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Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort-A Cardiac Magnetic
Karolina Dorniak1, Ludmiła Daniłowicz-Szymanowicz2, Katarzyna Sikorska3
1Department of Noninvasive Cardiac Diagnostics, Medical University of Gdansk, Dębinki 7, 80-210 Gdansk, Poland.
Insights
In hemochromatosis (HCH), significant heart iron overload is uncommon. However, reduced heart or liver iron measurements may signal future risk, warranting close monitoring in HCH patients.
Area of Science:
- Cardiology
- Genetics
- Radiology
Background:
- Hereditary hemochromatosis (HCH) is a common genetic disorder causing progressive iron overload.
- Organ iron loading and cardiac function in HCH patients are not fully understood.
- This study prospectively evaluated iron deposition and cardiac function in an HCH cohort.
Purpose of the Study:
- To assess organ iron loading and cardiac function in HCH patients.
- To investigate the utility of cardiac magnetic resonance (CMR) for iron screening.
Main Methods:
- 42 HCH patients and 36 controls underwent laboratory tests and CMR with T1 and T2* mapping.
- Cardiac and liver iron levels were measured using T2* mapping.
- Correlation between tissue iron parameters and ferritin levels was analyzed.
Main Results:
- HCH patients had lower myocardial T2* (myoT2*), myocardial T1 (myoT1), and liver T2* (livT2*) than controls.
- No patients exhibited clinically significant myocardial iron overload (myoT2* < 20 ms).
- 18 patients (42.9%) had reduced livT2*, and 10 (23.8%) had reduced myoT2* and/or myoT1, with 4 having normal livT2*.
Conclusions:
- Significant myocardial iron overload is rare in contemporary HCH patients.
- Reduced myocardial T1/T2* or liver T2* may indicate risk for accelerated iron overload, even with normal liver iron.
- CMR T2* mapping can screen for liver iron concurrently with cardiac assessment.
Abstract:
Background: Haemochromatosis (HCH), a common genetic disorder with variable penetrance, results in progressive but understudied iron overload. We prospectively evaluated organ iron loading and cardiac function in a tertiary center HCH cohort. Methods: 42 HCH patients (47 ± 14 years) and 36 controls underwent laboratory workup and cardiac magnetic resonance (CMR), including T1 and T2* mapping. Results: Myocardial T2* (myoT2*), myocardial T1 (myoT1) and liver T2* (livT2*) were lower in patients compared to controls (33 ± 4 ms vs. 36 ± 3 ms [p = 0.004], 964 ± 33 ms vs. 979 ± 25 ms [p = 0.028] and 21 ± 10 ms vs. 30 ± 5 ms [p < 0.001], respectively). MyoT2* did not reach the threshold of clinically significant iron overload (<20 ms), in any of the patients. In 22 (52.4%) patients, at least one of the tissue parameters was reduced. Reduced myocardial T2* and/or T1 were found in 10 (23.8%) patients, including 4 pts with normal livT2*. LivT2* was reduced in 18 (42.9%) patients. MyoT1 and livT2* inversely correlated with ferritin (rs = −0.351 [p = 0.028] and rs = −0.602 [p < 0.001], respectively). LivT2* by a dedicated sequence and livT2* by cardiac T2* mapping showed good agreement (ICC = 0.876 p < 0.001). Conclusions: In contemporary hemochromatosis, significant myocardial iron overload is rare. Low myocardial T2* and/or T1 values may warrant closer follow-up for accelerated myocardial iron overload even in patients without overt liver overload. Cardiac T2* mapping sequence allows for liver screening at the time of CMR.
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