Original Investigation
Atrioventricular Valve Failure in Fontan Palliation

https://doi.org/10.1016/j.jacc.2018.12.025Get rights and content
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Abstract

Background

Atrioventricular valve failure (moderate or greater regurgitation, or valve operation) is a risk factor for adverse outcomes in patients undergoing Fontan palliation.

Objectives

This study determined the incidence of atrioventricular valve failure and its clinical impact on patients undergoing Fontan palliation.

Methods

A retrospective cohort longitudinal study was conducted using patient data extracted from an existing bi-national, population-based registry.

Results

A total of 1,468 patients who underwent Fontan palliation were identified; complete follow-up data were available for 1,199 patients. Six hundred eighty-six patients had 2 atrioventricular valves, 286 had a single mitral valve, 130 had a common atrioventricular valve, and 97 had a single tricuspid valve. A total of 132 repairs were performed in 110 patients, and 15 replacements were performed in 13 patients. The cumulative incidence of atrioventricular valve failure at 25 years of age for patients with a common atrioventricular, single tricuspid, single mitral, and 2 atrioventricular valves was 56% (95% confidence interval [CI]: 46% to 67%), 46% (95% CI: 31% to 61%), 8% (95% CI: 4% to 12%), and 26% (95% CI: 21% to 30%), respectively. In patients without valve failure, freedom from Fontan failure at 10 and 20 years post-Fontan palliation was 91% (95% CI: 89% to 93%) and 77% (95% CI: 73% to 81%), respectively, compared with 77% (95% CI: 69% to 85%) and 54% (95% CI: 42% to 68%), respectively, in patients with valve failure (hazard ratio: 2.43; 95% CI: 1.74 to 3.39; p < 0.001).

Conclusions

Atrioventricular valve failure occurs frequently in patients undergoing Fontan palliation. Patients with valve failure are twice as likely to have their Fontan circulation fail than those without valve failure.

Key Words

Fontan
regurgitation
repair
replacement
single ventricle

Abbreviations and Acronyms

CI
confidence interval
HR
hazard ratio
IQR
interquartile range
NYHA
New York Heart Association

Cited by (0)

This work was supported by a National Health and Medical Research Council (NHMRC) Partnership grant (1076849). Dr. King is supported by an Avant Doctor in Training Research Scholarship. Dr. d’Udekem has been a consultant for Merck Sharp and Dohme and Actelion. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

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