North American Society For Pediatric Gastroenterology, Hepatology & Nutrition

North American Society For Pediatric Gastroenterology, Hepatology & Nutrition

October 19, 2019
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Clinical scores predict risks for liver disease after isolated heart transplant

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CHICAGO — Researchers determined clinical scores that assessed short-term risks for liver disease associated with isolated heart transplantation with the Fontan procedure in pediatric patients, according to a study presented at NASPGHAN 2019.

“Long term survivors of the Fontan operation universally develop hepatic congestion, hepatic fibrosis and portal hypertension of varying degrees,” Dellys Soler-Rodriguez, MD, from the Emory University School of Medicine, said during his presentation, “Assessment of the severity of this Fontan associated liver disease (FALD) is critically important prior to consideration of isolated heart transplantation (IHT).”

He noted that up to 30% of long-term survivors develop arterially enhancing nodules on magnetic resonance imaging or computed tomography that can evolve to hepatocellular carcinoma.

Soler-Rodriguez and colleagues retrospectively evaluated nine Fontan patients aged 10 to 19 years who underwent IHT between October 2015 and October 2018 including standard blood tests, abdominal MRI and liver biopsy obtained 6 months before IHT and 12 months post-IHT heart catheterization.

Mean pre-IHT MELD was 9 with a maximum of 15, and the maximum MELD excluding international normalized ratio (MELD-XI) was 16. There were no differences in pre- or post-IHT white blood count, platelet count, albumin, creatinine, INR, aspartate aminotransferase to platelet ratio index, or MELD, whereas estimated glomerular renal function improved (P = .01).

After IHT, patients showed a decrease in central venous pressures (18.8 mmHg vs. 8.3 mmHg; P < .0001) and improvements in MELD-XI (9.9 vs. 7.7; P = .01) and vulnerability assessment scoring tool or VAST scores (1.44 vs. 0.33; P = .0005).

Soler-Rodriguez concluded that patients with FALD and MELD scores less than 16, Fontan Liver MRI scores less than 10 and VAST scores of 2 or less had successful short-term IHT outcomes and these scores improved at 1-year post-IHT. Additionally, blinded liver biopsy did not change at 1-year post-IHT.

“Given the persistence post-IHT of arterialized nodules, hepatic malignancy risk may persist post-IHT in FALD,” he said. “Clinical scores may be more useful than LB to assess short-term liver associated risk of IHT and for assessment of post IHT FALD clinical evolution.” – by Talitha Bennett

Reference:

Soler-Rodriguez D, et al. Abstract 223. Presented at: NASPGHAN; October 18-19, 2019; Chicago.

Disclosures: Soler-Rodriguez reports no relevant financial disclosures.