Extracardiac manifestations of the Fontan circulation in adults: Beyond the liver

Abstract

Patients with Fontan circulation are now surviving longer into adulthood and experience a multitude of extracardiac, non-hepatic sequelae that impact their quality of life and prognosis. These sequelae include pulmonary complications (pulmonary vascular disease, sleep-disordered breathing, restrictive lung disease, plastic bronchitis), gastrointestinal manifestations (protein losing enteropathy), chronic venous insufficiency, kidney abnormalities, reproductive issues (pregnancy, contraception and sexual dysfunction), neurocognitive changes, endocrinopathies (glycemic abnormalities, thyroid disorders) and hematological abnormalities (thromboembolic complications and secondary erythrocytosis). This review article aims to present a contemporary overview of the extracardiac, non-hepatic sequelae observed in adults with Fontan circulation.

Keywords

• Fontan
• Single ventricle
• Pulmonary vascular disease
• Kidney dysfunction
• Thromboembolic events