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Outcome of the Glenn procedure as definitive palliation in single ventricle patients
Astrid Vermaut1, Pieter De Meester2, Els Troost3
1Faculty of Medicine, Department of Internal Medicine, KU Leuven, Leuven, Belgium.
Insights
Single ventricle Glenn (SV-Glenn) palliation offers a reasonable outcome for selected patients, though heart failure and arrhythmias are common. Classic Fontan complications like cirrhosis were absent in this unique patient group.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Single ventricle (SV) physiology presents complex challenges in pediatric cardiac care.
- The Glenn procedure can serve as definitive palliation for select SV patients.
- The decision for SV-Glenn palliation is made when Fontan completion is unsuitable or not preferred.
Purpose of the Study:
- To describe the clinical course of patients undergoing single ventricle Glenn (SV-Glenn) procedure as definitive palliation.
- To evaluate the long-term mortality and morbidity associated with the SV-Glenn approach.
Main Methods:
- Retrospective analysis of SV-Glenn patients followed at University Hospitals Leuven before May 2018.
- Exclusion of patients undergoing or awaiting total cavopulmonary connection (TCPC) completion and those with one-and-a-half repair (OAHR).
Main Results:
- Of 44 SV-Glenn patients, 19 died within 6 months post-procedure.
- Among 25 survivors, long-term follow-up (median 11 years) showed 20% mortality.
- Common morbidities included heart failure (40%), arrhythmias (36%), and pacemaker implantation (28%); classic Fontan complications were absent.
Conclusions:
- SV-Glenn patients form a distinct and varied population.
- The SV-Glenn procedure offers a reasonable outcome, but requires careful management of comorbidities like heart failure and arrhythmias.
- Absence of cirrhosis and protein-losing enteropathy distinguishes SV-Glenn from complete Fontan physiology.
Objectives:
In selected single ventricle patients, a Glenn procedure (SV-Glenn) may be considered as definitive palliation. Either the patient is unsuited to progress to a Fontan circulation or a SV-Glenn circulation is preferred. This study aimed at describing the clinical course, and long-term mortality/morbidity of SV-Glenn patients.
Methods:
All SV-Glenn patients followed at the University Hospitals Leuven before May 2018 were included. Patients who underwent, or were awaiting, TCPC completion and those who underwent a Glenn in the setting of a biventricular circulation one-and-a-half repair (OAHR), were excluded.
Results:
Of 65 Glenn-only patients identified, 21 (32%) had OAHR, whereas 44 (68%) were SV-Glenn patients. Of SV-Glenn patients, 19 died within 6 months after the Glenn procedure. Of 25 SV-Glenn survivors, median age at Glenn was 6.3 (IQR 1.2-29.7) years. Eight were unsuited for TCPC completion; in 17 SV-Glenn was preferred over TCPC completion. Over a median follow-up time of 11 (IQR 3-18) years after the Glenn procedure, 5 (20%) patients died. At latest follow-up 10 (40%) had heart failure, 5 (20%) had atrial and 4 (16%) ventricular arrhythmias, 2 (8%) a thromboembolic event, 7 (28%) required pacemaker implantation, and 2 (8%) had infective endocarditis but none developed cirrhosis or protein-losing enteropathy. Mean saturation at latest follow-up was 87 ± 7%.
Conclusion:
SV-Glenn patients represent a unique and heterogeneous patient population. Outcome was reasonable, although comorbidities, such as heart failure and arrhythmias were not uncommon. In SV-Glenn patients, 'classic' complications related to Fontan physiology, such as cirrhosis and protein-losing enteropathy, were absent.
