PMID- 25605038
OWN - NLM
STAT- MEDLINE
DCOM- 20160407
LR  - 20181113
IS  - 1432-1971 (Electronic)
IS  - 0172-0643 (Linking)
VI  - 36
IP  - 5
DP  - 2015 Jun
TI  - Quantification and significance of diffuse myocardial fibrosis and diastolic 
      dysfunction in childhood hypertrophic cardiomyopathy.
PG  - 970-8
LID - 10.1007/s00246-015-1107-7 [doi]
AB  - The purpose of this study was to evaluate the presence of diffuse myocardial 
      fibrosis in children and adolescents with hypertrophic cardiomyopathy (HCM) and 
      to assess associations with echocardiographic and clinical parameters of disease. 
      While a common end point in adults with HCM, it is unclear whether diffuse 
      myocardial fibrosis occurs early in the disease. Cardiac magnetic resonance (CMR) 
      estimation of myocardial post-contrast longitudinal relaxation time (T1) is an 
      increasingly used method to estimate diffuse fibrosis. T1 measurements were taken 
      using standard multi-breath-hold spoiled gradient echo phase-sensitive 
      inversion-recovery CMR before and 15 min after the injection of gadolinium. The 
      tissue-blood partition coefficient was calculated as a function of the ratio of 
      T1 change of myocardium compared with blood. An echocardiogram and blood brain 
      natriuretic peptide (BNP) levels were obtained on the day of the CMR. Twelve 
      controls (mean age 12.8 years; 7 male) and 28 patients with HCM (mean age 12.8 
      years; 21 male) participated. The partition coefficient for both septal (0.27 +/- 
      0.17 vs. 0.13 +/- 0.09; p = 0.03) and lateral walls (0.22 +/- 0.09 vs. 0.07 +/- 0.10; p 
      < 0.001) was increased in patients compared with controls. Eight patients had 
      overt areas of late gadolinium enhancement (LGE). These patients did not show 
      increased partition coefficient compared with those without LGE (0.27 +/- 0.15 vs. 
      0.27 +/- 0.19 and 0.22 +/- 0.09 vs. 0.22 +/- 0.09; p = 0.95 and 0.98, respectively). 
      However, patients who were symptomatic (dyspnea, arrhythmia and/or chest pain) 
      had higher lateral wall partition coefficient than asymptomatic HCM patients 
      (0.27 +/- 0.08 vs. 0.17 +/- 0.08; p = 0.006). Similarly, patients with raised BNP 
      (>100 pg/ml) had raised lateral wall coefficients (0.27 +/- 0.07 vs. 0.20 +/- 0.07; p 
      = 0.03), as did those with traditional risk factors for sudden death (0.27 +/- 0.06 
      vs. 0.18 +/- 0.08; p = 0.007). Diffuse fibrosis, measured by the partition 
      coefficient technique, is demonstrable in children and adolescents with HCM. 
      Markers of fibrosis show an association with symptoms and raised serum BNP. 
      Further study of the prognostic implication of this technique in young patients 
      with HCM is warranted.
FAU - Hussain, Tarique
AU  - Hussain T
AD  - Department of Pediatrics, Labatt Family Heart Centre, The Hospital for Sick 
      Children, University of Toronto, Toronto, Canada, tarique@doctors.org.uk.
FAU - Dragulescu, Andreea
AU  - Dragulescu A
FAU - Benson, Lee
AU  - Benson L
FAU - Yoo, Shi-Joon
AU  - Yoo SJ
FAU - Meng, Howard
AU  - Meng H
FAU - Windram, Jonathan
AU  - Windram J
FAU - Wong, Derek
AU  - Wong D
FAU - Greiser, Andreas
AU  - Greiser A
FAU - Friedberg, Mark
AU  - Friedberg M
FAU - Mertens, Luc
AU  - Mertens L
FAU - Seed, Michael
AU  - Seed M
FAU - Redington, Andrew
AU  - Redington A
FAU - Grosse-Wortmann, Lars
AU  - Grosse-Wortmann L
LA  - eng
PT  - Journal Article
DEP - 20150121
PL  - United States
TA  - Pediatr Cardiol
JT  - Pediatric cardiology
JID - 8003849
RN  - 0 (Contrast Media)
RN  - K2I13DR72L (Gadolinium DTPA)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Cardiomyopathy, Hypertrophic/diagnostic imaging/*pathology
MH  - Child
MH  - Contrast Media
MH  - *Diastole
MH  - Echocardiography
MH  - Female
MH  - Fibrosis
MH  - Gadolinium DTPA
MH  - Humans
MH  - Magnetic Resonance Imaging/*methods
MH  - Male
MH  - Myocardium/*pathology
MH  - Ventricular Dysfunction/diagnostic imaging/*pathology
EDAT- 2015/01/22 06:00
MHDA- 2016/04/08 06:00
CRDT- 2015/01/22 06:00
PHST- 2014/10/08 00:00 [received]
PHST- 2015/01/13 00:00 [accepted]
PHST- 2015/01/22 06:00 [entrez]
PHST- 2015/01/22 06:00 [pubmed]
PHST- 2016/04/08 06:00 [medline]
AID - 10.1007/s00246-015-1107-7 [doi]
PST - ppublish
SO  - Pediatr Cardiol. 2015 Jun;36(5):970-8. doi: 10.1007/s00246-015-1107-7. Epub 2015 
      Jan 21.