Repair of Atrioventricular Canal Defects: Long-Term Outcome

Objectives: Atrioventricular (AV) canal defects are rare but complex congenital diseases characterized by the presence of abnormal communications between the atria and ventricles along with malformations of the AV valves. These defects lead to pulmonary overload resulting in pulmonary hypertension and heart failure. Down syndrome is often associated with AV-canal defects. The aim of the study was the analysis of outcomes after correction of AV-canal defects.

Methods: We performed a retrospective study of patients who underwent repair of AV-canal defects during a 20-year period (from 1986 to 2016). Mean follow-up was 113 months.

Results: During the study period 241 patients (male n = 100 and female n = 141) underwent surgical repair of AV-canal defects, 124 patients (51%) had had Down´s syndrome. Twenty-one patients had undergone previous cardiac surgery, mostly pulmonary artery banding (n = 13). Regarding the type of the defect most patients had had complete AV-canal defect (n = 132). In most cases of complete defects the repair was performed using the single-patch or the modified single-patch technique (n = 86 and n = 17, respectively). The 30-day mortality was 6.2%. In 20 patients, a permanent pacemaker was implanted. During follow-up, 30 patients underwent redo procedures. The most common indications for re-operation were left (mitral) AV-valve regurgitation and left ventricular outflow tract obstruction. The echocardiographic examination at last follow-up appointment demonstrated a good function of the left-sided AV-valve, in most patients with no or mild regurgitation (n = 157) and moderate regurgitation (n = 35). There was no case of severe regurgitation.

Conclusion: Long-term outcome after repair of AV-canal defects is very good with low re-operation rate during long-term follow-up.

Publication History

Article published online:
10 June 2022