Long-Term Outcome of Patients With Complete Atrioventricular Septal Defect Combined With the Tetralogy of Fallot: Staged Repair Is Not Inferior to Primary Repair

Background: Surgical strategies vary for repair of complete atrioventricular septal defect (AVSD). The impact of age, surgical technique and trisomy 21 on contemporary outcomes is poorly defined. Methods: From 6/04 to 2/06, 124 children with primary repair of complete AVSD were studied in a Pediatric Heart Network sponsored observational study. Demographic, procedural, and outcome data were obtained within 28 days of surgery and 6 months later. Results: Median age at repair was 115 days (9 – 396 days). Type of surgical repair was single patch (19%), double patch (71%), and single ASD patch with primary VSD closure (10%). Repair types varied significantly across centers (p=0.002) but there were no differences noted in residual atrial or ventricular septal defects or degree of mitral regurgitation (MR) by repair type within 28 days. Median intensive care unit (ICU) stay was 4 days, length of ventilation 2 days, total hospital stay 7 days; there were no differences by presence of trisomy 21(80% of cohort). A patch margin VSD was detected by echo within 28 days in 43% and at 6 months in 14% (p<0.05). A VSD > 3mm was seen in 6% at discharge and 1% at 6 months. At least moderate MR was present in 26% early and 22% at 6 months. Hospital mortality was 3/124 (2.4%); one within 30 days (0.8%). Overall survival at 6 months was 96% (119/124). Pleural effusion was the most common reported postoperative complication (20%). Earlier age at surgery was associated with longer time on ventilator (p=0.02), longer ICU stay (p=0.03) and use of circulatory arrest (p=0.01). Older age was associated with longer duration of aortic cross clamp time (p=0.05). Age at repair was not associated with residual abnormalities including residual VSD and moderate or greater MR at 6 months. . Conclusions: Contemporary outcomes following repair of complete AVSD are excellent regardless of repair type or presence of trisomy 21. Although a residual VSD may be detected by echo in 43%, most are trivial and resolve by 6 months. Earlier age at surgery is associated with increased resource utilization but has no influence on incidence of residual VSD or significant MR.

Download Full-text

Surgical management of complete atrioventricular septal defect with tetralogy of Fallot: results from a national audit database

European Heart Journal ◽

10.1093/ehjci/ehaa946.2200 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

D Dorobantu ◽

R Tulloh ◽

S Stoica

Keyword(s):

Tetralogy Of Fallot ◽

Surgical Management ◽

Septal Defect ◽

Atrioventricular Septal Defect ◽

National Audit ◽

Treatment Pathways ◽

Complete Atrioventricular Septal Defect ◽

Complete Repair ◽

Complete Atrioventricular

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

Download Full-text