Patients with Fontan circulation: long-term complications and follow-up strategy

The Fontan operation is a palliative surgical correction, which is performed on children with congenital heart defects (CHD) in cases when surgical creation of two equally working ventricles is impossible. Today there are about 70,000 patients with Fontan circulation all over the world. Due to improvement of methods in early detection and treatment of CHD, as well as monitoring algorithms, an increasing number of patients with a single ventricle (SV) stay alive until adulthood. However, this group’s mortality is still quite high, despite the constant development of the surgery approach using modern technical equipment and high-tech examination methods. Chronic increase in systemic venous pressure, non-physiological passive pulmonary blood flow and progressive heart failure (HF) with a decreasing cardiac output (CO) gradually lead to complications from other organs and systems. That is why a multidisciplinary approach and development of optimal management tactics are required to follow-up patients with Fontan hemodynamics. Thus, the purpose of our work was to analyze and summarize current data of the management strategy and timely diagnosis complications in patients with Fontan circulation.