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Ultrasound diagnosis of Fontan-associated diseases of parenchymatous organs

Authors: Fedoseeva M.M., Baryshnikova I.Yu., Golukhova E.Z.

Company: 1 Bakoulev National Medical Research Center for Cardiovascular Surgery, Moscow, Russian Federation
2 Moscow Clinical Municipal Children Hospital St. Vladimir, Moscow, Russian Federation

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Type:  Original articles


DOI: https://doi.org/10.24022/1997-3187-2025-19-1-60-69

For citation: Fedoseeva M.M., Baryshnikova I.Yu., Golukhova E.Z. Ultrasound diagnosis of Fontan-associated diseases of parenchymatous organs. Creative Cardiology. 2025; 19 (1): 60–69 (in Russ.). DOI: 10.24022/1997-3187-2025-19-1-60-69

Received / Accepted:  09.01.2025 / 22.01.2025

Keywords: single ventricle hemodynamics Fontan surgery ultrasound Fontaine-associated diseases Fontaine-associated liver disease hepatocellular carcinoma Fontaine-associated nephropathy protein loss enteropathy plastic bronchitis



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Abstract

The development of cardiac surgical care for patients with single ventricle heart (SVH), wide use of hemodynamic correction according to the Fontaine method, allowed to significantly increase their life expectancy. Fontan-associated diseases are a new type of disease arising in patients with SVH. These morbidities develop due to altered cardiac anatomy, pathologic central hemodynamics, Fontaine's palliative circulatory model, and multiple surgeries. They affect all extracardiac organs, causing Fontaine-associated diseases of the liver, kidneys, lungs (plastic bronchitis), intestines (enteropathy with protein loss), musculoskeletal and reproductive systems, cognitive and behavioral deficits. Diagnosis of these diseases requires accessible and safe imaging methods, which can be ultrasound as a tool of the “first line”. Most often the object of ultrasound is the liver, which Fontaine-associated diseases include congestive hepatopathy, fibrosis, cirrhosis. Particular vigilance cause focal liver formations, the most formidable of which is hepatocellular carcinoma. Fontan-associated diseases of the kidneys, lungs, and intestines are practically inaccessible to ultrasound, and in some cases we can visualize only their nonspecific manifestations. Since abdominal anomalies are critical for the management of patients with univentricular hemodynamics, the aim of this review was to examine the capabilities of ultrasound diagnostics in imaging the abnormalities in patients after the Fontan procedure. The materials were searched in PubMed database using the keywords “Fontan”, “ultrasound”, “hepatocellular carcinoma”, “nephropathy”, “liver”, “lungs”, “intestine”, “the lymphatic system”.

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About Authors

  • Maria M. Fedoseeva, Radiologist; ORCID
  • Irina Yu. Baryshnikova, Cand. Med. Sci., Head of the Group of Innovation decisions in pediatric ultrasound; ORCID
  • Elena Z Golukhova, Academician of Russian Medical Sciences, Director; ORCID

Chief Editor

Elena Z. Golukhova, MD, PhD, DSc, Professor, Academician of Russian Academy of Sciences, Director of Bakoulev National Medical Research Center for Cardiovascular Surgery


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