Fontan circulation versus cirrhotic cardiomyopathy: A comparative overview

Fontan circulation and cirrhotic cardiomyopathy (CCM) represent distinct but overlapping entities characterized by concomitant hepatic and cardiac dysfunction. Fontan patients exhibit chronically elevated central venous pressure (CVP), impaired preload, limited cardiac reserve, systolic-diastolic dysfunction, and frequent arrhythmias, while CCM arises in cirrhosis with blunted stress response, diastolic dysfunction, and electrophysiologic abnormalities despite preserved resting ejection fraction. Both share exercise intolerance and stress-induced contractile failure but differ in underlying hemodynamics: Low-output, high-CVP Fontan physiology versus hyperdynamic, low-resistance CCM. We highlight mechanistic links, including inflammation, nitric oxide, and bile acid-mediated cardiotoxicity, by which Fontan-associated cirrhosis may induce a CCM-like phenotype, with implications for surveillance and management.