Patients with single ventricle physiology and a common atrioventricular valve experienced a continuous decline in valve function, with a 62% cumulative incidence of valve failure at 28 years.
Cohort (n=136)
Yes
What is the long-term risk of atrioventricular valve failure in Fontan survivors with single ventricle physiology and common atrioventricular valve?
In Fontan survivors with a common atrioventricular valve, there is a continuous decline in valve function, with 62% experiencing valve failure by 28 years.
OBJECTIVES: To determine the risk of atrioventricular valve failure (valve intervention or moderate or greater regurgitation) during the lifetime of patients with single ventricle physiology and common atrioventricular valve. METHODS: Patients' data were extracted from an existing bi-national, population based registry. A retrospective review of their medical records was undertaken to determine the incidence of atrioventricular valve repair/replacement or moderate or greater regurgitation. RESULTS: From a registry of 1468 Fontan survivors, 136 patients with common atrioventricular valve were identified. Complete echocardiographic follow-up was available for 114 patients. Median length of follow-up was 10.2 years (interquartile range 5-15 years). Twenty-five year survival and freedom from Fontan failure were 94% 95% confidence interval (CI), 88-100% and 74% (95% CI, 64-87%), respectively. Twenty-eight patients underwent 24 initial repairs and 4 replacements. The 24 patients undergoing repair subsequently needed 6 re-repairs, 2 replacements and 8 had moderate or greater regurgitation at last follow-up. Four-year freedom from atrioventricular valve repair failure was 50% (95% CI, 34-75%). An additional 30 patients developed moderate or greater atrioventricular valve regurgitation (6 New York Heart Association ≥3, 10 Fontan failures, 0 deaths). Cumulative incidence of the composite endpoint of atrioventricular valve failure at 28 years was 62% (95% CI, 49-74%). CONCLUSIONS: Patients with single ventricle physiology and common atrioventricular valve experience a continuous decline in valve function. The majority of patients experience valve failure in the first 30 years of life.
King et al. (Tue,) conducted a cohort in Single ventricle physiology and common atrioventricular valve (n=136). Observation was evaluated on Composite of atrioventricular valve failure (valve intervention or moderate or greater regurgitation) at 28 years (95% CI 49-74). Patients with single ventricle physiology and a common atrioventricular valve experienced a continuous decline in valve function, with a 62% cumulative incidence of valve failure at 28 years.