© 2021 Wiley Periodicals LLC

Journal of Cardiac Surgery (2021) 36: 2329-2335

DOI 10.1111/jocs.15553

Received: 11 March 2021 / Accepted: 24 March 2021


Fontanassociated liver disease and total cavopulmonary anatomical flow effectors



William N Evans, MD, Ruben J Acherman, MD, Alvaro Galindo, MD, Abraham Rothman, MD, Michael L Ciccolo, MD, Juan Lehoux, MD, Brody J Winn, MD, Noel S Yumiaco, MD, Humberto Restrepo, MD, MPH



Children’s Heart Center Nevada, 3006 S Maryland Parkway, Ste 690, Las Vegas, NV 89109, USA. Email: WNevans50@aol.com.



Objective: We investigated a relationship between a composite index comprised of

Fontancircuit 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 anato-mical variants in extracardiac Fontan patients are associated with higher Fontanassociated liver disease (FALD)related hepatic total fibrosis scores than others, despite similar Fontan durations.


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