Combination of complete atrioventricular septal defect and tetralogy of Fallot: surgical management and its results

OBJECTIVES
Complete atrioventricular septal defect (AVSD) with tetralogy of Fallot (TOF) is a rare congenital heart anomaly. We evaluated surgical results of total repair in patients with TOF and AVSD.

STUDY DESIGN
Seven patients (5 girls, 2 boys; age range 2.5 to 14 years) underwent total repair for AVSD and TOF. Three patients had Down syndrome. Three patients had previous systemic-to-pulmonary shunt operations. Accompanying anomalies were left superior vena cava (n=2), left atrial isomerism (n=1), muscular VSD (n=1), and double outlet right ventricle (n=1). Preoperative diagnoses were based on echocardiographic examinations. The two-patch technique was used for surgical repair. Reconstruction of the right ventricular outflow tract was performed using transannular and infundibular patches in four and three patients, respectively. The follow-up period ranged from six months to nine years (mean 3.4±2.9 years).

RESULTS
No mortality occurred throughout the follow-up period. Two patients had prolonged hospitalization (>1 month) due to pulmonary infection and sepsis. At the latest follow-up, functional capacity was NYHA class I in five patients, and class II in two patients. Final echocardiographic examinations showed mild left atrioventricular (AV) valve insufficiency in five patients, and mild (n=3) or moderate (n=1) right AV valve insufficiency. Two patients had mild residual pulmonary stenosis, and three patients with a transannular patch had free pulmonary insufficiency. All the patients had proper right and left ventricular functions and all were in sinus rhythm.

CONCLUSION
With a proper surgical strategy and technique, AVSD and TOF can be corrected successfully. Long-term follow-up is necessary for AV valve dysfunction and pulmonary insufficiency.