COMPLETE ATRIOVENTRICULAR SEPTAL-DEFECT WITH TETRALOGY OF FALLOT - DIAGNOSIS AND MANAGEMENT

Objective-To report recent experience of patients with complete atriov entricular septal defect and tetralogy of Fallot, with emphasis on ana tomical features, diagnosis, and management. Design-Case notes were re viewed and patients were assessed at follow up by clinical examination and cross sectional and Doppler echocardiography. Setting-Tertiary ca rdiothoracic referral centre. Patients-Between 1987 and 1999 13 patien ts with atrioventricular septal defect and tetralogy of Fallot (12 wit h concordant and one with double outlet ventriculoarterial connections ) underwent surgery; 10 underwent complete intracardiac repair. 11 pat ients had Down's syndrome. The complete diagnosis was established preo peratively by cross sectional echocardiography in all but one patient. A tri-leaflet left atrioventricular valve as seen in parasternal shor t axis views was the diagnostic feature of atrioventricular septal def ect, with tetralogy of Fallot diagnosed from the presence of anterocep halad deviation of the outlet septum producing subvalvar pulmonary ste nosis as seen in subcostal right anterior oblique views. Interventions -Total correction consisted of closure of the atrioventricular septal defect by a combined right atrial and ventricular approach, reconstruc tion of the atrioventricular valves, and relief of the obstruction wit hin the right ventricular outflow tract. Separate patches were used to close the atrial and ventricular septal defects. Modified Blalock-Tau ssig shunts were performed in three patients, who await intracardiac r epair. Surgical correction was carried out at mean (range) age of 5 (2 to 15) years. Main outcome measures-Diagnostic methods, surgical resu lts, and functional state after complete correction. Results-The prese nce of an atrioventricular septal defect was missed preoperatively in one patient with tetralogy of Fallot. The characteristic goose neck de formity on the left ventriculogram was not present and the tri-leaflet nature of the left atrioventricular valve was not sought on echocardi ography. Of the 10 patients who underwent complete repair, nine are al ive and one died 34 days after operation with adult respiratory distre ss syndrome. Examination at necropsy showed an excellent surgical corr ection. Mean (range) follow up was 23 (8 to 48) months. Al nine patien ts are alive and well (New York Heart Association Class 1). Conclusion -Accurate diagnosis and staged management with improved surgical techn iques have lowered mortality of this complex combination of cardiac de fects. The current policy of this group is to recommend a systemic to pulmonary arterial shunt procedure for symptomatic children younger th an 2 years and total correction in older children.