Liver abnormalities and post‐transplant survival in pediatric Fontan patients. (3rd November 2017)

• Record Type: Journal Article
• Authors: Hofferberth, Sophie C.; Singh, Tajinder P.; Bastardi, Heather; Blume, Elizabeth D.; Fynn‐Thompson, Francis
• Is Part Of: Pediatric transplantation. Volume 21:Number 8(2017)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2017-11-03
• Subjects: liver -- liver biopsy -- pediatric heart transplant -- pediatrics -- survival; Transplantation of organs, tissues, etc. in children -- Periodicals; 617.95408305
• DOI: 10.1111/petr.13061
• Languages: English
• ISSNs: 1397-3142

Abstract

Abstract: The impact of liver parenchymal abnormalities on survival post‐heart transplant remains unknown in pediatric Fontan patients. We assessed pediatric Fontan patients who underwent heart transplant and had documented pretransplant hepatic ultrasound (U/S) studies. Liver U/S findings were classified as normal (Group 1), mildly abnormal (Group 2, hepatomegaly/vascular congestion), or severely abnormal (Group 3, heterogeneous echotexture/nodularity). Among 30 study patients, 8 were classified as Group 1, 14 as Group 2, while 8 met Group 3 criteria. Pretransplant liver biochemistry and synthetic function were similar in all groups. Six Group 3 patients underwent liver biopsy; 4 demonstrated perisinusoidal or centrilobular fibrosis, and 2 had cirrhosis. Overall mortality was 30% (n = 9). Median follow‐up was 5 years (range, 0.25‐13 years). One‐year survival was similar among all 3 groups ( P = .37), with a trend toward higher cumulative 5‐year survival in Group 1 (100%). The majority of pediatric Fontan patients who underwent heart transplant demonstrated abnormal preoperative liver ultrasound findings. Heterogeneous echotexture or nodularity detected on U/S frequently indicates underlying liver parenchymal abnormalities. The presence of severe liver abnormalities was not associated with higher early mortality post‐heart transplant in pediatric Fontan patients; however, late outcomes must be further elucidated.