Transient Liver Elastography in the Follow-Up of Fontan Patients—Results of a Nation-Wide Survey in Germany

Background: In recent years, Fontan associated liver disease (FALD) has gained increasing interest and importance in the long-term outcome after Fontan-surgery. Liver biopsy is considered the “gold standard” for diagnosis but may impose an increased risk. To date there are no consensus guidelines for diagnosing and monitoring FALD. Transient elastography (TE) is a rapid, noninvasive method to assess FALD and liver fibrosis. Our study aimed to assess if and how TE is practiced in Germany for the follow-up of Fontan patients.

Methods: Our questionnaire consisted of 10 items to assess the use of TE in Germany. They were categorized in three parts: I. general information on whether the TE is practiced and how it is done. II. indication for TE. III. condition of the patient to practice TE.

Results: In total, 95 valid questionnaires were collected. Only 20% of the centers offer the TE investigation directly. Most transfer the patients to another department or center (40%) or did not offer TE (40%). In only 2.6% of the centers TE is performed directly by the cardiologist. In 29.2% TE is performed only at a certain age, in 27.7% if the patient is symptomatic. In only 13.9% TE is proposed for all the Fontan patients on a routine basis. In 29.1%, TE is performed for other indications such as deterioration. Most often TE is performed only from the beginning of the adolescence (28,1%). For 15.6% before the beginning of the adolescence, for 12.5% in school age, for 3.1% after the age of 16 years and for 6.2% not before an age of 18 years. 34.5% of the hospitals performed TE only for a given indication. In the majority of answers it was not known if the patients are fasting for the examination (68%) and if the TE examination had to be performed in a specific breathing phase (90%). In the majority, TE is not offered routinely (46.9%) .

Conclusion: To date in Germany, TE is only used in a few numbers of centers. A standardized protocol is not existing. TE is however a safe, easy to use tool and well tolerated by the patients. It can be an effective monitoring and screening tool to identify and to stage FALD. We strongly suggest to introduce TE systematically for the evaluation of liver congestion and fibrosis, as a part of the routine follow-up of Fontan patients.

Publication History

Article published online:
13 February 2024