Forty-year survival after Glenn procedure without Fontan procedure in patients with single ventricle

OBJECTIVES: There are no data on long-term outcomes beyond 30 years after the Glenn procedure without the subsequent Fontan procedure in patients with single-ventricle physiology. Hence, this study aimed to clarify the very long-term outcomes of these patients.METHODS: This single-centre, retrospective cohort study investigated the clinical outcomes of patients with single-ventricle physiology who underwent the Glenn procedure between 1970 and 1999. Those who underwent the subsequent Fontan procedure were excluded. The primary outcome was all-cause death. The secondary outcome was a composite of all-cause death, arrhythmic events, neurological events or infective endocarditis. The prognostic factors associated with the long-term outcomes were also evaluated.RESULTS: In total, 36 patients were enrolled (median age at Glenn procedure: 6.2 years, 56% male). During a median follow-up of 17.6 years (interquartile range: 6.1-33.4), 21 patients died and 29 experienced the composite outcome. The 20-, 30- and 40-year overall survival after the Glenn procedure was 51.2%, 44.4% and 40.3%, respectively. The 20-, 30- and 40-year event-free survival was 36.0%, 25.5% and 14.5%, respectively. Patients with dominant left ventricular morphology had better overall survival than those with dominant right ventricular morphology (hazard ratio: 0.24, 95% confidence interval: 0.08-0.76, P = 0.014). None of the patients had liver cirrhosis but 1 had protein-losing enteropathy.CONCLUSIONS: The 40-year overall survival after the Glenn procedure without the subsequent Fontan procedure in patients with single-ventricle physiology was 40.3%. Dominant left ventricular morphology may be associated with better long-term overall survival than dominant right ventricular morphology.