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Published on: November 7, 2020
Liver abnormalities and post-transplant survival in pediatric Fontan patients
Sophie C Hofferberth1, Tajinder P Singh2, Heather Bastardi2
1Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Insights
Pediatric Fontan patients undergoing heart transplant often have abnormal liver ultrasound findings. Severe liver abnormalities did not increase early mortality, but long-term outcomes require further study.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Transplant Surgery
Background:
- Liver parenchymal abnormalities' impact on survival post-heart transplant in pediatric Fontan patients is not well understood.
- Pretransplant hepatic ultrasound (U/S) is a key diagnostic tool.
Purpose of the Study:
- To assess the impact of pretransplant liver parenchymal abnormalities, identified via ultrasound, on survival outcomes in pediatric Fontan patients following heart transplant.
Main Methods:
- Retrospective analysis of 30 pediatric Fontan patients who underwent heart transplant.
- Classification of pretransplant liver ultrasound findings into normal, mildly abnormal, or severely abnormal groups.
- Correlation of ultrasound findings with liver biochemistry, synthetic function, biopsy results, and survival data.
Main Results:
- The majority of patients (22/30) showed abnormal pretransplant liver ultrasound findings.
- Severe abnormalities (heterogeneous echotexture/nodularity) were present in 8 patients, with biopsy confirming fibrosis or cirrhosis in 6.
- One-year survival was similar across all groups (P=.37), with a trend towards better 5-year survival in the normal ultrasound group.
Conclusions:
- Pretransplant liver ultrasound frequently detects parenchymal abnormalities in pediatric Fontan patients awaiting heart transplant.
- Severe liver abnormalities identified on ultrasound do not appear to increase early post-transplant mortality.
- Further investigation is needed to understand the long-term survival implications of these findings.
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.
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