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遗传性血色素沉着症对左心室壁厚度的影响:铁过载会加重心肌肥厚吗?

Influence of hereditary haemochromatosis on left ventricular wall thickness: does iron overload exacerbate cardiac hypertrophy?

作者信息

Rozwadowska K, Raczak G, Sikorska K, Fijałkowski M, Kozłowski D, Daniłowicz-Szymanowicz L

机构信息

Clinical Centre of Cardiology, University Clinical Centre, Gdańsk, Poland.

2nd Department of Cardiology, Medical University of Gdańsk, Poland.

出版信息

Folia Morphol (Warsz). 2019;78(4):746-753. doi: 10.5603/FM.a2019.0025. Epub 2019 Mar 5.

DOI:10.5603/FM.a2019.0025
PMID:30835340
Abstract

BACKGROUND

The left ventricular (LV) hypertrophy increases the risk of heart failure. Hypertension and infiltrative cardiomyopathies are the well-known reasons of LV hypertrophy. The growing interest of scientists in this issue affects hereditary haemochromatosis (HH), which is characterised by the excess deposition of iron mostly due to HFE gene mutation. The aim of our study was to investigate the possible influence of HH on LV parameters in patients with early-diagnosed (early HH) and long-lasting and long-treated (old HH) disease.

MATERIALS AND METHODS

Thirty nine early HH and 19 old HH patients were prospectively enrolled in the study; age- and sex-matched healthy volunteers constituted the appropriate control groups. All participants had echocardiography performed (including three-dimension volume and mass analysis); the iron turnover parameters were measured at the time of enrolment in every HH patients.

RESULTS

Echocardiographic parameters regarding to left atrium (LA), LV thickness, mass and long axis length were significantly higher, whereas LV ejection fraction was lower in early HH in comparison to healthy persons. In old HH patients the differences were similar to those mentioned before, except LV ejection fraction. The presence of hypertension in both HH groups did not influence echo parameters, as well as diabetes in old HH. The strongest correlation in all HH group was found between the time from HH diagnosis and LA, LV thickness and volumes parameters, but the correlations between iron turnover and echo parameters were non-existent.

CONCLUSIONS

Hereditary haemochromatosis, not only long-lasting, but also early-diagnosed, could lead to exacerbation of LV wall thickness and cardiac hypertrophy. This effect is not simply connected with hypertension and diabetes that are frequent additional diseases in these patients, but with the time from HH diagnosis.

摘要

背景

左心室肥厚会增加心力衰竭的风险。高血压和浸润性心肌病是左心室肥厚的常见原因。科学家们对这个问题的兴趣日益浓厚,这涉及到遗传性血色素沉着症(HH),其特征是主要由于HFE基因突变导致铁过度沉积。我们研究的目的是调查HH对早期诊断(早期HH)和病程长且治疗时间长(老年HH)患者左心室参数的可能影响。

材料与方法

前瞻性纳入39例早期HH患者和19例老年HH患者;年龄和性别匹配的健康志愿者组成相应的对照组。所有参与者均接受了超声心动图检查(包括三维容积和质量分析);在每位HH患者入组时测量铁代谢参数。

结果

与健康人相比,早期HH患者左心房(LA)、左心室厚度、质量和长轴长度的超声心动图参数显著更高,而左心室射血分数更低。在老年HH患者中,除左心室射血分数外,差异与上述相似。两个HH组中高血压的存在以及老年HH患者中的糖尿病均未影响超声心动图参数。在所有HH组中,HH诊断时间与LA、左心室厚度和容积参数之间的相关性最强,但铁代谢与超声心动图参数之间不存在相关性。

结论

遗传性血色素沉着症,不仅病程长,而且早期诊断,都可能导致左心室壁厚度增加和心脏肥大加剧。这种影响不仅仅与这些患者中常见的合并疾病高血压和糖尿病有关,还与HH诊断后的时间有关。

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