Isolated congenital complete heart block: longterm outcome of children and immunogenetic study

Abstract

Objective: To assess the longterm cardiologic and immunologic outcome of children with isolated congenital complete heart block (CCHB) and their HLA antigens.

Methods: Sixteen children with isolated CCHB were investigated. HLA typing was done using a microcytotoxicity test.

Results: Three patients died (18.7%), one in utero (35 weeks), one 2 days after birth, and one at 6 years of age. The mean age of the 13 living children is now 18.3 years (range 2-34). Eight (50%) have been permanently paced for symptoms. No patient developed clinical symptoms or serological abnormalities suggesting immune disease. The A31 antigen was more prevalent, but one pair of HLA identical twins was observed, and only one had CCHB.

Conclusion: Patients with isolated CCHB have significant cardiac mortality, and after a long followup many of them are paced 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 to a specific HLA pattern in affected children.