O70 - Long-term outcome after single ventricle palliation: A Swedish nationwide cohort study
8. Surgery and Intensive carePetru Liuba2, Mats Synnergren1, Michal Odemarsky2, Jens Johansson Ramgren2, Magnus Dalén3
1 The Queen Silvia Children´s Hospital, Sahlgrenska University Hospital, Institution of Clinical Sciences, Gothenburg University, Sweden
2 Children´s Heart Center, Skåne University Hospital, Lund, Sweden
3 Department of Molecular Medicine and Surgery, Karolinska Institutet, Department of Cardiac Surgery, Karolinska University Hospital, Stockholm, Sweden
Introduction
To examine long-term survival after surgery for univentricular heart defects.
Methods
All children in Sweden operated due to univentricular heart defects before 18 years of age from 1994 to 2019 were included. Medical records were analyzed and survival cross-checked against the National Population Registry allowing for reliable and complete follow-up. Primary outcome was all-cause mortality.
Results
778 patients (39% female) had univentricular heart surgery. Mean follow up time was 11.3 ± 8.4 years (range 0-26.7 years). 10 patients (1.3%) were lost to follow up due to emigration. 176 deaths (22.6%) occurred. Main diagnoses were; hypoplastic left heart syndrome 268 patients (34.4%), tricuspid atresia 107 (13.7%), double inlet left ventricle 90 (11.5%), atrio-ventricular septal defect 89 (11.4%), double outlet right ventricle 73 (9.4%), pulmonary atresia with intact ventricular septum 60 (7.7%), and miscellanous defects 91 (11.7%). 83 (10.7%) patients had an extracardiac defect, syndrome and/or chromosomal aberration. Atrial isomerism was diagnosed in 56 patients (7.2%). Right ventricular dominance was found in 432 (55.5%) patients, left in 303 (38.9%), biventricular in 40 (5.1%) and indeterminate morphology in 3 (0.4%). Stage I palliation was performed in 716 patients with 30-day mortality in 53 (7.4%) and late mortality in 60 (8.4%). Bidirectional cavopulmonary connection was performed in 645 patients, with 30-day mortality in 3 (0.5%) and late mortality in 26 (4%). Total cavo-pulmonary connection was performed in 547 patients with 30-day mortality in 1 (0.4%) and late mortality in 23 (4.2%). Survival at ten years of age in patients with right ventricular dominance was significantly lower as compared to patients with left or biventricular dominance (65.5% vs 89.4%, p<0.001), but similar in patients with and without atrial isomerism. At follow-up, 11 patients were alive with stage I palliation, 50 with bidirectional cavo-pulmonary connection and 508 with total cavo-pulmonary anastomosis. Heart transplantation was performed in 28 patients (3.6%).
Conclusions
Overall long-term survival was 77.4% with a significantly higher survival in patients with left ventricular dominance as compared to patients with right ventricular dominance (89.4% vs 65.5%, p<0.001). Heart transplantation was performed in 3.6% of all patients.