Original article
Pediatric cardiac
Repair of Atrioventricular Septal Defect Associated With Tetralogy of Fallot or Double-Outlet Right Ventricle: 30 Years of Experience

https://doi.org/10.1016/j.athoracsur.2012.02.070Get rights and content

Background

The surgical outcomes of atrioventricular septal defect (AVSD) associated with tetralogy of Fallot (TOF) or double-outlet right ventricle (DORV) have improved in recent times. However, high mortality and reoperation rates are still reported. This study reviews our surgical experience in patients with complete AVSD and TOF or DORV.

Methods

Between 1980 and 2010, 48 consecutive patients with AVSD associated with TOF (n = 26) or DORV (n = 22) underwent complete repair; of which, 19 had staged repair. A transatrial-transpulmonary approach with the 2-patch technique was the preferred surgical technique. Data were obtained from inpatient and outpatient medical files.

Results

The mortality rates were 8.3% (4 of 48) for in-hospital death and 13.6% (6 of 44) for late death. Mean follow-up was 8.0 ± 8.7 years (median 10.8 years; range from 2 months to 30 years). Actuarial survival was 76% at 5 years and 71% at 20 years. No deaths occurred after 2001. Reoperations were required in 16 of 48 patients (33%). The overall freedom from reoperation was 55% at 5 and 20 years. Down syndrome was protective for reoperation (p = 0.022).

Conclusions

Complete AVSD associated with TOF or DORV can be repaired with good survival. Detachment of the superior bridging leaflet provided excellent exposure. However, reoperation rate remains high.

Section snippets

Patients and Methods

This study was approved by the ethics committee of the Royal Children's Hospital. Between February 1980 and December 2010, 48 consecutive CAVSD–TOF/DORV patients (26 boys, 22 girls) underwent biventricular repair at the Royal Children's Hospital in Melbourne (Fig 2). Median age was 1.8 years (range, 0.2 to 14.8 years). Diagnosis was determined by echocardiogram or cardiac catheterization in all patients. DORV was diagnosed in 22 of 36 patients (45.8%), defined as aortic override greater than

Results

Between January 1980 and December 2010, 26 patients were operated on for CAVSD-TOF. This represented 1.7% of all TOF patients and 5.9% of all CAVSD patients operated on during the same interval. During this interval, 22 patients were operated on for CAVSD-DORV. This represented 13.6% of all DORV patients and 5.2% of all CAVSD patients. Detachment of the superior bridging leaflet was introduced in 1999 and was used in 13 patients, 1 of 21 (4.8%) from 1990 to 1999 and 12 of 17 (70.6%) from 2000

Comment

The CAVSD-TOF/DORV is a rare combination of cardiac anomalies. At the Royal Children's Hospital in Melbourne, 26 patients were operated on for CAVSD-TOF between February 1980 and December 2010. This represented 1.7% of all TOF patients and 5.9% of all CAVSD patients operated on during the same interval. During this time, 22 patients were operated on for CAVSD-DORV. This represents 13.6% of all DORV patients and 5.2% of all CAVSD patients. These figures are similar to those reported in other

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