The History of Partially Palliated (Post-Bidirectional Glenn) Patients with Univentricular Physiology in Mumbai,India

Abstract

Background

Management of patients with univentricular physiology requires multiple palliative surgeries. In India, these patients often remain partially palliated with the bidirectional Glenn (BDG) procedure due to delayed presentation and limited resources. We performed this study to assess the clinical outcomes of these patients.

Methods and Results

This was a single-center, retrospective cohort study. All patients with univentricular physiology who had undergone a BDG without having undergone a Fontan procedure were included. Clinical failure was defined as a composite of death, severe systemic ventricular dysfunction, and/or severe impairment of exercise capacity on cardiopulmonary exercise testing. Kaplan-Meier survival analysis and univariate analysis were performed. The study included 37 patients. The mean age at the time of evaluation was 18.7 ± 6.1 years. Mean age at BDG was 7.5 ± 5.58 years. There were 22/32 (68.8%) of patients who experienced clinical failure in our study cohort. The median time to clinical failure was 14 years. Among those eligible for Fontan, only 4/23 (17.4%) underwent Fontan completion. Of those awaiting Fontan (7/19, 36.8%) had been deferred earlier as they were deemed to be at high risk for surgery.

Conclusion

In resource-limited settings, it may be common to see patients with partial palliation with BDG ultimately experience “clinical failure” and need Fontan completion for alleviation of their symptoms. Among those eligible, the proportion of patients eventually undergoing a Fontan procedure is small.