Congenital complete heart block (CCHB), cardiac Purkinje cells antibodies (CPCA) and HLA typing: Long term follow-up

M. Gasparini, A. Brucato, S. Riccobono, M. Lunati, D. Massari, G. Vignati, G. Ferraro, C. Bortolon, G. Gadaleta

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Thirty-eight children affected by isolated congenital complete heart block (CCHB) were studied, with a mean follow up of 14.5 years. Nineteen cases were detected after the first year of life (1-10 years) (Late CCHB), and 19 cases were detected within the first year of life (9 neonatal cases (Early CCHB). Clinical, ECG electrophysiological (15 patients) data and the prevalence of permanent pacing were analyzed blindly from immunological results (HLA typing and cardiac Purkinje cells antibodies (CPCA)), and viceversa. The aim of the study was to verify the correlation between CCHB, permanent pacing, CPCA, and HLA typing during such a long follow up. PM indications were: syncope (9 pts), faintness (5 pts), low cardiac output (2 pts) and severe rest bradycardia with effort dyspnea and/or effort ventricular tachyarrhythmias (4 pts). Results. No patient showed overt major immunological disease during a mean follow up of 14.5 years. Late CCHB: 8 suprahisian CHB; permanent pacing in 10 pts (mean age at implantation ± SE: 18.7 ± 1.7 yrs) (PM implantation 13.8 ± 2.0 yrs after CCHB detection); 4 pts were CPCA positive (22%). Early CCHB: 1 infrashisian and 6 suprahisian CHB, permanent pacing in 10 pts (mean age at implantation: 6.9 ± 1.9 yrs; p <0.0005 vs Late CCHB) (PM implantation 6.9 ± 1.9 yrs after CCHB detection; p <0.02 vs Late CCHB); 6 pts were CPCA positive (33%) (p <0.05 vs normal controls). 7 out of 9 neonatal detected CCHB required permament pacing at the end of the follow up (78%). 29 pts were tested for HLA: DQ2 prevalence in paced pts (2/17) was significantly reduced as compared to normal controls (78/200) (p <0.05). Conclusions: 1) After a mean follow up of 14.5 yrs 20/38 (53%) CCHB pts are permanently paced. 2) 78% of neonatal CCHB are permanently paced (1 paced pt died). 3) No pt developed immunopathological disease. 4) The DQ2 antigen was significantly reduced in placed CCHB. 5) The prevalence of CPCA in our series was significantly higher than in healthy control subjects. Since no CPCA positivity was observed in post-infarction and post-fulgoration CHB, this datum supports the hypothesis of prenatal immunological damage to the conduction system in CCHB.

Original languageEnglish
Pages (from-to)969-974
Number of pages6
JournalNew Trends in Arrhythmias
Issue number4
Publication statusPublished - 1991

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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