Abstract
Background
Complete atrioventricular septal defect (CAVSD) with tetralogy of Fallot (CAVSD-ToF) is an uncommon association, and the management is more difficult than for each lesion in isolation. Depending on the anatomy, clinical status and ventricular morphology, definitive repair is offered either as staged or single operation with few undergoing single or one and a half ventricle procedures. Currently available data are limited to single centre studies.
Purpose
We aim to use data from a national audit dataset to describe outcomes in the surgical management of CAVSD-ToF with data from the national audit.
Methods
All children with CAVSD and tetralogy of Fallot (including double outlet right ventricle and pulmonary atresia variants) undergoing the first cardiac procedure between 2000–2013 were included and all subsequent procedures extracted. Three treatment pathways were defined: definitive primary procedure (“primary complete repair”), staged repair or “unbalanced ventricles repair” (Glenn shunt or modified Fontan). Continuous data is expressed as median (range). Survival data obtained using the Kaplan Meier method and subgroup comparisons done using the log-rank test.
Results
A total of 125 patients were included, 50.4% male, first procedure at 150 (1–2770) days. Down syndrome was present in 79.4% (63/79 of those with reported non-cardiac comorbidities), while 8% (10/125) had heterotaxy syndrome. “Primary complete repair” was performed in 40% (n=50), at 319 (33–2770) days. Staged repair was undertaken in 28% (n=35), first palliation at 80 (1–733) days. An “unbalanced ventricles” approach was used in 16.8% (n=21), first procedure at 135 (1–1742) days). The other 15.2% (n=19), first procedure at 53 (range 1–436) days, had at least one palliation procedure, but no identifiable committed pathway.
Survival at 5 years from the first procedure in “primary complete repair” compared to the remainder of the group is shown in the Figure. No significant differences in survival were found. Specifically in the “unbalanced ventricle repair” group, just 1 patient died during follow-up.
A total of 85 patients (68%) achieved either primary or staged “complete repair”. Early mortality (30 day) after the “complete repair” was 6.6% (similar for both approaches, p=0.9). Survival at 5 years after “complete repair” was 82.7% (similar for both approaches, p=0.8). Freedom from any cardiac re-intervention at 5 years was 69.5% (77.6% after primary, 58.1% after staged repair, p=0.03).
Conclusion
Early-life mortality in this heterogeneous group of CAVSD-ToF remains high, even in patients receiving definitive “complete repair”. We found no survival differences in the medium term between primary repair and a palliation approach, but more cardiac re-interventions were undertaken after staged complete repair. Surprisingly good results were seen in the unbalanced ventricles group, but longer follow up is needed to examine their attrition and longer term outcome.
Survival at 5 years by treatment option
Funding Acknowledgement
Type of funding source: None
A Single Misstep in Cardiac Development Explains the Co-Occurrence of Tetralogy of Fallot and Complete Atrioventricular Septal Defect in Down Syndrome