Platelet slope and long-term clinical outcomes in children and adults with Fontan-associated liver disease.

Objective: There is a lack of robust literature describing a relationship between platelet count as a reflection of liver fibrosis and Fontan-associated liver disease (FALD). The present study investigated the serial longitudinal relationship of laboratory tests to cirrhosis and clinical outcomes in patients following Fontan procedure.

Methods: This was a retrospective study of patients with Fontan procedure who underwent laboratory evaluation at least 1 year after surgery. Clinical data, including death, failing Fontan physiology, and heart transplantation, were investigated. Cirrhosis was defined as stage 4 fibrosis on liver biopsy and/or evidence of cirrhosis from imaging. Portal hypertension (PHTN) was calculated using the VAST score (one point each for Varices, Ascites, Splenomegaly, and Thrombocytopenia); VAST score ≥ 2 indicating PHTN features.

Results: Among 376 patients (184 children and 192 adults), cirrhosis was recorded in 52/376 (13.8%). Platelet counts in those with FALD-associated cirrhosis decreased significantly starting 25, 30 and 35 years after Fontan, compared to the non-cirrhosis group (151 vs. 188; p = 0.01, 134 vs. 174; p = 0.02, and 127 vs. 202 × 103/uL; p = 0.04, respectively). Patients with cirrhosis and PHTN features had significantly worse heart transplant-free survival, overall survival, and failing Fontan physiology compared to patients without cirrhosis.

Conclusions: FALD patients with cirrhosis develop decreasing platelet counts 25 years after Fontan procedure. Lower platelets, even if near normal range, can be a marker of cirrhosis in FALD. Cirrhosis with PHTN is an associated with worse heart transplant-free survival, overall survival, and failing Fontan.