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Fontan‐associated liver disease and total cavopulmonary anatomical flow effectors
Author(s) -
Evans William N.,
Acherman Ruben J.,
Galindo Alvaro,
Rothman Abraham,
Ciccolo Michael L.,
Lehoux Juan,
Winn Brody J.,
Yumiaco Noel S.,
Restrepo Humberto
Publication year - 2021
Publication title -
journal of cardiac surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.428
H-Index - 58
eISSN - 1540-8191
pISSN - 0886-0440
DOI - 10.1111/jocs.15553
Subject(s) - medicine , fontan procedure , liver biopsy , fibrosis , cardiac catheterization , cohort , biopsy , cardiology , hemodynamics , hepatic fibrosis , liver disease , heart disease , gastroenterology , surgery
Objective We investigated a relationship between a composite index comprised of Fontan‐circuit anatomical features and hepatic fibrosis scores from biopsy. Methods We identified living extracardiac Fontan patients, ≥7 years old and ≥5 but <20 years postoperative, that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2020. We divided patients into anatomical groups and applied a risk score to each patient. We compared average anatomical risk scores with average hepatic total fibrosis scores by group. Results We identified 111 patients that met inclusion criteria. After excluding four patients, we assigned 107 to one of 12 anatomical variant groups ( n ≥ 3). For the 107, the average age at liver biopsy was 14 ± 6 years old. Of the 107, 105 (98%) were New York Heart Association Class 1. We found average anatomical risk scores by group correlated with average total fibrosis scores by group ( R = 0.8; p = .005). An average Fontan duration to biopsy of 10 ± 1 years was similar for all 12 anatomical groups. We found no other clinical variables, laboratory, or hemodynamic values that trended with anatomical risk scores or hepatic total fibrosis scores. Conclusions In a cohort of relatively young, stable extracardiac Fontan patients, average composite anatomical risk scores strongly correlated with average hepatic total fibrosis scores by anatomical group. These findings suggest that some anatomical variants in extracardiac Fontan patients are associated with higher Fontan‐associated liver disease (FALD)‐related hepatic total fibrosis scores than others, despite similar Fontan durations.