Congenital complete heart block.
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.
Department of Pediatrics, Taichung Veterans General Hospital, 160, Sec 3, Chung-Kang Road, Taichung 407, Taiwan.
M C
MC
Lin
Y C
YC
Fu
J J
JJ
Tseng
S L
SL
Jan
C S
CS
Chi
D000974:Antibodies, Antinuclear / Q000097:blood; D004562:Electrocardiography; D005260:Female; D006327:Heart Block / Q000151:congenital* / Q000276:immunology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies
D000974:Antibodies, Antinuclear; C035356:SS-A antibodies