST- 2023/06/07 09:50 [entrez]
PHST- 2023/06/05 00:00 [pmc-release]
AID - PUL212246 [pii]
AID - 10.1002/pul2.12246 [doi]
PST - epublish
SO  - Pulm Circ. 2023 Jun 5;13(2):e12246. doi: 10.1002/pul2.12246. eCollection 2023 
      Apr.