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Congenital complete atrioventricular block (CCAVB) is a rare and potentially lethal disease with an estimated incidence of 1 in 15.000 to 20.000 live born infants. Most of the patients with CCAVB have structurally normal hearts, referred to as an 'isolated' CCAVB.
We present the case of a premature infant with CCAVB who underwent implantation of a permanent pacemaker. The male infant was born at 33 weeks of gestation and weighed 2150 g. Repeat fetal ultrasound assessment before demonstrated fetal cardiomegaly increased at 30 weeks gestation. The decision was made to deliver the baby by cesarean section at 33 0/7 weeks gestation. After birth, the infant showed respiratory distress despite antenatal corticosteroid therapy. There were no clinical signs of hydrops fetalis. The heart rate ranged between 40 and 50 bpm. An electrocardiogram showed that the rate of P wave was 120 bpm and the rate of QRS wave was 50 bpm. The chest x-ray demonstrated dilated heart and echocardiogram showed dilated chambers, small non significant PDA with left to right shunt, no ASD or VSD, and satisfactory contracted ventricles. Respiratory problem was resolved after supportive treatment with temporary pacing. He underwent succesfull implantation of a permanent transepicardial pacemaker (VVIR mode, stimulation rate 120 bpm, output 1,5 mV and sensitivity 2,6 mA). A unipolar epicardial lead was used and the pulse generator was implanted in a pocket made under at the anterior rectus sheath. Surgery was performed without any complications. There was no respiratory problem associated with pacemaker implantations in the abdominal wall. He was discharged at the age of 31 days with a weight of 2350 g. At the 1-year follow up he remains in well condition without any complications.
We have reported a case of a CCAVB with succesfull implantation of permanent pacemaker.