PMID- 29767574
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 2045-8932 (Print)
IS  - 2045-8940 (Electronic)
IS  - 2045-8932 (Linking)
VI  - 8
IP  - 3
DP  - 2018 Jul-Sep
TI  - Right ventricular stroke work correlates with outcomes in pediatric pulmonary 
      arterial hypertension.
PG  - 2045894018780534
LID - 10.1177/2045894018780534 [doi]
LID - 2045894018780534
AB  - Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary 
      artery pressures (PAP) and pulmonary vascular resistance (PVR). Optimizing 
      treatment strategies and timing for transplant remains challenging. Thus, a 
      quantitative measure to predict disease progression would be greatly beneficial 
      in treatment planning. We devised a novel method to assess right ventricular (RV) 
      stroke work (RVSW) as a potential biomarker of the failing heart that correlates 
      with clinical worsening. Pediatric patients with idiopathic PAH or PAH secondary 
      to congenital heart disease who had serial, temporally matched cardiac 
      catheterization and magnetic resonance imaging (MRI) data were included. RV and 
      PA hemodynamics were numerically determined by using a lumped parameter (circuit 
      analogy) model to create pressure-volume (P-V) loops. The model was tuned using 
      optimization techniques to match MRI and catheterization derived RV volumes and 
      pressures for each time point. RVSW was calculated from the corresponding P-V 
      loop and indexed by ejection fraction and body surface area (RVSW(EF)) to compare 
      across patients. Seventeen patients (8 boys; median age = 9.4 years; age 
      range = 4.4-16.3 years) were enrolled. Nine were clinically stable; the others 
      had clinical worsening between the time of their initial matched studies and 
      their most recent follow-up (mean time = 3.9 years; range = 1.1-8.0 years). 
      RVSW(EF) and the ratio of pulmonary to systemic resistance (Rp:Rs) values were 
      found to have more significant associations with clinical worsening within one, 
      two, and five years following the measurements, when compared with PVR index 
      (PVRI). A receiver operating characteristic analysis showed RVSW(EF) outperforms 
      PVRI, Rp:Rs and ejection fraction for predicting clinical worsening. RVSW(EF) 
      correlates with clinical worsening in pediatric PAH, shows promising results 
      towards predicting adverse outcomes, and may serve as an indicator of future 
      clinical worsening.
FAU - Yang, Weiguang
AU  - Yang W
AUID- ORCID: 0000-0003-3324-4346
AD  - 1 Department of Pediatrics (Cardiology), Stanford University, Stanford, CA, USA.
FAU - Marsden, Alison L
AU  - Marsden AL
AD  - 1 Department of Pediatrics (Cardiology), Stanford University, Stanford, CA, USA.
AD  - 2 Department of Bioengineering, Stanford University, Stanford, CA, USA.
FAU - Ogawa, Michelle T
AU  - Ogawa MT
AD  - 1 Department of Pediatrics (Cardiology), Stanford University, Stanford, CA, USA.
FAU - Sakarovitch, Charlotte
AU  - Sakarovitch C
AD  - 1 Department of Pediatrics (Cardiology), Stanford University, Stanford, CA, USA.
FAU - Hall, Keeley K
AU  - Hall KK
AD  - 1 Department of Pediatrics (Cardiology), Stanford University, Stanford, CA, USA.
FAU - Rabinovitch, Marlene
AU  - Rabinovitch M
AD  - 1 Department of Pediatrics (Cardiology), Stanford University, Stanford, CA, USA.
FAU - Feinstein, Jeffrey A
AU  - Feinstein JA
AD  - 1 Department of Pediatrics (Cardiology), Stanford University, Stanford, CA, USA.
AD  - 2 Department of Bioengineering, Stanford University, Stanford, CA, USA.
LA  - eng
GR  - P01 HL108797/HL/NHLBI NIH HHS/United States
GR  - R01 HL121754/HL/NHLBI NIH HHS/United States
GR  - R01 HL138473/HL/NHLBI NIH HHS/United States
PT  - Journal Article
DEP - 20180516
PL  - United States
TA  - Pulm Circ
JT  - Pulmonary circulation
JID - 101557243
PMC - PMC6432686
OTO - NOTNLM
OT  - clinical worsening
OT  - lumped parameter model
OT  - pressure-volume (P-V) loop
OT  - right ventricular failure
OT  - risk stratification
EDAT- 2018/05/17 06:00
MHDA- 2018/05/17 06:01
PMCR- 2018/05/16
CRDT- 2018/05/17 06:00
PHST- 2018/05/17 06:00 [pubmed]
PHST- 2018/05/17 06:01 [medline]
PHST- 2018/05/17 06:00 [entrez]
PHST- 2018/05/16 00:00 [pmc-release]
AID - 10.1177_2045894018780534 [pii]
AID - 10.1177/2045894018780534 [doi]
PST - ppublish
SO  - Pulm Circ. 2018 Jul-Sep;8(3):2045894018780534. doi: 10.1177/2045894018780534. 
      Epub 2018 May 16.