1. Long-term outcomes of patients with single ventricle who do not undergo Fontan palliation
- Author
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Wayne C. Zheng, Yves d’Udekem, Leeanne E. Grigg, Dominica Zentner, Rachael Cordina, David S. Celermajer, Edward Buratto, Igor E. Konstantinov, and Melissa G.Y. Lee
- Subjects
Arrythmia ,Fontan ,Heart failure ,Mortality ,Outcomes ,Single ventricle ,Diseases of the circulatory (Cardiovascular) system ,RC666-701 - Abstract
Background: Patients with single ventricle (SV) without Fontan palliation are uncommon, and their long-term outcomes remain unclear. Methods: Retrospective study of 35 adult patients with SV without Fontan from two tertiary centers. Primary outcome was mortality. Results: Median age at first follow-up was 31 years (IQR: 20–40). Most common defect was double inlet left ventricle (34%), and 69% had left ventricular morphology. Patients were unoperated (46%), had systemic-to-pulmonary artery shunt (31%) or bidirectional cavopulmonary shunt (23%) as final palliation. Most common reasons for not progressing to Fontan palliation were pulmonary vascular disease (54%), patient refusal (17%), Fontan takedown (14%), and hypoplastic pulmonary arteries (11%). Baseline mean hemoglobin was 195 ± 29 g/L, mean O2 saturation 83 ± 6.9%, and 4 patients in NYHA Class III‒IV. After a mean follow-up of 10 ± 8.3 years, there were 9 deaths with heart failure being the leading cause (n = 6). Age-adjusted survival of these adult SV survivors was 73% and 53% at 40 and 50 years of age, respectively. Deceased patients more frequently had renal impairment (50% vs 0%) and QRS prolongation (75% vs 16%) at baseline (all p
- Published
- 2023
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