[Symptomatic congenital complete atrioventricular block--a medical challenge]

Congenital complete atrioventricular block is a rare entity. The association between this disease, maternal connective tissue disease and maternal antibodies [anti-Ro (SS-A) resp. anti-La (SS B)] is well known. Diagnosis can be made by means of fetal Doppler-echocardiography by the 16th week of gestation. In our case diagnosis was established in the 21st week of gestation. Ventricular rate was 55/min, atrial rate 70/min. There were no signs of fetal hydrops. There were no signs of maternal connective tissue disease, but anti-Ro and anti-La antibodies could be detected. The mother was treated with steroids from the time of diagnosis until the end of pregnancy. Altogether 9 Doppler-echocardiographic studies were performed. A recurrence of normal rhythm did not occur. A slow but continuous decrease of atrial and ventricular rate was observed. Interestingly, there was no development of fetal hydrops until the very end of pregnancy when the fetal heart rate reached a low of 28 beats per minute. We speculate, that the therapy with steroids might have played an important role in the prevention of early hydrops. At the onset of fetal hydrops delivery should be considered. In symptomatic complete atrioventricular block we prefer the implantation of a permanent pacemaker system immediately after birth. Efficient care for the fetus resp. the newborn can only be achieved through well planned cooperation.