How early can myocardial iron overload occur in beta thalassemia major?

Myocardial siderosis is the most common cause of death in patients with beta thalassemia major(TM). This study aimed at investigating the occurrence, prevalence and severity of cardiac iron overload in a young Chinese population with beta TM.We analyzed T2* cardiac magnetic resonance (CMR), left ven...

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Main Authors: Gaohui Yang, Rongrong Liu, Peng Peng, Liling Long, Xinhua Zhang, Weijia Yang, Shaohong Tan, Hongfei Pan, Xingjiang Long, Taigang He, Lisa Anderson, Yongrong Lai
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC3899006?pdf=render
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author Gaohui Yang
Rongrong Liu
Peng Peng
Liling Long
Xinhua Zhang
Weijia Yang
Shaohong Tan
Hongfei Pan
Xingjiang Long
Taigang He
Lisa Anderson
Yongrong Lai
author_facet Gaohui Yang
Rongrong Liu
Peng Peng
Liling Long
Xinhua Zhang
Weijia Yang
Shaohong Tan
Hongfei Pan
Xingjiang Long
Taigang He
Lisa Anderson
Yongrong Lai
author_sort Gaohui Yang
collection DOAJ
description Myocardial siderosis is the most common cause of death in patients with beta thalassemia major(TM). This study aimed at investigating the occurrence, prevalence and severity of cardiac iron overload in a young Chinese population with beta TM.We analyzed T2* cardiac magnetic resonance (CMR), left ventricular ejection fraction (LVEF) and serum ferritin (SF) in 201 beta TM patients. The median age was 9 years old. Patients received an average of 13 units of blood per year. The median SF level was 4536 ng/ml and 165 patients (82.1%) had SF>2500 ng/ml. Myocardial iron overload was detected in 68 patients (33.8%) and severe myocardial iron overload was detected in 26 patients (12.6%). Twenty-two patients ≤10 years old had myocardial iron overload, three of whom were only 6 years old. No myocardial iron overload was detected under the age of 6 years. Median LVEF was 64% (measured by CMR in 175 patients). Five of 6 patients with a LVEF<56% and 8 of 10 patients with cardiac disease had myocardial iron overload.The TM patients under follow-up at this regional centre in China patients are younger than other reported cohorts, more poorly-chelated, and have a high burden of iron overload. Myocardial siderosis occurred in patients younger than previously reported, and was strongly associated with impaired LVEF and cardiac disease. For such poorly-chelated TM patients, our data shows that the first assessment of cardiac T2* should be performed as early as 6 years old.
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spelling doaj.art-ddd0506f4b24471caf315f642f28cf5f2022-12-22T01:36:31ZengPublic Library of Science (PLoS)PLoS ONE1932-62032014-01-0191e8537910.1371/journal.pone.0085379How early can myocardial iron overload occur in beta thalassemia major?Gaohui YangRongrong LiuPeng PengLiling LongXinhua ZhangWeijia YangShaohong TanHongfei PanXingjiang LongTaigang HeLisa AndersonYongrong LaiMyocardial siderosis is the most common cause of death in patients with beta thalassemia major(TM). This study aimed at investigating the occurrence, prevalence and severity of cardiac iron overload in a young Chinese population with beta TM.We analyzed T2* cardiac magnetic resonance (CMR), left ventricular ejection fraction (LVEF) and serum ferritin (SF) in 201 beta TM patients. The median age was 9 years old. Patients received an average of 13 units of blood per year. The median SF level was 4536 ng/ml and 165 patients (82.1%) had SF>2500 ng/ml. Myocardial iron overload was detected in 68 patients (33.8%) and severe myocardial iron overload was detected in 26 patients (12.6%). Twenty-two patients ≤10 years old had myocardial iron overload, three of whom were only 6 years old. No myocardial iron overload was detected under the age of 6 years. Median LVEF was 64% (measured by CMR in 175 patients). Five of 6 patients with a LVEF<56% and 8 of 10 patients with cardiac disease had myocardial iron overload.The TM patients under follow-up at this regional centre in China patients are younger than other reported cohorts, more poorly-chelated, and have a high burden of iron overload. Myocardial siderosis occurred in patients younger than previously reported, and was strongly associated with impaired LVEF and cardiac disease. For such poorly-chelated TM patients, our data shows that the first assessment of cardiac T2* should be performed as early as 6 years old.http://europepmc.org/articles/PMC3899006?pdf=render
spellingShingle Gaohui Yang
Rongrong Liu
Peng Peng
Liling Long
Xinhua Zhang
Weijia Yang
Shaohong Tan
Hongfei Pan
Xingjiang Long
Taigang He
Lisa Anderson
Yongrong Lai
How early can myocardial iron overload occur in beta thalassemia major?
PLoS ONE
title How early can myocardial iron overload occur in beta thalassemia major?
title_full How early can myocardial iron overload occur in beta thalassemia major?
title_fullStr How early can myocardial iron overload occur in beta thalassemia major?
title_full_unstemmed How early can myocardial iron overload occur in beta thalassemia major?
title_short How early can myocardial iron overload occur in beta thalassemia major?
title_sort how early can myocardial iron overload occur in beta thalassemia major
url http://europepmc.org/articles/PMC3899006?pdf=render
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