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Isolated congenital complete heart block: longterm outcome of children and immunogenetic study
A Brucato1, M Gasparini, G Vignati
1Dipartimento di Cardiologia De Gasperis, Centro Trasfusionale, Ospedale Niguarda, Milano, Italy.
Insights
Congenital complete heart block (CCHB) in children leads to significant cardiac mortality and requires pacing for many survivors. However, long-term immunological outcomes appear favorable, with no specific HLA antigen association found.
Area of Science:
- Pediatric Cardiology
- Immunology
- Genetics
Background:
- Congenital complete heart block (CCHB) is a rare condition affecting fetal and infant cardiac function.
- Understanding the long-term outcomes and potential immunologic associations is crucial for patient management.
Purpose of the Study:
- To evaluate the long-term cardiologic and immunologic outcomes in children diagnosed with isolated CCHB.
- To investigate the association between CCHB and specific Human Leukocyte Antigen (HLA) profiles.
Main Methods:
- A cohort of 16 children with isolated CCHB was studied.
- Human Leukocyte Antigen (HLA) typing was performed using a microcytotoxicity test.
Main Results:
- A mortality rate of 18.7% was observed, including in utero and early childhood deaths.
- Of the 13 surviving children, 50% required permanent pacing for symptom management.
- No patients exhibited clinical or serological signs indicative of immune disease; the A31 antigen was more prevalent, but no specific HLA pattern was identified.
Conclusions:
- Isolated CCHB presents significant cardiac mortality risks and often necessitates long-term pacing.
- Survivors of CCHB, particularly those who overcome perinatal challenges, generally experience a normal life.
- The long-term immunological prognosis for children with CCHB appears positive, and the condition is not linked to a specific HLA pattern.
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.