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Long-Standing Cyanosis in Congenital Heart Disease Does not Cause Diffuse Myocardial Fibrosis

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Abstract

The assumption of the presence of diffuse myocardial fibrosis in long-standing cyanotic congenital heart disease (CHD) inspired us to noninvasively determine the myocardial extracellular volume (ECV) using contrast CMR. T1 maps were measured pre and 10 min after the injection of 0.15 mmol/kg of gadolinium in 25 subjects. Seven patients with long-standing cyanotic CHD and no previous cardiac surgery (aged 16–53 years and oxygen saturations of 69–90%), nine normal subjects (aged 14–49 years), and nine patients with previously cyanotic CHD, who had been corrected by open heart surgery (aged 2 months–58 years, mean 9 years). Late gadolinium enhancement was performed to exclude scar areas. The T1 values were measured in the interventricular septum and in the left lateral or inferior ventricular wall, such that same areas were assessed in every patient in the pre- and post-contrast T1 scan. ECV was calculated according to ΔR1myocardium/ΔR1blood * (1 − hematocrit). Cyanotic patients had significantly lower ECV percentage than the previous cyanotic patients (septum: 22 ± 2.7% vs 35 ± 4.6%, p = 0.002; LV wall: 22 ± 2.2% vs 30 ± 3.7%, p = 0.01, respectively). No significant differences were found between cyanotic patients and normal controls (septum: 22 ± 2.7% vs 24 ± 1.4%, p = 0.44; LV wall: 22 ± 2.2% vs 24 ± 2%, p = 0.57, respectively). Long-standing cyanosis in CHD without cardiac surgery does not cause diffuse myocardial fibrosis or expansion of the myocardial ECV.

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Acknowledgements

We are very grateful to Sohrab Fratz, MD, PhD, who left us too early, for his scientific enthusiasm and knowledge. He was deeply involved in the scientific discussion and design of this manuscript.

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Authors and Affiliations

  1. Department of Radiology, Cairo University Hospitals, Cairo, Egypt

    Ahmed Kharabish

  2. Department of Congenital Heart Disease and Pediatric Cardiology, Deutsches Herzzentrum München, Technische Universität München, Munich, Germany

    Christian Meierhofer, Peter Ewert & Heiko Stern

  3. Department of Radiology, Deutsches Herzzentrum München, Technische Universität München, Munich, Germany

    Martin Hadamitzky & Stefan Martinoff

  4. Department of Diagnostic and Interventional Radiology, Klinikum rechts der Isar, Technische Universität München, Munich, Germany

    Jonathan Nadjiri

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  1. Ahmed Kharabish
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Correspondence to Ahmed Kharabish.

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Ethical Approval

The study complies with the Declaration of Helsinki, and the protocol complies with the institution’s guidelines.

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A written informed consent was obtained from all patients or the accompanying parents.

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Kharabish, A., Meierhofer, C., Hadamitzky, M. et al. Long-Standing Cyanosis in Congenital Heart Disease Does not Cause Diffuse Myocardial Fibrosis. Pediatr Cardiol 39, 105–110 (2018). https://doi.org/10.1007/s00246-017-1734-2

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  • DOI: https://doi.org/10.1007/s00246-017-1734-2

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