PMID- 27810156
OWN - NLM
STAT- MEDLINE
DCOM- 20170615
LR  - 20181013
IS  - 1097-6833 (Electronic)
IS  - 0022-3476 (Linking)
VI  - 180
DP  - 2017 Jan
TI  - Pulmonary Arterial Capacitance Index Is a Strong Predictor for Adverse Outcome in 
      Children with Idiopathic and Heritable Pulmonary Arterial Hypertension.
PG  - 75-79.e2
LID - S0022-3476(16)31060-5 [pii]
LID - 10.1016/j.jpeds.2016.10.003 [doi]
AB  - OBJECTIVES: To evaluate the clinical utility of pulmonary artery capacitance 
      index (PACi) in the assessment of disease severity and prognostic value in 
      children with idiopathic and heritable pulmonary arterial hypertension (PAH). 
      STUDY DESIGN: PACi is defined as the ratio of stroke volume index over pulmonary 
      pulse pressure. A retrospective study was performed to compare PACi, brain 
      natriuretic peptide (BNP), 6-minute walk distance, New York Heart association 
      (NYHA) functional class, and adverse outcomes (hospitalization due to heart 
      failure, lung transplantation, and cardiac mortality) in 72 Japanese children 
      (10 +/- 3.6 years) with idiopathic and heritable PAH. RESULTS: PACi had significant 
      correlations with pulmonary vascular resistance index (r =-0.73, P < .0001), BNP 
      levels (r = -0.40, P = .0008), and 6-minute walk distance (r = 0.57, P < .05). 
      Statistically significant differences in PACi were observed between NYHA 
      functional class II vs combined III and IV (median; 1.1 vs 0.6 mL/mm Hg/m(2), 
      respectively, P < .05). There were 25 of 72 (35%) children who had an adverse 
      event including initiation of hospitalization due to heart failure, lung 
      transplantation, and death. Cumulative event-free survival rate was significantly 
      lower when PACi was <0.85 mL/mm Hg/m(2) (log-rank test, P < .0001). CONCLUSIONS: 
      PACi correlated with BNP and NYHA functional class and may serve as a strong 
      prognostic marker in children with idiopathic and heritable PAH.
CI  - Copyright (c) 2016 Elsevier Inc. All rights reserved.
FAU - Takatsuki, Shinichi
AU  - Takatsuki S
AD  - Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan.
FAU - Nakayama, Tomotaka
AU  - Nakayama T
AD  - Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan.
FAU - Ikehara, Satoshi
AU  - Ikehara S
AD  - Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan.
FAU - Matsuura, Hiroyuki
AU  - Matsuura H
AD  - Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan.
FAU - Ivy, David Dunbar
AU  - Ivy DD
AD  - Department of Pediatrics, Children's Hospital Colorado, University of Colorado 
      School of Medicine, Aurora, CO.
FAU - Saji, Tsutomu
AU  - Saji T
AD  - Department of Pediatrics, Toho University Omori Medical Center, Tokyo, Japan.
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20161031
PL  - United States
TA  - J Pediatr
JT  - The Journal of pediatrics
JID - 0375410
SB  - IM
CIN - J Pediatr. 2017 May;184:240. PMID: 28233545
CIN - J Pediatr. 2017 May;184:239-240. PMID: 28434572
MH  - Child
MH  - Familial Primary Pulmonary Hypertension/*physiopathology
MH  - Female
MH  - Humans
MH  - Male
MH  - Predictive Value of Tests
MH  - Prognosis
MH  - Pulmonary Artery/*physiopathology
MH  - Retrospective Studies
MH  - Severity of Illness Index
MH  - *Vascular Capacitance
OTO - NOTNLM
OT  - idiopathic pulmonary arterial hypertension
OT  - pulmonary arterial capacitance index
OT  - right ventricular failure
OT  - right ventricular-pulmonary arterial coupling
EDAT- 2016/11/05 06:00
MHDA- 2017/06/16 06:00
CRDT- 2016/11/05 06:00
PHST- 2016/03/30 00:00 [received]
PHST- 2016/08/03 00:00 [revised]
PHST- 2016/10/03 00:00 [accepted]
PHST- 2016/11/05 06:00 [pubmed]
PHST- 2017/06/16 06:00 [medline]
PHST- 2016/11/05 06:00 [entrez]
AID - S0022-3476(16)31060-5 [pii]
AID - 10.1016/j.jpeds.2016.10.003 [doi]
PST - ppublish
SO  - J Pediatr. 2017 Jan;180:75-79.e2. doi: 10.1016/j.jpeds.2016.10.003. Epub 2016 Oct 
      31.