Regression of Advanced Liver Fibrosis After Heart Transplantation in a Patient With Prior Fontan Surgery for Complex Congenital Heart Disease

Bouchardy, Judith; Meyer, Philippe; Yerly, Patrick; Blanche, C; Hullin, Roger; Giostra, Emiliano; Hanquinet, Sylviane; Rubbia‐Brandt, Laura; Sekarski, Nicole; Prêtre, René; Beghetti, Maurice

doi:10.1161/circheartfailure.117.003754

Cited by 22 publications

(9 citation statements)

References 4 publications

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“…In patients who survived cardiac transplantation, improvements in morphological and laboratory FALD parameters were detected. This observation has also been reported by other institutions and might underline the remarkable hepatic potential for regeneration ( 11 , 30 , 31 ). Therefore, patients with mild to moderate impairment of metabolic liver function might be appropriate candidates for isolated cardiac transplantation, whereas a severely impaired maximum liver function capacity may indicate the necessity of a combined heart and liver transplantation.…”

Section: Discussionsupporting

confidence: 88%

Functional hepatic deterioration determined by 13C-methacetin breath test is associated with impaired hemodynamics and late Fontan failure in adults

Schleiger

Krämer

Sallmon

et al. 2022

Front. Cardiovasc. Med.

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BackgroundDespite improved survival a substantial number of Fontan patients eventually develop late failure. Fontan-associated liver disease (FALD) is the most frequent end-organ dysfunction. Although impaired hemodynamics and Fontan failure correlate with FALD severity, no association between hepatic functional metabolic impairment and Fontan hemodynamics has been established.HypothesisMetabolic liver function measured by liver maximum function capacity test (LiMAx®) correlates with Fontan hemodynamics and Fontan failure.MethodsFrom 2020 to 2022, 58 adult Fontan patients [median age: 29.3 years, IQR (12.7), median follow-up time after Fontan operation: 23.2 years, IQR (8.7)] were analyzed in a cross-sectional study. Hemodynamic assessment included echocardiography, cardiopulmonary exercise testing and invasive hemodynamic evaluation. Fontan failure was defined based on commonly applied clinical criteria and our recently composed multimodal Fontan failure score.ResultsLiMAx® test revealed normal maximum liver function capacity in 40 patients (>315 μg/h*kg). In 18 patients a mild to moderate impairment was detected (140–314 μg/h*kg), no patient suffered from severe hepatic deterioration (≤ 139 μg/kg*h). Fontan failure was present in 15 patients. Metabolic liver function was significantly reduced in patients with increased pulmonary artery pressure (p = 0.041. r = −0.269) and ventricular end-diastolic pressure (p = 0.033, r = −0.325), respectively. In addition, maximum liver function capacity was significantly impaired in patients with late Fontan failure (289.0 ± 99.6 μg/kg*h vs. 384.5 ± 128.6 μg/kg*h, p = 0.007).ConclusionMaximum liver function capacity as determined by LiMAx® was significantly reduced in patients with late Fontan failure. In addition, elevated pulmonary artery pressure and end-diastolic ventricular pressure were associated with hepatic functional metabolic impairment.

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Section: Discussionsupporting

confidence: 88%

Functional hepatic deterioration determined by 13C-methacetin breath test is associated with impaired hemodynamics and late Fontan failure in adults

Schleiger

Krämer

Sallmon

et al. 2022

Front. Cardiovasc. Med.

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“…Indeed, although several studies have demonstrated reduced survival with a high post‐transplant complication rate in cirrhotic patients, another study failed to demonstrate a correlation between the degree of fibrosis and 1‐year post‐transplant survival 21 . Interestingly, one study has demonstrated reversibility in liver fibrosis in heart transplanted patients 23 …”

Section: Discussionmentioning

confidence: 99%

Non‐invasive diagnostic imaging tests largely underdiagnose cardiac cirrhosis in patients undergoing advanced therapy evaluation: How can we identify the high‐risk patient?

Belzile

Turgeon

Leblanc

et al. 2021

Clinical Transplantation

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Background Patients with liver cirrhosis are generally considered ineligible for isolated cardiac transplantation or left ventricular assist device (LVAD) implantation. The aim of this retrospective study is to explore the diagnostic value of abdominal ultrasound, computed tomography scan (CT scan) and liver‐spleen scintigraphy to detect the presence of cirrhosis in patients with advanced heart failure. Methods Among 567 consecutive patients who underwent pre‐transplantation or LVAD evaluation, 54 had a liver biopsy to rule out cardiac cirrhosis; we compared the biopsy results with the imaging investigations. Results In about 26% (n = 14) of patients undergoing liver biopsy, histopathological evaluation identified cirrhosis. The respective sensitivity of abdominal ultrasound, CT scan and liver‐spleen scintigraphy to detect cirrhosis was 57% [29–82], 50% [16–84], and 25% [3–65]. The specificity was 80% [64–91], 89% [72–98], and 44% [20–70], respectively. Conclusion Ultrasonography has the best‐combined sensitivity and specificity for the diagnosis of cirrhosis. However, more than a third of patients with cirrhosis will go undiagnosed by conventional imaging. As liver biopsy is associated with a low rate of complication, it should be considered in patients with a high‐risk of cirrhosis or with evidence of portal hypertension to assess their eligibility for heart transplantation or LVAD implantation.

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“…Although limited in nature, some reports suggest that liver disease may stabilize after successful IHT. [ 151 ] A retrospective study of 41 patients with Fontan physiology with liver imaging evaluated for IHT compared liver imaging studies obtained pretransplant with those done 1 year or longer after transplant. [ 152 ] The median age at the time of IHT was 12 years (range 3–23).…”

Section: Transplantation In Patients With Faldmentioning

confidence: 99%

Fontan‐associated liver disease

et al. 2022

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Regression of Advanced Liver Fibrosis After Heart Transplantation in a Patient With Prior Fontan Surgery for Complex Congenital Heart Disease

Cited by 22 publications

References 4 publications

Functional hepatic deterioration determined by 13C-methacetin breath test is associated with impaired hemodynamics and late Fontan failure in adults

Functional hepatic deterioration determined by 13C-methacetin breath test is associated with impaired hemodynamics and late Fontan failure in adults

Non‐invasive diagnostic imaging tests largely underdiagnose cardiac cirrhosis in patients undergoing advanced therapy evaluation: How can we identify the high‐risk patient?

Fontan‐associated liver disease

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