PMID- 21241474
OWN - NLM
STAT- MEDLINE
DCOM- 20110404
LR  - 20240117
IS  - 1532-429X (Electronic)
IS  - 1097-6647 (Print)
IS  - 1097-6647 (Linking)
VI  - 13
IP  - 1
DP  - 2011 Jan 17
TI  - Low prevalence of fibrosis in thalassemia major assessed by late gadolinium 
      enhancement cardiovascular magnetic resonance.
PG  - 8
LID - 10.1186/1532-429X-13-8 [doi]
AB  - BACKGROUND: Heart failure remains a major cause of mortality in thalassaemia 
      major. The possible role of cardiac fibrosis in thalassemia major in the genesis 
      of heart failure is not clear. It is also unclear whether cardiac fibrosis might 
      arise as a result of heart failure. METHODS: We studied 45 patients with 
      thalassaemia major who had a wide range of current cardiac iron loading and 
      included patients with prior and current heart failure. Myocardial iron was 
      measured using T2* cardiovascular magnetic resonance (CMR), and following this, 
      late gadolinium enhancement (LGE) was used to determine the presence of 
      macroscopic myocardial fibrosis. RESULTS: The median myocardial T2* in all 
      patients was 22.6 ms (range 5.3-58.8 ms). Fibrosis was detected in only one 
      patient, whose myocardial T2* was 20.1 ms and left ventricular ejection fraction 
      57%. No fibrosis was identified in 5 patients with a history of heart failure 
      with full recovery, in 3 patients with current left ventricular dysfunction 
      undergoing treatment, or in 18 patients with myocardial iron loading with 
      cardiacT2* < 20 ms at the time of scan. CONCLUSION: This study shows that 
      macroscopic myocardial fibrosis is uncommon in thalassemia major across a broad 
      spectrum of myocardial iron loading. Importantly, there was no macroscopic 
      fibrosis in patients with current or prior heart failure, or in patients with 
      myocardial iron loading without heart failure. Therefore if myocardial fibrosis 
      indeed contributes to myocardial dysfunction in thalassemia, our data combined 
      with the knowledge that the myocardial dysfunction of iron overload can be 
      reversed, indicates that any such fibrosis would need to be both microscopic and 
      reversible.
FAU - Kirk, Paul
AU  - Kirk P
AD  - Royal Brompton Hospital and Imperial College, London, UK.
FAU - Carpenter, John Paul
AU  - Carpenter JP
FAU - Tanner, Mark A
AU  - Tanner MA
FAU - Pennell, Dudley J
AU  - Pennell DJ
LA  - eng
GR  - PG/09/074/27961/BHF_/British Heart Foundation/United Kingdom
GR  - 5 R01 DK066084-02/DK/NIDDK NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20110117
PL  - England
TA  - J Cardiovasc Magn Reson
JT  - Journal of cardiovascular magnetic resonance : official journal of the Society 
      for Cardiovascular Magnetic Resonance
JID - 9815616
RN  - 0 (Contrast Media)
RN  - E1UOL152H7 (Iron)
RN  - K2I13DR72L (Gadolinium DTPA)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - *Contrast Media
MH  - England
MH  - Female
MH  - Fibrosis
MH  - *Gadolinium DTPA
MH  - Heart Failure/etiology/metabolism/*pathology/physiopathology
MH  - Humans
MH  - Iron/metabolism
MH  - *Magnetic Resonance Imaging
MH  - Male
MH  - Myocardium/metabolism/*pathology
MH  - Predictive Value of Tests
MH  - Prevalence
MH  - Stroke Volume
MH  - Ventricular Dysfunction, Left/etiology/pathology
MH  - Ventricular Function, Left
MH  - Young Adult
MH  - beta-Thalassemia/*complications/metabolism/pathology/physiopathology
PMC - PMC3025880
EDAT- 2011/01/19 06:00
MHDA- 2011/04/05 06:00
PMCR- 2011/01/17
CRDT- 2011/01/19 06:00
PHST- 2011/01/04 00:00 [received]
PHST- 2011/01/17 00:00 [accepted]
PHST- 2011/01/19 06:00 [entrez]
PHST- 2011/01/19 06:00 [pubmed]
PHST- 2011/04/05 06:00 [medline]
PHST- 2011/01/17 00:00 [pmc-release]
AID - S1097-6647(23)01363-7 [pii]
AID - 1532-429X-13-8 [pii]
AID - 10.1186/1532-429X-13-8 [doi]
PST - epublish
SO  - J Cardiovasc Magn Reson. 2011 Jan 17;13(1):8. doi: 10.1186/1532-429X-13-8.