One of the important causes of congenital heart block is the transplacental transmission of maternal anti-Ro (SSA) antibody. An 18 month old girl was brought to our hospital because of dyspnea from which she had been suffered after birth. A
complete
heart block, atrial septal defect (secundum type) and patent ductus arteriosus wer found. Closure of defects was done and permanent pace maker installe. She died of pneumonia and cong4estive heart fail ure at the age of 30 months. Her mother had
this
baby at the age of 26 years old and had suffered from systemic sclerosis since her early twenties. The laboratory studies on her revealed as follows. ANA 1 : 2650,. Anti-nRNP Ab (-), anti-Scl 70 Ab (-), anti-centromenre Ab (-), rheumatoid factor
)-),
anti-Ro (SSA) Ab (+), anti-La (SSB) Ab (+), ionterstitial pulmonary fibrosis (DLCO : 50% of predicted value).
|