Document Type

Article

Publication Date

4-29-2026

Abstract

Structured Summary

a. Background/Introduction:

For patients with single ventricle congenital heart disease, the Fontan procedure is the definitive palliative procedure. Although advancements have been made in the surgical technique and management, the Fontan circulation is inefficient and prone to progressive dysfunction. Elevated central venous pressure, non-pulsatile pulmonary blood flow, and reduced cardiac output can lead to many complications, and eventual failure. These conditions can lead to protein losing enteropathy, plastic bronchitis, and hepatic fibrosis which often indicate advanced decline. Though a known problem, there is a lack of standardized criteria to guide optimal intervention timing of Fontan revision prior to irreversible failure. This scoping review identifies current evidence regarding early physiologic markers of Fontan circulatory failure, and how it can inform preemptive interventional strategies.

b. Methods:

This review was conducted according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews (PRISMA-ScR) guidelines. Electronic databases were searched for studies on Fontan physiology, hemodynamic markers, ventricular function, other organ complications, and outcomes related to Fontan failure in pediatric patients (2-18 years). Case studies, cohort studies, reviews, and informational guidelines were included. Data was evaluated to identify patterns across studies and recurrent predictors of Fontan deterioration.

c. Results:

The literature consistently showed associations between progressive dysfunction and rising central venous pressure, increased pulmonary vascular resistance, declining ventricular compliance, reduced cardiac output, and a lower exercise capacity. Prolonged venous hypertension was associated with secondary complications involving other organ systems, such as protein losing enteropathy, plastic bronchitis, hepatic and renal complications, as well as lymphatic congestion. Across the evidence, considerable diversity exists in definitions of “Fontan failure” and the reported hemodynamic and clinical thresholds. Overall, the evidence suggests a measurable, gradual, progressive decline, rather than an abrupt failure of the system.

d. Conclusions:

Early identification of Fontan dysfunction may allow for intervention prior to irreversible end organ damage. However, the variability in diagnostic practices and protocols across congenital heart centers, as well as the absence of standardized predictive models limits the ability to make a preemptive diagnosis. Prospective multicenter studies and development of predictive algorithms are needed to refine the timing of Fontan revision and improve clinical long term outcomes.

e. Keywords:

Fontan circulation; Fontan failure; single ventricle; congenital heart disease; hemodynamic parameters; clinical outcomes; central venous pressure; pulmonary vascular resistance; ventricular function; pediatric cardiac surgery.

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