State of the art of the Fontan strategy for treatment of univentricular heart disease

Abstract

In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed. Common surgical and catheter-based interventions following Fontan completion are outlined. We describe functional status measurements such as quality of life and developmental outcomes in the contemporary Fontan patient. The current role of drug therapy in the Fontan patient is explored. Furthermore, we assess the current use and outcomes of mechanical circulatory support in the Fontan circulation and novel surgical innovations. Despite large improvements in outcomes for contemporary Fontan patients, a large burden of disease exists in this patient population. Continued efforts to improve outcomes are warranted. Several remaining challenges in the Fontan field are outlined.

Keywords: Congenital heart defects; Fontan Procedure; Morbidity; Mortality; Pediatrics; Re-interventions; Single ventricle; Total cavopulmonary connection.

Figure 1.

Illustration of the anatomic relationships following partial cavopulmonary connection ( A) and total cavopulmonary connection ( B) palliation. IVC, inferior vena cava; PA, pulmonary artery; SV, single ventricle; SVC, superior vena cava. This figure has been reproduced with permission from Kerlo et al. and Springer Nature .

Figure 2.

Scheme of pressures in the normal circulation ( A) and the Fontan circulation ( B). This scheme illustrates the effects of the lack of a prepulmonary pump in the Fontan physiology. Red represents oxygenated blood and blue represents deoxygenated blood. Ao, aorta; CV, caval veins; LA, left atrium; LV, left ventricle; P, pulmonary circulation; PA, pulmonary artery; RA, right atrium; RV, right ventricle; S, systemic circulation; V, single ventricle. This figure has been reproduced with permission from Gewillig and Brown and the British Medical Journal Publishing Group Ltd .

Figure 3.. Survival following Fontan completion.

Each line represents a study assessing survival at multiple time points and is colored by surgical era. Dots represent Kaplan-Meier survival estimates. Studies marked with an asterisk show survival curves for death, transplant, or Fontan revision; others show survival curves for only death. This figure has been reproduced with permission from Kverneland et al. and John Wiley and Sons .