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Improvements in cardiac function detected using echocardiography in patients with hereditary haemochromatosis

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Irish Journal of Medical Science (1971 -) Aims and scope Submit manuscript

Abstract

Background

Hereditary haemochromatosis is often not diagnosed until adulthood. Iron overload cardiomyopathy initially results in diastolic dysfunction and can result in arrhythmias and irreversible cardiac failure if untreated. The aim of this study was to investigate whether patients with newly diagnosed hereditary haemochromatosis without signs of heart failure exhibit subclinical alterations of cardiac function and to determine if cardiac function improved after 1 year of venesection.

Methods

Baseline echocardiography was performed on 25 patients with newly diagnosed hereditary haemochromatosis with elevated serum ferritin levels. The test was repeated after 1 year of treatment with venesection. Tissue Doppler imaging (TDI) and deformation (strain) imaging using speckle tracking were performed. Left atrial force was measured according to the Newtonian principle, in which force (dynes) = mass × acceleration. Left atrial force was calculated by the Manning method expressed as ρ × 0.53 × mitral annular orifice area × (peak A velocity)2.

Results

Radial strain showed a significant improvement after 1 year of venesection (increase from 38.8 to 52.6). The LAF showed a significant decrease after 1 year of venesection (median decrease = 0.6 (IQR 0, 1.60), p = 0.0004). Iso-volumetric relaxation time (IVRT) decreased significantly in patients after 1 year of venesection (decrease from 107.4 ± 16.2 to 97.68 ± 15.4 ms, p (0.0187)).

Conclusion

Among all measurements, radial strain, IVRT and left atrial force were shown to significantly improve following a 1-year course of venesection, suggesting that these parameters could be used to identify subclinical cardiac dysfunction in patients with iron overload secondary to hereditary haemochromatosis and to guide intensification of venesection therapy.

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Acknowledgments

We would like to acknowledge and sincerely thank Ms. Liz Ellis who was the clinical nurse specialist for venesection treatment in St James Hospital at the time of our study.

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Authors and Affiliations

  1. Department of Cardiology, St James Hospital, Dublin 8, Ireland

    Danielle Byrne & Caroline Daly

  2. Department of Neuroradiology, Vancouver General Hospital, 899 W 12th Ave., Vancouver, BC, V5Z 1M9, Canada

    Danielle Byrne

  3. Department of Radiology, St James Hospital, Dublin 8, Ireland

    John Patrick Walsh

  4. Department of Gastroenterology, St James Hospital, Dublin 8, Ireland

    Susan McKiernan & Suzanne Norris

  5. Institute of Cardiovascular Science, St James Hospital, Dublin 8, Ireland

    Caroline Daly, Ross T. Murphy & Gerard King

Authors
  1. Danielle Byrne
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  2. John Patrick Walsh
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  3. Caroline Daly
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  4. Susan McKiernan
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  5. Suzanne Norris
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  6. Ross T. Murphy
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  7. Gerard King
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Corresponding author

Correspondence to Danielle Byrne.

Ethics declarations

The Local Research Ethics Committee approved the study. Informed consent was obtained from each patient. Standard views and techniques were used according to the American Society of Echocardiography (ASE)/European Association of Echocardiography (EAE) guidelines.

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The authors declare that they have no conflict of interest.

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Glossary

ASE

American Society of Echocardiography

UIBC

unsaturated iron binding capacity

BSA

body surface area

EAE

European Association of Echocardiography

Fe

ferritin

Hb

haemoglobin

HH

hereditary haemochromatosis

IVRT

iso-volumetric relaxation time

LAF

left atrial force

LAV max

left atrial volume

LV

left ventricular

MRI

magnetic resonance imaging

STE

speckle tracking echocardiography

TEI

myocardial performance index

TIBC

total iron binding capacity

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Byrne, D., Walsh, J.P., Daly, C. et al. Improvements in cardiac function detected using echocardiography in patients with hereditary haemochromatosis. Ir J Med Sci 189, 109–117 (2020). https://doi.org/10.1007/s11845-019-02032-5

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  • DOI: https://doi.org/10.1007/s11845-019-02032-5

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