RT Journal Article
SR Electronic
T1 Haemodynamic and prognostic associations of liver fibrosis scores in Fontan-associated liver disease
JF Heart
JO Heart
FD BMJ Publishing Group Ltd and British Cardiovascular Society
SP heartjnl-2022-321435
DO 10.1136/heartjnl-2022-321435
A1 Irene Martin de Miguel
A1 Patrick S Kamath
A1 Alexander C Egbe
A1 C Charles Jain
A1 Frank Cetta
A1 Heidi M Connolly
A1 William R Miranda
YR 2022
UL http://heart.bmj.com/content/early/2022/12/28/heartjnl-2022-321435.abstract
AB Objectives Fontan-associated liver disease (FALD) is universal post-Fontan palliation; however, its impact on survival remains controversial and current diagnostic tools have limitations. We aimed to assess the prognostic role of liver fibrosis scores (aminotransferase to platelet ratio [APRI] and fibrosis-4 [FIB-4]) and their association with haemodynamics and other markers of liver disease.Methods 159 adults (age ≥18 years) post-Fontan undergoing catheterisation at Mayo Clinic, Minnesota, between 1999 and 2017 were included. Invasive haemodynamics and FALD-related laboratory, imaging and pathology data were documented.Results Mean age was 31.5±9.3 years, while median age at Fontan procedure was 7.5 years (4–14). Median APRI score (n=159) was 0.49 (0.33–0.61) and median FIB-4 score (n=94) was 1.12 (0.71–1.65). Correlations between APRI and FIB-4 scores and Fontan pressures (r=0.30, p=0.0002; r=0.34, p=0.0008, respectively) and pulmonary arterial wedge pressure (r=0.25, p=0.002; r=0.30, p=0.005, respectively) were weak. Median average hepatic stiffness by magnetic resonance elastography was 4.9 kPa (4.3–6.0; n=26) and 24 (77.4%) showed stage 3 or 4 liver fibrosis on biopsy; these variables were not associated with APRI/FIB-4 scores. On multivariable analyses, APRI and FIB-4 scores were independently associated with overall mortality (HR 1.31 [1.07–1.55] per unit increase, p=0.003; HR 2.15 [1.31–3.54] per unit increase, p=0.003, respectively).Conclusions APRI and FIB-4 scores were associated with long-term all-cause mortality in Fontan patients independent of other prognostic markers. Correlations between haemodynamic status and liver scores were weak; furthermore, most markers of liver fibrosis failed to correlate with non-invasive indices, underscoring the complexity of FALD.Data are available on reasonable request.