Prevalence and clinical correlates and characteristics of "Super Fontan"

Abstract

Background: Super-Fontan (SF) is an excellent phenotype of patients with Fontan circulation and normal exercise capacity. This study aimed to clarify the prevalence and clinical correlates and characteristics of SF.

Methods: We reviewed 404 Fontan patients who had undergone cardiopulmonary exercise testing, and the results were compared with clinical profiles.

Results: Seventy-seven (19%) patients had SF, and the postoperative prevalence at 5, 10, 15, 20, and ≥ 25 years was 16 (35%), 30 (39%), 18 (19%), 13 (14%), and 0 (0%), respectively. Compared with non-SF, SF patients were younger (P < .001) and were mostly men (P < .05). SF was characterized by a current high arterial blood pressure and oxygen saturation (SaO₂), low systemic ventricle (SV) end-diastolic pressure, favorable body composition, superior pulmonary function, preserved hepatorenal and hemostatic functions, and better glucose tolerance (P < .05-.001). Pre-Fontan better SV function, low pulmonary artery resistance, and high SaO₂ were associated with current SF (P < .05-.01). Furthermore, positive trajectory of exercise capacity and high daily activity during childhood were associated with current adult SF (P < .05). During the follow-up, 25 patients died, and 74 patients were unexpectedly hospitalized. There was no death in the SF group, and the rate of hospitalization was 67% lower than that of the non-SF group (P < .01-.001).

Conclusions: The prevalence of SF gradually decreased over time. SF was characterized by preserved multi-end-organ function and an excellent prognosis. Pre-Fontan hemodynamics and post-Fontan childhood daily activity were associated with being adult SF.