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Paediatric Rheumatology

 

Plasmapheresis, intravenous immunoglobulins and bethametasone - a combined protocol to treat autoimmune congenital heart block: a prospective cohort study

1, 2, 3, 4, 5, 6, 7, 8, 9

 

  1. Rheumatology Unit, Department of Medicine, University of Padua, Italy. amelia.ruffatti@unipd.it
  2. Paediatric Cardiology Unit, Department of Women's and Children's Health, University of Padua, Italy.
  3. Rheumatology Unit, Department of Medicine, University of Padua, Italy.
  4. Rheumatology Unit, Department of Medicine, University of Padua, Italy.
  5. Rheumatology Unit, Department of Medicine, University of Padua, Italy.
  6. Rheumatology Unit, Department of Medicine, University of Padua, Italy.
  7. Apheresis Unit, Blood Transfusion Service, University Hospital of Padua, Italy.
  8. Cardiology Unit, Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Italy.
  9. Paediatric Cardiology Unit, Department of Women's and Children's Health, University of Padua, Italy.

CER9085
2016 Vol.34, N°4
PI 0706, PF 0713
Paediatric Rheumatology

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PMID: 27385463 [PubMed]

Received: 31/10/2015
Accepted : 08/02/2016
In Press: 22/06/2016
Published: 14/07/2016

Abstract

OBJECTIVES:
At the moment there are no standard guidelines for the treatment of autoimmune congenital heart block (CHB). We set out to carry out a prospective cohort study to evaluate the benefits, limits, and safety of a combined therapy protocol to treat antibody-related CHB.
METHODS:
Twelve consecutive pregnant patients positive to anti-SSA/Ro ± anti-SSB/La antibodies in whom CHB was detected were prospectively evaluated from 2009 to 2014. The treatment protocol consisted of: weekly plasmapheresis, fortnightly intravenous immunoglobulins (IVIG), and daily 4 mg betamethasone from CHB detection until delivery; IVIG was administered to the neonates soon after birth.
RESULTS:
At the time CHB was detected, six of the foetuses presented atrioventricular blocks of 2nd degree type and six of 3rd degree type. Two of the foetuses with a 2nd degree block reverted to a 1st degree block and one to a normal atrioventricular conduction. The condition was stable throughout the pregnancy in the other three cases of 2nd degree block. All six 3rd degree blocks were stable during pregnancy and confirmed at birth. After a mean of 37.6 months ± 19.6 SD post-birth, the infants with 1st, normal sinus rhythm, and 2nd degree blocks at birth were all found to be stable. During the follow-up (29 months ± 19.8 SD), pacemakers were implanted in three of the six infants with 3rd degree blocks.
CONCLUSIONS:
This combined therapy seems to be effective and safe in treating 2nd degree CHB, while its efficacy in treating 3rd degree CHB remains to be established.

Rheumatology Article