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Relation of Magnetic Resonance Elastography to Fontan Circulatory Failure in a Cohort of Pediatric and Adult Patients

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Abstract

Elevated magnetic resonance elastography (MRE)-derived liver stiffness may be associated with worse outcomes in people with Fontan circulation. We sought to evaluate the association between liver stiffness and Fontan failure or portal hypertension. Single center cross-sectional retrospective study of people with Fontan circulation who underwent MRE between 2011 and 2020. The cohort was divided into adult (age ≥ 21 years) and pediatric (< 21 years) groups. Fontan circulatory failure (FF) was defined as any of the following: death, transplantation, ventricular assist device, heart failure symptoms requiring escalation of diuretics. Radiologic portal hypertension was defined as the presence of one or more of the following: splenomegaly, ascites, or gastrointestinal varices. 128 patients were included (average age = 22.6 ± 8.7 years) and 58 (45%) were children. Median liver stiffness was 4.3 kPa (interquartile range (IQR) 3.8–5.8) for the entire cohort. Thirty patients (23%) developed FF (16 adults, 14 children). Liver stiffness was higher in adults with FF compared to those without FF (4.9 (IQR 4.0–6.0) vs. 4.2 (IQR 3.8–4.7) kPa, p = 0.04). There was no difference in liver stiffness between pediatric patients with and without FF (4.4 (IQR 4.1–5.4) vs. 4.4 (IQR 3.8–5.0), p = 0.5). Adults with radiologic portal hypertension and adults with moderate or severe atrioventricular valve regurgitation had higher liver stiffness than adults without. MRE-derived liver stiffness is associated with atrioventricular valve regurgitation, portal hypertension, and poor clinical outcomes in adults with Fontan circulation. There was no association between liver stiffness and FF in pediatric patients. This difference may be due to the progressive nature of Fontan-associated liver disease.

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Funding

Funds for this work were provided by Academic and research committee of Cincinnati Children’s Hospital grant support for the Fontan multidisciplinary clinic. This work was conducted with support from the Heart Institute Research Core (HIRC) at Cincinnati Children’s Hospital Medical Center (ARO).

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Authors and Affiliations

  1. Pediatric Residency Training Program, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH, USA

    Samuel W. Brayer & Faizeen Zafar

  2. Department of Pediatrics, Cincinnati Children’s Hospital Heart Institute, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Adam M. Lubert & Alexander R. Opotowsky

  3. Department of Radiology, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Andrew T. Trout & Jonathan R. Dillman

  4. Division of Gastroenterology, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Joseph J. Palermo

  5. Division of Gastroenterology, Department of Medicine, University of Cincinnati Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA

    Nadeem Anwar

  6. UPMC Children’s Hospital of Pittsburgh Heart and Vascular Institute, Pittsburgh, PA, USA

    Tarek Alsaied

Authors
  1. Samuel W. Brayer
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  2. Faizeen Zafar
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  3. Adam M. Lubert
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  4. Andrew T. Trout
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  7. Nadeem Anwar
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  8. Jonathan R. Dillman
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  9. Tarek Alsaied
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by Samuel Brayer and Tarek Alsaied. The first draft of the manuscript was written by Samuel Brayer, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Samuel W. Brayer.

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This cross-sectional retrospective study was approved by our institutional review board and the need for informed consent was waived due to the retrospective nature of this study.

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Brayer, S.W., Zafar, F., Lubert, A.M. et al. Relation of Magnetic Resonance Elastography to Fontan Circulatory Failure in a Cohort of Pediatric and Adult Patients. Pediatr Cardiol 42, 1871–1878 (2021). https://doi.org/10.1007/s00246-021-02707-w

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  • DOI: https://doi.org/10.1007/s00246-021-02707-w

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