Key result
The current surgical strategy for right isomerism and functional single ventricle yielded 1- and 10-year survival rates of 78.0% and 66.2%, with pulmonary atresia strongly predicting mortality.
Why the study?
Cardiac malformations cause significant mortality and morbidity in heterotaxy syndrome with right isomerism and functional single ventricle, prompting evaluation of a surgical strategy aimed at achieving optimal Fontan haemodynamics.
What are the outcomes and predictors of mortality for the current surgical strategy in patients with right isomerism and functional single ventricle?
Cohort (n=91)
No
What are the outcomes and predictors of mortality for the current surgical strategy in patients with right isomerism and functional single ventricle?
In patients with right isomerism and functional single ventricle, the current surgical strategy yields a 10-year survival of 66.2%, with pulmonary atresia and atrioventricular valve regurgitation being significant risk factors for mortality.
Current strategy yields acceptable Fontan outcomes in RI-FSV; leaves open identification of modifiable risk factors for further mortality reduction.
OBJECTIVES: Cardiac malformations are a major component of heterotaxy syndrome that result in significant mortality and morbidity, especially in patients with right isomerism (RI) and functional single ventricle (FSV). Over the past 20 years, we have adopted a surgical strategy at initial palliation for achieving optimal Fontan haemodynamics. Here, we aimed to evaluate the mortality and morbidity associated with the current surgical strategy and to identify predictive risk factors for mortality. METHODS: A retrospective review of medical records was performed on 91 consecutive patients with RI and FSV who underwent initial palliation at Mt. Fuji Shizuoka Children's Hospital between 2000 and 2023. RESULTS: The estimated survival rates of the overall cohort at 1 and 10 years were 78.0% and 66.2%, respectively. The completion rate of the Fontan procedure excluding waiting cases was 57.1% (52/84). In multivariable analysis, pulmonary atresia (hazard ratio 5.44 [95% confidence interval, 1.57-22.2]) and the grade of common atrioventricular valve regurgitation before initial palliation (hazard ratio 2.17 [95% confidence interval, 1.07-4.76]) were predictors of mortality. Haemodynamic data from pre-Fontan cardiac catheterization were available for 52 patients. A comparison with a concurrently studied cohort of patients with non-RI and FSV (n = 253) revealed no significant differences in any of the measured haemodynamic variables. CONCLUSIONS: Surgical intervention in patients with RI and FSV remains challenging. Findings from the pre-Fontan catheter studies suggest that our current surgical strategy may provide a safe and effective pathway towards Fontan candidacy.
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Hirose et al. (2025) conducted a cohort in Right isomerism and functional single ventricle (n=91). Current surgical strategy at initial palliation was evaluated on Survival at 1 and 10 years. The current surgical strategy for right isomerism and functional single ventricle yielded 1- and 10-year survival rates of 78.0% and 66.2%, with pulmonary atresia strongly predicting mortality.
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