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Introduction: Patients with Fontan palliation are susceptible to congestive hepatopathy and Fontan-associated liver disease (FALD) because of hemodynamic changes. The staging of liver fibrosis involves various methods, including invasive biopsy. Transjugular liver biopsy (TJLB) offers a less invasive alternative, enhancing liver disease surveillance in routine cardiac catheterization. We detail the technical aspects, share initial outcomes, and discuss existing literature. Methods/results: During routine follow-up cardiac catheterization indicated by hemodynamic or clinical alterations, four patients aged between 16 and 26 years with univentricular Fontan circulation and three patients with biventricular circulation underwent TJLB during routine surveillance catheterization. The examinations were performed under conscious sedation and local anesthesia without general anesthesia. Jugular access was obtained at the site of liver localization, and a 5 F multipurpose catheter was inserted into the liver veins. After hand angiography to delineate the local hepatic venous anatomy, an exchange wire was used to place the bioptome, and three consecutive biopsies were performed. There were no complications, especially perforation or bleeding. The technical success rate was 100%, with all obtained samples appropriate for histopathological diagnostics. The total additional procedure time was less than 20â min. Conclusion: TJLB is an attractive alternative method for obtaining liver specimens in the scope of FALD care. We believe that it should be performed during routine hemodynamic evaluations in Fontan patients and can be performed safely with very low additional time expenditure. As the biopsy site is intravascular, the risk of external bleeding or hematoma is significantly reduced despite the high intrahepatic pressures and the usually impaired coagulation profile in these patients. Based on our initial experience and the lower complication rates compared with other techniques, TJLB should be considered a standard approach in these patients and used more often during the long-term follow-up of Fontan patients. It can be performed in the same setting whenever a hemodynamic assessment of patients with congenital heart defects is required.
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Objectives: For several years, patients with single ventricle hearts have been palliated according to the Fontan principle. One well known long-term consequence in these patients is the Fontan-associated liver disease and fibrosis, which occurs due to the chronically increased Central Venous Pressure (CVP) after Fontan palliation. It carries an increased risk of liver cirrhosis and hepatocellular carcinoma over time. Liver elastography (LE) is a non-invasive, safe, and feasible ultrasound method to determine liver stiffness and the stage of liver fibrosis. Usually, this examination must be performed in a sober condition and strict inspiratory hold to optimize the results and may therefore be difficult to perform on children as a routine examination. However, the influence of food intake and respiration on these results in Fontan patients is unclear. To optimize the implementation for this examination especially in children, the effects of food intake and breathing maneuvers on liver stiffness in patients with Fontan circulation were investigated. Methods: For this prospective study, 25 Fontan patients (group 1) and 50 healthy volunteers (group 2) were examined. The two groups were additionally divided into two age categories (group 1a: 10-19 years; group 1b: 20-29 years; group 2a: 15-19 years; group 2b: 20-25 years). Liver stiffness was measured by liver elastography once before food intake (=T0, with 6 h of fasting). Subsequently the participants consumed a standardized chocolate drink (500 mL) with nutritional distribution corresponding to a standardized meal (600 kcal). Liver stiffness was then determined 15, 30, 45, 60, 90, 120, 150, and 180 min after ingestion. Each measurement of liver stiffness was performed during maximal inspiratory and expiratory holds. The study was reviewed and approved by the responsible ethics committee. Results: In group 2 there was a significant increase in liver stiffness after food intake at T15, T30, and T45 during inspiration measurements (T0 = 4.0 kPa vs. T15 = 4.9 kPa, difference = 22.5%; T0 = 4.0 kPa vs. T30 = 4.9 kPa difference = 22.5%; T0 = 4.0 kPa vs. T45 = 4.3 kPa difference = 7.5%), as well as during expiration at T15 and T30 (T0 = 4.5 kPa vs. T15 = 5.1 kPa, difference = 14.7%; T0 = 4.5 kPa vs. T30 = 4.9 kPa difference = 7.8%). Whereas in Fontan patients (group 1) liver stiffness did not differ significantly at any time between fasting (T0) and postprandial values. The respiratory maneuvers in the healthy subjects (group 2) differed significantly only before food intake (T0) (group 2: insp = 3.97 kPa vs. exp. = 4.48 kPa difference = 11.3%). In the Fontan group (group 1), there was no significant difference between the respiratory phases at any point. The different age categories showed no significant difference in liver stiffness. Conclusion: With these results we could demonstrate for the first time that in Fontan patients the time of food intake (i.e., fasting) has no clinical significance for the values obtained in liver elastography. We could also demonstrate that the breathing maneuvers during the examination had only minimal clinical impact on the results of liver elastography in patients with normal circulation and no effect in patients with Fontan-circulation. Consequently, liver elastography for Fontan patients is reliable independently of food intake and breathing maneuvers and can also be performed on younger patients, who are unable to follow breathing commands or longer fasting periods, without any impairment of the results. These results should encourage a routine use of LE in the follow-up of Fontan patients.
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Introduction: Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). Currently, there is no consensus on how to stage FALD. Transient elastography (TE) is a rapid, non-invasive method to assess FALD and liver fibrosis. Method: To assess the availability and conditions of using TE to monitor liver disease in Fontan patients in german centers for pediatric cardiology and to propose the introduction of a standardized national protocol for the monitoring of liver disease, we developed a questionnaire. Results: In total, 95 valid questionnaires were collected. Only 20% of the centers offer the TE investigation directly. Most of the centers transfer the patients to another department or center (40%) or didnt offer TE (40%). In only 2.6% of the centers TE is performed directly by the cardiologist. Most of the centers transfer the patients to a other department. In 29.2% TE is performed only at a certain age of the patients and in 27.7% it is performed if the patients present symptoms of failing Fontan. In only 13.9% of the centers TE is proposed in all the Fontan patients on a routine basis. Most often TE is performed only from the beginning of the adolescence. In the majority of answers it was not known if the patients are fasting for the examination (68%) or not and if the TE examination had to be performed in a specific breathing phase during TE (Inspiration/Expiration, 90%). In the majority, TE is not offered routinely (46.9%). Discussion: To date in Germany, TE is only used in a few numbers of centers specialized in Fontan follow-up. A standardized protocol to use TE is currently not existing. With regard to the feasibility of the examination, it is evident that TE is a quick, cheap and easy method to distinguish between cases with and without progressive FALD. This makes TE a useful and prognostic tool for screening of liver disease and to failing Fontan circulation. Conclusion: We propose a systematic TE evaluation of possible liver congestion and fibrosis, as a part of the routine follow-up of Fontan patients.
