ISOLATED CONGENITAL COMPLETE HEART-BLOCK - LONG-TERM OUTCOME OF CHILDREN AND IMMUNOGENETIC STUDY

Objective, To assess the longterm cardiologic and immunologic outcome of children with isolated congenital complete heart block (CCHB) and t heir HLA antigens. Methods, Sixteen children with isolated CCHB were i nvestigated. HLA typing was done using a microcytotoxicity test. Resul ts, Three patients died (18.7%), one in utero (35 weeks), one 2 days a fter birth, and one at 6 years of age. The mean age of the 13 living c hildren is now 18.3 years (range 2-34). Eight (50%) have been permanen tly paced for symptoms. No patient developed clinical symptoms or sero logical abnormalities suggesting immune disease. The A31 antigen was m ore prevalent, but one pair of HLA identical twins was observed, and o nly one had CCHB. Conclusion, Patients with isolated CCHB have signifi cant cardiac mortality, and after a long followup many of them are pac ed to control symptoms, but in our small sample those who survive the perinatal period mostly lead a normal life. The longterm immunological outcome of these children seems good. CCHB is not related a specific HLA pattern in affected children.