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Congenital complete heart block
1Department of Pediatrics, Taichung Veterans General Hospital, 160, Sec 3, Chung-Kang Road, Taichung 407, Taiwan.
Insights
Congenital complete heart block (CCHB) in infants is linked to maternal autoantibodies, often seen in neonatal lupus. Pacemaker insertion in newborns has a high complication rate, suggesting it should be used cautiously.
Area of Science:
- Pediatrics
- Cardiology
- Immunology
Background:
- Congenital complete heart block (CCHB) is frequently associated with neonatal lupus syndrome.
- Maternal autoantibodies play a significant role in the pathogenesis of CCHB.
Purpose of the Study:
- To evaluate the clinical spectrum of congenital complete heart block (CCHB) in infants.
- To investigate the association between CCHB and maternal autoantibodies.
Main Methods:
- Retrospective analysis of nine CCHB cases diagnosed between 1994 and 1999.
- Review of birth history, electrocardiography, Holter monitoring, pacemaker data, maternal disease, and autoantibody levels.
Main Results:
- All nine CCHB cases were diagnosed prenatally and associated with maternal autoantibodies (ANA, anti-SSA/Ro, anti-SSB/La).
- Five cases (55.6%) presented with hydrops fetalis; three pregnancies were terminated.
- Permanent pacemaker insertion in live-born infants had a 50% complication rate.
Conclusions:
- Maternal autoantibodies are consistently linked to CCHB.
- Due to high complication rates, permanent pacemaker insertion in neonates with CCHB should be reserved for specific indications.
Abstract:
Congenital complete heart blocks (CCHB) are mostly related to the neonatal lupus syndrome. The purpose of this paper was to assess the clinical spectrum of CCHB in our hospital. Nine patients were retrospectively enrolled between 1994 and 1999. The birth history, electrocardiography, 24-hour Holter monitoring, pacemaker insertion and its complications, maternal disease, and maternal and infant autoantibody levels were studied. All nine cases were diagnosed prenatally. Hydrops fetalis was noted in five (55.6%). Six cases were live births and the other three were terminated. No anatomical heart defects were noted. Initial electrocardiography revealed the atrial rates ranged from 150 to 166 beats per minute. The minimal ventricular rates ranged from 46 to 80 beats per minute. VVI mode pacemakers were inserted through xyphoid approach in all live-birth infants. Complications were noted in three of them (50%). Antinuclear antibody and anti-SSA/Ro antibody were positive in all 8 mothers (100%). The anti-SSB/La antibody was positive in 6 of the eight mothers (75%). Five infants tested positive for anti-SSA/Ro antibody. None of the infants tested positive for anti-SSB/La antibody. In conclusion, all CCHBs in our series were associated with maternal autoantibodies. Because of high complication rate of permanent pacemaker insertion during the neonatal period, it should be restricted in certain conditions.