Cardiology Letters 2023, 32(2):115-128

Complications in patients with Fontan circulation

Záhorec M

Oddelenie detskej kardiológie, Detské kardiocentrum, Národný ústav srdcových a cievnych chorôb, a. s., Bratislava, Slovenská republika

It has been 50 years since the Fontan operation was introduced and it is still used in clinical praxis as the final interventional solution for complex congenital heart disease with single ventricle physiology. As a results of its modifications, improvements in patient selection and perioperative care, survival has steadily increased. A growing number of longterm survivors is discovering the challenges of this non-physiologic circuit. The absence of a subpulmonary ventricle is characterized by chronically elevated systemic venous pressures and decreased cardiac output. Systolic and more importantly diastolic dysfunction, arrhythmia, thromboembolism and different forms of lymphatic failure such as plastic bronchitis or protein-losing enteropathy are the most frequent complications of the Fontan circulation. Liver fibrosis, gradual decline of renal function and abnormalities in growth and bone structure are also consequences of this abnormal hemodynamic state. Oral medical therapies of the Fontan circulatory failure have little data demonstrating benefit. It is important to look proactively for optimalization of the Fontan circuit with catheter-based or surgical interventions. The only widely prescribed medication for this population is antithrombotic prophylaxis. Fontan circulatory failure is a new clinical phenotype for most adult cardiologists, and therefore the principles of acute and long-term management of the most common complications are highlighted. The survival and quality of life of this growing population can be even more favourable in the near future by implementing innovative approaches such as lymphatic interventions, mechanical circulatory support or medical treatment of diastolic dysfunction.

Keywords: single ventricle; Fontan operation; Fontan failure; heart failure; lymphatic failure

Published: February 1, 2023  Show citation