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Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort—A Cardiac Magnetic Resonance Study
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...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9689750/ https://www.ncbi.nlm.nih.gov/pubmed/36359463 http://dx.doi.org/10.3390/diagnostics12112620 |
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author | Dorniak, Karolina Daniłowicz-Szymanowicz, Ludmiła Sikorska, Katarzyna Rozwadowska, Katarzyna Fijałkowska, Jadwiga Glińska, Anna Tuzimek, Magdalena Sabisz, Agnieszka Żarczyńska-Buchowiecka, Marta Świątczak, Michał Dudziak, Maria Szurowska, Edyta |
author_facet | Dorniak, Karolina Daniłowicz-Szymanowicz, Ludmiła Sikorska, Katarzyna Rozwadowska, Katarzyna Fijałkowska, Jadwiga Glińska, Anna Tuzimek, Magdalena Sabisz, Agnieszka Żarczyńska-Buchowiecka, Marta Świątczak, Michał Dudziak, Maria Szurowska, Edyta |
author_sort | Dorniak, Karolina |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-9689750 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-96897502022-11-25 Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort—A Cardiac Magnetic Resonance Study Dorniak, Karolina Daniłowicz-Szymanowicz, Ludmiła Sikorska, Katarzyna Rozwadowska, Katarzyna Fijałkowska, Jadwiga Glińska, Anna Tuzimek, Magdalena Sabisz, Agnieszka Żarczyńska-Buchowiecka, Marta Świątczak, Michał Dudziak, Maria Szurowska, Edyta Diagnostics (Basel) Article 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. MDPI 2022-10-28 /pmc/articles/PMC9689750/ /pubmed/36359463 http://dx.doi.org/10.3390/diagnostics12112620 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Dorniak, Karolina Daniłowicz-Szymanowicz, Ludmiła Sikorska, Katarzyna Rozwadowska, Katarzyna Fijałkowska, Jadwiga Glińska, Anna Tuzimek, Magdalena Sabisz, Agnieszka Żarczyńska-Buchowiecka, Marta Świątczak, Michał Dudziak, Maria Szurowska, Edyta Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort—A Cardiac Magnetic Resonance Study |
title | Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort—A Cardiac Magnetic Resonance Study |
title_full | Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort—A Cardiac Magnetic Resonance Study |
title_fullStr | Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort—A Cardiac Magnetic Resonance Study |
title_full_unstemmed | Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort—A Cardiac Magnetic Resonance Study |
title_short | Left Ventricular Function and Iron Loading Status in a Tertiary Center Hemochromatosis Cohort—A Cardiac Magnetic Resonance Study |
title_sort | left ventricular function and iron loading status in a tertiary center hemochromatosis cohort—a cardiac magnetic resonance study |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9689750/ https://www.ncbi.nlm.nih.gov/pubmed/36359463 http://dx.doi.org/10.3390/diagnostics12112620 |
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