PMID- 20186992
OWN - NLM
STAT- MEDLINE
DCOM- 20100722
LR  - 20211020
IS  - 1932-8737 (Electronic)
IS  - 0160-9289 (Print)
IS  - 0160-9289 (Linking)
VI  - 33
IP  - 2
DP  - 2010 Feb
TI  - Restrictive right ventricular physiology and right ventricular fibrosis as 
      assessed by cardiac magnetic resonance and exercise capacity after biventricular 
      repair of pulmonary atresia and intact ventricular septum.
PG  - 104-10
LID - 10.1002/clc.20711 [doi]
AB  - BACKGROUND: The hypertrophic myocardium, myocardial fiber disarray, and 
      endocardial fibroelastosis in pulmonary atresia and intact ventricular septum 
      (PAIVS) may provide anatomic substrates for restrictive filling of the right 
      ventricle. HYPOTHESIS: Restrictive right ventricle (RV) physiology is related to 
      RV fibrosis and exercise capacity in patients after biventricular repair of 
      PAIVS. METHODS: A total of 27 patients, age 16.5 +/- 5.6 years, were recruited 
      after biventricular repair of PAIVS. Restrictive RV physiology was defined by the 
      presence of antegrade diastolic pulmonary flow and RV fibrosis assessed by late 
      gadolinium enhancement (LGE) cardiac magnetic resonance. Their RV function was 
      compared with that of 27 healthy controls and related to RV LGE score and 
      exercise capacity. RESULTS: Compared with controls, PAIVS patients had lower 
      tricuspid annular systolic and early diastolic velocities, RV global longitudinal 
      systolic strain, systolic strain rate, and early and late diastolic strain rates 
      (all P < 0.05). A total of 22 (81%, 95% confidence interval: 62%-94%) PAIVS 
      patients demonstrated restrictive RV physiology. Compared to those without 
      restrictive RV physiology (n = 5), these 22 patients had lower RV global systolic 
      strain, lower RV systolic and early diastolic strain rates, higher RV LGE score, 
      and a greater percent of predicted maximum oxygen consumption (all P < 0.05). 
      CONCLUSION: Restrictive RV physiology reflects RV diastolic dysfunction and is 
      associated with more severe RV fibrosis but better exercise capacity in patients 
      after biventricular repair of PAIVS.
CI  - Copyright 2010 Wiley Periodicals, Inc.
FAU - Liang, Xue-Cun
AU  - Liang XC
AD  - Division of Pediatric Cardiology, Department of Pediatrics and Adolescent 
      Medicine, Queen Mary Hospital, University of Hong Kong, Hong Kong, China.
FAU - Lam, W W M
AU  - Lam WW
FAU - Cheung, E W Y
AU  - Cheung EW
FAU - Wu, A K P
AU  - Wu AK
FAU - Wong, Sophia J
AU  - Wong SJ
FAU - Cheung, Yiu-Fai
AU  - Cheung YF
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Clin Cardiol
JT  - Clinical cardiology
JID - 7903272
RN  - 0 (Contrast Media)
RN  - K2I13DR72L (Gadolinium DTPA)
SB  - IM
MH  - Adolescent
MH  - *Cardiac Surgical Procedures/adverse effects
MH  - Cardiomyopathy, Restrictive/*diagnosis/etiology/physiopathology
MH  - Case-Control Studies
MH  - Catheterization
MH  - Contrast Media
MH  - Echocardiography, Doppler
MH  - *Exercise Test
MH  - *Exercise Tolerance
MH  - Female
MH  - Fibrosis
MH  - Gadolinium DTPA
MH  - Heart Ventricles/pathology/physiopathology
MH  - Humans
MH  - *Magnetic Resonance Imaging
MH  - Male
MH  - Oxygen Consumption
MH  - Predictive Value of Tests
MH  - Pulmonary Atresia/complications/diagnosis/physiopathology/*surgery
MH  - Pulmonary Circulation
MH  - Severity of Illness Index
MH  - Treatment Outcome
MH  - Ventricular Dysfunction, Right/*diagnosis/etiology/physiopathology
MH  - *Ventricular Function, Right
MH  - Young Adult
PMC - PMC6653126
EDAT- 2010/02/27 06:00
MHDA- 2010/07/23 06:00
PMCR- 2010/02/23
CRDT- 2010/02/27 06:00
PHST- 2010/02/27 06:00 [entrez]
PHST- 2010/02/27 06:00 [pubmed]
PHST- 2010/07/23 06:00 [medline]
PHST- 2010/02/23 00:00 [pmc-release]
AID - CLC20711 [pii]
AID - 10.1002/clc.20711 [doi]
PST - ppublish
SO  - Clin Cardiol. 2010 Feb;33(2):104-10. doi: 10.1002/clc.20711.