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[Symptomatic congenital complete atrioventricular block--a medical challenge]
G Hofstadler1, G Tulzer, K Schmitt
1Department für pädiatrische Kardiologie, Landeskinderklinik Linz.
Insights
Congenital complete atrioventricular block, associated with maternal antibodies, can be diagnosed via fetal echocardiography. Steroid therapy in the mother may prevent fetal hydrops, though a normal rhythm did not recur.
Area of Science:
- Cardiology
- Immunology
- Obstetrics
Background:
- Congenital complete atrioventricular block (CCAVB) is a rare condition.
- It is frequently associated with maternal connective tissue disease and antibodies like anti-Ro (SS-A) and anti-La (SS-B).
Observation:
- Diagnosis of CCAVB can be achieved through fetal Doppler-echocardiography as early as the 16th week of gestation.
- In this case, CCAVB was diagnosed at 21 weeks gestation with ventricular and atrial rates of 55/min and 70/min, respectively.
- Maternal anti-Ro and anti-La antibodies were detected, despite no overt signs of maternal connective tissue disease.
Findings:
- Fetal hydrops was absent throughout the pregnancy, despite a continuous decrease in heart rate, reaching 28 bpm at term.
- Maternal steroid therapy was administered from diagnosis until delivery.
- No recurrence of normal cardiac rhythm was observed.
Implications:
- Steroid therapy may play a role in preventing early fetal hydrops in cases of CCAVB.
- Delivery should be considered upon the onset of fetal hydrops.
- Permanent pacemaker implantation is preferred for symptomatic neonates with CCAVB.
- Optimal management requires close collaboration between maternal and neonatal care teams.
Abstract:
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.