Despite early pacing, CHB carries high mortality during the first 12 months of life. High incidences of DCM and associated heart defects indicate close echocardiographic monitoring of all children with CHB.

Our results confirm that in addition to high survival rate, the long-term psychosocial outcome of patients with surgically treated congenital heart defects is good if they do not have any additional syndromes that cause mental retardation.

In many very-low-birth-weight (VLBW) infants the ductus arteriosus fails to close spontaneously, and they subsequently develop signs and symptoms of poor tissue perfusion and heart failure. This study evalutes the results of early surgical closure of patent ductus arteriosus (PDA). We retrospectively reviewed the records of all 101 VLBW infants who weighed 1,500 g or less when their PDA was surgically ligated in Turku University Hospital between 1988 and 1998. The mean gestational age at birth was 27.2 weeks and mean birth weight 963+/-239 g. The operation was performed at 12+/-8 days of age; the infants' weight at operation was 969+/-231 g and they were tracheally extubated 11+/-14 days after the operation. The surgery-related mortality was 3% (3/101) and overall mortality 10% (10/101). We conclude that surgical closure of PDA is safe and effective in VLBW infants.

Congenital heart block (CHB) is a syndrome of uncertain pathogenesis leading to cardiac conduction disturbances in the foetus and newborns. It has been proposed that maternal antibodies transmit immunological injury in the developing foetal heart, thus causing irreversible damage of the atrioventricular node, leading to third-degree atrioventricular block. However, some genetic or environmental factors may also be involved. We have searched for genetic markers that play a role in immune response and that would be pathognomonic for the disease, either in mothers by regulating their immune response or in children by affecting antigen presentation and target for the maternal immune response. We have compared HLA class I and II alleles of the children with their mother and with healthy individuals and searched for HLA markers that would be emphasized in children. We have shown that particular DQ alleles in the child predispose to CHB, perhaps serving as antigen-presenting molecules on site. In addition, the HLA-Cw3 allele is involved, although its function remains to be clarified. In our results, children with CHB were often identical to their mothers in alleles of DRB, DQA and DQB loci, thus affecting foetomaternal recognition and suggesting that cell-mediated mechanisms could be involved in the pathogenesis.

No abstract