Congenital heart disease

Prevalence and clinical correlates and characteristics of “Super Fontan”

Am Heart JPublished Retrospective cohort study

Authors' conclusion

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.

Study context

Population
Fontan patients who had undergone cardiopulmonary exercise testing
Participants
404 patients
Exposure
Super-Fontan (SF) vs non-SF
  • SFn = 77
Outcome
Prevalence of SF

Main finding

As reported in the abstract

GroupValuePostoperative 5 yearsPostoperative 10 yearsPostoperative 15 yearsPostoperative 20 yearsPostoperative ≥ 25 years
SF19%16 (35%)30 (39%)18 (19%)13 (14%)0 (0%)

SF

Value19%
Postoperative 5 years16 (35%)
Postoperative 10 years30 (39%)
Postoperative 15 years18 (19%)
Postoperative 20 years13 (14%)
Postoperative ≥ 25 years0 (0%)

Observational data: associations, not effects.

Further findings

OutcomeSF
Death and unexpected hospitalization during follow-up
Hospitalization rate67% lower than non-SF
Death and unexpected hospitalization during follow-up — Deaths overall
25
Death and unexpected hospitalization during follow-up — Unexpected hospitalizations overall
74
Death and unexpected hospitalization during follow-up — SF vs non-SF death and hospitalization
P < .01-.001

Clinical correlates of SF vs non-SF

SF patients younger
P < 0.001
SF patients mostly men
P < 0.05
SF: high arterial BP and saturation (SaO2), low systemic ventricle (SV) end-diastolic pressure, favorable body composition, superior pulmonary function, preserved hepatorenal and hemostatic functions, better glucose tolerance
P < 0.05-.001
Pre-Fontan better SV function, low pulmonary artery resistance and high SaO2 associated with current SF
P < 0.05-.01
Positive trajectory of exercise capacity and high daily activity during childhood associated with current adult SF
P < 0.05

Original publication

Prevalence and clinical correlates and characteristics of “Super Fontan”. Am Heart J. Published 20 May 2023. doi:10.1016/j.ahj.2023.05.010

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