PMID- 28904266
OWN - NLM
STAT- MEDLINE
DCOM- 20190219
LR  - 20190320
IS  - 1347-4820 (Electronic)
IS  - 1346-9843 (Linking)
VI  - 82
IP  - 2
DP  - 2018 Jan 25
TI  - Clinical Impact of Main Pulmonary Artery Dilatation on Outcome in Pediatric 
      Idiopathic and Heritable Pulmonary Arterial Hypertension.
PG  - 541-545
LID - 10.1253/circj.CJ-17-0092 [doi]
AB  - BACKGROUND: Few studies have investigated the clinical impact of pulmonary artery 
      (PA) dilatation on outcomes in pediatric pulmonary arterial hypertension 
      (PAH).Methods and Results:This study investigated the clinical outcomes of 
      idiopathic or heritable PAH in 66 children aged <18 years at diagnosis. Main 
      PA/thorax (MPA/T) ratio was measured on chest radiography in PAH patients. 
      Patients were divided into 2 groups based on MPA/T ratio, and compared with a 
      control group of 166 age- and gender-matched healthy children. Group A had higher 
      MPA/T ratio than normal, and group B had normal MPA/T ratio. Composite outcomes 
      included cardiac death, lung transplantation, and hospitalization due to heart 
      failure. Group A consisted of 27 patients and group B, 39 patients. At diagnosis, 
      group A had significantly higher brain natriuretic peptide (BNP), cardiothoracic 
      ratio, PA pressure, and pulmonary vascular resistance index compared with group 
      B. The number of patients with New York Heart Association (NYHA) functional class 
      III and IV was significantly higher in group A than in group B. Cumulative 
      event-free survival rate was significantly lower in group A. CONCLUSIONS: MPA 
      dilatation correlated with BNP, NYHA functional class, and hemodynamics with 
      regard to disease severity, and may be a potential prognostic factor in pediatric 
      idiopathic and heritable PAH.
FAU - Ikehara, Satoshi
AU  - Ikehara S
AD  - Department of Pediatrics, Toho University Omori Medical Center.
FAU - Takatsuki, Shinichi
AU  - Takatsuki S
AD  - Department of Pediatrics, Toho University Omori Medical Center.
FAU - Nakayama, Tomotaka
AU  - Nakayama T
AD  - Department of Pediatrics, Toho University Omori Medical Center.
FAU - Naoi, Kazuyuki
AU  - Naoi K
AD  - Department of Pediatrics, Toho University Omori Medical Center.
FAU - Matsuura, Hiroyuki
AU  - Matsuura H
AD  - Department of Pediatrics, Toho University Omori Medical Center.
FAU - Saji, Tsutomu
AU  - Saji T
AD  - Department of Pediatrics, Toho University Omori Medical Center.
LA  - eng
PT  - Journal Article
PT  - Observational Study
DEP - 20170913
PL  - Japan
TA  - Circ J
JT  - Circulation journal : official journal of the Japanese Circulation Society
JID - 101137683
SB  - IM
MH  - Adolescent
MH  - Case-Control Studies
MH  - Child
MH  - Child, Preschool
MH  - Death
MH  - Dilatation, Pathologic/diagnostic imaging/mortality
MH  - Familial Primary Pulmonary Hypertension/*complications/mortality
MH  - Female
MH  - Hemodynamics
MH  - Hospitalization
MH  - Humans
MH  - Hypertension, Pulmonary/*complications/mortality
MH  - Infant
MH  - Male
MH  - Pulmonary Artery/*pathology
MH  - Radiography, Thoracic
MH  - Retrospective Studies
MH  - Survival Analysis
OTO - NOTNLM
OT  - Children
OT  - Main pulmonary artery dilatation
OT  - Outcome
OT  - Pulmonary hypertension
EDAT- 2017/09/15 06:00
MHDA- 2019/03/21 06:00
CRDT- 2017/09/15 06:00
PHST- 2017/09/15 06:00 [pubmed]
PHST- 2019/03/21 06:00 [medline]
PHST- 2017/09/15 06:00 [entrez]
AID - 10.1253/circj.CJ-17-0092 [doi]
PST - ppublish
SO  - Circ J. 2018 Jan 25;82(2):541-545. doi: 10.1253/circj.CJ-17-0092. Epub 2017 Sep 
      13.