Significant atrioventricular valve regurgitation at diagnosis in single-ventricle patients has been associated with mortality and morbidity. However, longitudinal data on the effect of valve regurgitation at diagnosis on outcomes in the era of surgical valve interventions are scarce.

This is a retrospective review of single-ventricle patients admitted to a regional centre from 2005 to 2008. Data were reviewed from birth to 18 months, and association of atrioventricular valve regurgitation at diagnosis with mortality and morbidity was evaluated.

A total of 118 patients were studied, 73% with a single right ventricle. At diagnosis, 37 patients (31%) had mild, 5 (4%) had mild to moderate, and 4 (3%) had ≥ moderate atrioventricular valve regurgitation. Moderate or greater valve regurgitation was associated with mortality (HR 5.51, 95% CI 1.24–24.61, p = 0.025), and all four patients with ≥ moderate valve regurgitation died. However, valve regurgitation was not associated with mortality for left ventricle patients. In all, 12 patients (10%) had surgical atrioventricular valve interventions. There were no independent predictors of valve intervention, and no patient having an intervention had > mild valve regurgitation at diagnosis. There was no association between valve regurgitation and days of hospitalisation or chest tube drainage.

Significant atrioventricular valve regurgitation at diagnosis remains a risk factor for mortality in single-ventricle patients, although it may be less important for single left ventricle patients. However, it is not associated with increased morbidity or surgical atrioventricular valve intervention in survivors. Reliably predicting surgical atrioventricular valve intervention remains a challenge in single-ventricle patients.

The objective of this study was to evaluate the early and late results of atrioventricular valve surgery after Fontan operation.

Atrioventricular valve regurgitation is a known perioperative risk factor for Fontan operation. There are limited data on the outcomes of late atrioventricular valve surgery following Fontan operation.

Patients who underwent atrioventricular valve surgery following Fontan procedure were identified from the Mayo Clinic Fontan database. Medical records were reviewed for pre-operative, operative, and post-operative clinical and haemodynamic data. All patients not known to be deceased were sent health status questionnaires.

A total of 61 patients (28 females) underwent atrioventricular valve surgery following Fontan procedure. The median age at atrioventricular valve surgery was 14 years. The median duration between Fontan and atrioventricular valve surgery was 4.7 years. Median follow-up was 9 years. There were a total of 32 (52%) deaths with 8 (13%) within 30 days of surgery. The 5-, 10-, and 15-year survival rates were 67%, 57%, and 45%, respectively. On follow-up, 44 of 61 (72%) had arrhythmias, 21 of 29 (72%) were symptomatic, and 12 of 61 (20%) developed protein-losing enteropathy. On multivariate analysis, reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

Atrioventricular valve surgery after Fontan procedure is associated with substantial late morbidity and mortality. Atrioventricular valve surgery in this cohort of patients portends poor long-term outcome and is associated with a high incidence of protein-losing enteropathy. Reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.