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

BACKGROUND AND

OBJECTIVES

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.