2039 t2* cmr in hereditary hemochromatosis

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BioMed Central Page 1 of 2 (page number not for citation purposes) Journal of Cardiovascular Magnetic Resonance Open Access Meeting abstract 2039 T2* CMR in hereditary hemochromatosis Agata Grasso* 1 , Paul Kirk 1 , Mark Westwood 2 , Lisa Anderson 3 , Chiara Bucciarelli-Ducci 1 , Rory O'Hanlon 1 and Dudley Pennell 1 Address: 1 Royal Brompton Hospital, London, UK, 2 University College of London, London, UK and 3 St George's Hospital, London, UK * Corresponding author Background Hemochromatosis (HH) is a disease where the intestinal absorption of iron is high and multi-organ iron overload may result with organ failure. Dilated cardiomyopathy in this condition is considered a consequence of cardiac siderosis, but there is contradictory evidence for this. Car- diovascular Magnetic Resonance (CMR) detects iron dep- osition in the liver and in the heart with the T2* technique. Several studies have confirmed the normal range of iron in the liver and heart for normal subjects and the abnormal value above which heart failure is a com- mon risk. T2* is also useful to follow the chelation treat- ment of these patients and recent medical trials have confirmed the utility of deferiprone and deferoxamine to reduce the heart iron overload and increase the left ventri- cle systolic function in patients with thalassaemia. How- ever, while the direct link between cardiac siderosis and dilated cardiomyopathy is confirmed in thalassaemia, in HH the correlation between heart iron overload and dilated cardiomyopathy requires further investigation. In this study, we hypothesized the existence of a direct rela- tionship between myocardial iron deposition and dilated cardiomyopathy in HH. Methods We retrospectively analyzed 1016 consecutive patients presenting for the evaluation of myocardial and liver iron overload from 1996 to 2006. All these patients had a con- firmed diagnosis and were followed by specialized centers across the UK. Of this cohort, 53 patients had HH. Patients underwent cardiovascular magnetic resonance at 1.5 T (Siemens Sonata or Avanto, Erlangen, Germany). Each scan included the measurement of liver and heart T2*, and left and right ventricular function, volumes and mass using standard techniques with analysis using CMR- tools (Cardiovascular Imaging Solutions, London). Results The CMR characteristic (LV volumes, T2* of liver and heart) of the patients are shown in Table 1. The age of the patients varied from 36 to 76 years. All patients were on medical treatment appropriate to the presenting symp- toms from their HH. Figure 1 shows the scatterplot of myocardial T2* against LV EF. Six patients (11%) had myocardial iron loading, and of these, 3 had (50%) significant LV dysfunction (EF < 56%). A further 5 patients had impaired LV function with normal myocardial T2* values, and of these 4 (80%) from 11 th Annual SCMR Scientific Sessions Los Angeles, CA, USA. 1–3 February 2008 Published: 22 October 2008 Journal of Cardiovascular Magnetic Resonance 2008, 10(Suppl 1):A308 doi:10.1186/1532-429X-10-S1-A308 <supplement> <title> <p>Abstracts of the 11<sup>th </sup>Annual SCMR Scientific Sessions - 2008</p> </title> <note>Meeting abstracts – A single PDF containing all abstracts in this Supplement is available <a href="http://www.biomedcentral.com/content/files/pdf/1532-429X-10-s1-full.pdf">here</a>.</note> <url>http://www.biomedcentral.com/content/pdf/1532-429X-10-S1-info.pdf</url> </supplement> This abstract is available from: http://jcmr-online.com/content/10/S1/A308 © 2008 Grasso et al; licensee BioMed Central Ltd. Table 1: LVEDV LVESV LVEF LV mass Liver T2* Cardiac T2* Mean 95% CI 152 mL (138, 166) 58.5 mL (44.7, 72.2) 65.0% (60.7, 69.2) 151 g (138, 165) 11.3 ms (8.3, 4.2) 30.0 ms (26.6, 33.6)

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BioMed Central

Journal of Cardiovascular Magnetic Resonance

ss

Open AcceMeeting abstract2039 T2* CMR in hereditary hemochromatosisAgata Grasso*1, Paul Kirk1, Mark Westwood2, Lisa Anderson3, Chiara Bucciarelli-Ducci1, Rory O'Hanlon1 and Dudley Pennell1

Address: 1Royal Brompton Hospital, London, UK, 2University College of London, London, UK and 3St George's Hospital, London, UK

* Corresponding author

BackgroundHemochromatosis (HH) is a disease where the intestinalabsorption of iron is high and multi-organ iron overloadmay result with organ failure. Dilated cardiomyopathy inthis condition is considered a consequence of cardiacsiderosis, but there is contradictory evidence for this. Car-diovascular Magnetic Resonance (CMR) detects iron dep-osition in the liver and in the heart with the T2*technique. Several studies have confirmed the normalrange of iron in the liver and heart for normal subjects andthe abnormal value above which heart failure is a com-mon risk. T2* is also useful to follow the chelation treat-ment of these patients and recent medical trials haveconfirmed the utility of deferiprone and deferoxamine toreduce the heart iron overload and increase the left ventri-cle systolic function in patients with thalassaemia. How-ever, while the direct link between cardiac siderosis anddilated cardiomyopathy is confirmed in thalassaemia, inHH the correlation between heart iron overload anddilated cardiomyopathy requires further investigation. Inthis study, we hypothesized the existence of a direct rela-tionship between myocardial iron deposition and dilatedcardiomyopathy in HH.

MethodsWe retrospectively analyzed 1016 consecutive patientspresenting for the evaluation of myocardial and liver ironoverload from 1996 to 2006. All these patients had a con-firmed diagnosis and were followed by specialized centersacross the UK. Of this cohort, 53 patients had HH.Patients underwent cardiovascular magnetic resonance at1.5 T (Siemens Sonata or Avanto, Erlangen, Germany).Each scan included the measurement of liver and heartT2*, and left and right ventricular function, volumes andmass using standard techniques with analysis using CMR-tools (Cardiovascular Imaging Solutions, London).

ResultsThe CMR characteristic (LV volumes, T2* of liver andheart) of the patients are shown in Table 1. The age of thepatients varied from 36 to 76 years. All patients were onmedical treatment appropriate to the presenting symp-toms from their HH.

Figure 1 shows the scatterplot of myocardial T2* againstLV EF. Six patients (11%) had myocardial iron loading,and of these, 3 had (50%) significant LV dysfunction (EF< 56%). A further 5 patients had impaired LV functionwith normal myocardial T2* values, and of these 4 (80%)

from 11th Annual SCMR Scientific SessionsLos Angeles, CA, USA. 1–3 February 2008

Published: 22 October 2008

Journal of Cardiovascular Magnetic Resonance 2008, 10(Suppl 1):A308 doi:10.1186/1532-429X-10-S1-A308

<supplement> <title> <p>Abstracts of the 11<sup>th </sup>Annual SCMR Scientific Sessions - 2008</p> </title> <note>Meeting abstracts – A single PDF containing all abstracts in this Supplement is available <a href="http://www.biomedcentral.com/content/files/pdf/1532-429X-10-s1-full.pdf">here</a>.</note> <url>http://www.biomedcentral.com/content/pdf/1532-429X-10-S1-info.pdf</url> </supplement>

This abstract is available from: http://jcmr-online.com/content/10/S1/A308

© 2008 Grasso et al; licensee BioMed Central Ltd.

Table 1:

LVEDV LVESV LVEF LV mass Liver T2* Cardiac T2*

Mean 95% CI 152 mL (138, 166) 58.5 mL (44.7, 72.2) 65.0% (60.7, 69.2) 151 g (138, 165) 11.3 ms (8.3, 4.2) 30.0 ms (26.6, 33.6)

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Journal of Cardiovascular Magnetic Resonance 2008, 10(Suppl 1):A308 http://jcmr-online.com/content/10/S1/A308

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had an alternative explanation for the dysfunction (2patients with previous anterior infarction, 1 with previ-ously diagnosed idiopathic dilated cardiomyopathy, and1 with arrhythmia).

ConclusionThese findings suggest that in patients with HH, cardiacsiderosis is a relatively uncommon complication (11%),which directly results in impaired LV function cardiomy-opathy in half of these cases (6%).

Figure 1

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