Original article: cardiovascular
Anatomically corrective repair of complete atrioventricular septal defects and major cardiac anomalies

https://doi.org/10.1016/S0003-4975(01)02674-1Get rights and content

Abstract

Background. Although satisfactory results of corrective surgery for atrioventricular septal defects (AVSDs) with complex lesions such as double-outlet right ventricle (DORV) or atrial isomerism have been reported in recent years, the optimal surgical options for isomerism hearts is still a controversial issue.

Methods. We performed anatomically corrective repair on 13 children with balanced forms of complete AVSDs and associated major cardiac anomalies. Eight of the 13 patients had atrial isomerism (right in 1, left in 7), 5 of whom had DORV. Four others had DORV with trisomy 21, and 1 had tetralogy of Fallot. Atrial septation for isomerism with the placement of an additional prosthesis was performed on 4 patients.

Results. Two hospital deaths and one late death occurred only in the isomerism group. Three reoperations were required solely in the left isomerism group: one replacement of the valved external conduit concomitantly with reconstruction of the left ventricular outflow obstruction, one mitral valve replacement for severe regurgitation of left atrioventricular valve, and one relief of progressing left ventricular outflow obstruction.

Conclusions. The results of anatomically corrective surgery for AVSDs with major associated cardiac anomalies in the nonisomerism group were excellent. The optimal surgical options for isomerism heart, however, remain a controversial issue.

Section snippets

Patient population

Thirteen patients with balanced forms of complete AVSDs and associated major cardiac anomalies underwent anatomically corrective repair between January 1988 and February 2000 at Kobe Children’s Hospital. Eight of the 13 patients had atrial isomerism (right in 1, left in 7) with DORV in 5 and associated cardiac anomalies in 3. Four of the other patients had DORV with trisomy 21, and 1 had tetralogy of Fallot. Their ages at operation ranged from 5 months to 10 years (median 4.7 years). All but 1

Results

Two hospital deaths (15%) occurred only in the isomerism group. One (patient 1) had a cardiac arrest 15 hours after the operation; another (patient 6) had severe right ventricular cardiac failure and died 8 days postoperatively. Doppler echocardiography immediately after each operation revealed moderate to severe mitral incompetence in 1, moderate in 2, mild in 5, and trivial in 3.

Patients were followed up from 2 months to 12 years (average 4.4 years). There was one late death in the isomerism

Comment

Recently satisfactory results of corrective surgery for complete AVSDs with complex cardiac lesions such as DORV or atrial isomerism have been reported 1, 2, 3, 4. In Kobe Children’s Hospital, DORV has been observed in 14% of the patients who underwent corrective repair of complete AVSDs. One-third of the candidates for definitive repair among the isomerism patients received corrective surgery, the goal of which involves both anatomical and physiological correction of these complex anomalies.

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