CC BY 4.0 · AJP Rep 2025; 15(02): e73-e78
DOI: 10.1055/a-2616-5273
Case Reports

Management and Outcomes of Congenital Atrioventricular Block in Neonates: A 6-Year Experience at a Tunisian Tertiary Center

1   Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
2   University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
,
Sameh Hajji
2   University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
,
Khaoula Samaali
2   University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
3   Department of Obstetrics and Gynecology B, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
,
Hajer Chourou
1   Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
2   University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
,
Wafa B. Ammar
1   Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
2   University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
,
Neila B. Aba
1   Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
2   University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
,
Khaled Neji
2   University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
3   Department of Obstetrics and Gynecology B, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
,
Samia Kacem
1   Neonatal Intensive Care Unit, Maternity and Neonatology Center of Tunis, University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
2   University of Tunis El Manar Faculty of Medicine of Tunis, Tunisia
› Author Affiliations

Funding None.

Abstract

Background

Congenital atrioventricular block (CAVB) is a rare and serious condition often associated with maternal autoimmune diseases or congenital heart defects. This study aims to evaluate the clinical presentation, management, and outcomes of neonates diagnosed with CAVB.

Methods

We conducted a retrospective study from January 2018 to December 2024, including eight neonates diagnosed with CAVB. Data on demographics, clinical features, treatment, and follow-up were analyzed.

Results

All cases were prenatally diagnosed between 20 and 25 weeks of gestation, with positive anti-SSA/SSB antibodies in five cases. Management included cesarean delivery, Holter ECG monitoring, and pacemaker implantation for four patients. One case resulted in intrauterine fetal death, and two patients died in the neonatal period. Survivors had successful pacemaker implantation with an average follow-up of 18 months.

Conclusion

Early prenatal detection and timely management of CAVB are crucial. Pacemaker implantation significantly improves survival, though challenges such as resource limitations and the lack of long-term follow-up data remain. Future studies should address these gaps to optimize care for CAVB patients.

Authors' Contributions

This work was performed in collaboration with all the authors. Material preparation, data collection, and analysis were performed by R.B.A., H.C., and W.B.A. The first draft of the manuscript was written by R.B.A. and S.H. N.B.A. and K.S. were involved in collecting and reviewing the bibliography and proposing recommendations for writing the article. S.K. and K.N. commented on previous versions of the manuscript. All authors have read and approved the final manuscript.


Ethical Approval

This study was performed in line with the principles of the Declaration of Helsinki. Approval was obtained from the Ethical Committee of Maternity and Neonatology Center of Tunis (November 2024). All patients' parents received explanations of the purposes, procedures, risks, and benefits of this study.


Consent to Participate

Informed consent was obtained from all the participants' parents included in the study.




Publication History

Received: 16 January 2025

Accepted: 12 March 2025

Article published online:
09 June 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Melim C, Pimenta J, Areias JC. Congenital atrioventricular heart block: From diagnosis to treatment. Rev Port Cardiol 2022; 41 (03) 231-240
  • 2 Thongnak C, Limprasert P, Tangviriyapaiboon D. et al. Exome sequencing identifies compound heterozygous mutations in SCN5A associated with congenital complete heart block in the Thai population. Dis Markers 2016; 2016: 3684965
  • 3 Baruteau AE, Pass RH, Thambo JB. et al. Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management. Eur J Pediatr 2016; 175 (09) 1235-1248
  • 4 Luo JL, Zhu JF. Complete congenital atrioventricular block found by ultrasound in pregnancy. Ann Emerg Med 2022; 79 (06) 568-577
  • 5 Manolis AA, Manolis TA, Melita H, Manolis AS. Congenital heart block: pace earlier (childhood) than later (adulthood). Trends Cardiovasc Med 2020; 30 (05) 275-286
  • 6 Gozar L, Marginean C, Fagarasan A. et al. Congenital complete atrioventricular block from literature to clinical approach - a case series and literature review. Med Ultrason 2021; 23 (02) 188-193
  • 7 Deshpande S, Shenthar J, Khanra D. et al. Outcomes in congenital and childhood complete atrioventricular block: a meta-analysis. J Cardiovasc Electrophysiol 2022; 33 (03) 493-501
  • 8 The importance of the level of maternal anti-Ro/SSA antibodies as a prognostic marker of the development of cardiac neonatal lupus erythematosus a prospective study of 186 antibody-exposed fetuses and infants - PubMed. Accessed October 22, 2024 at: https://pubmed-ncbi-nlm-nih-gov.accesdistant.sorbonne-universite.fr/20538173/
  • 9 Sonesson SE, Ambrosi A, Wahren-Herlenius M. Benefits of fetal echocardiographic surveillance in pregnancies at risk of congenital heart block: single-center study of 212 anti-Ro52-positive pregnancies. Ultrasound Obstet Gynecol 2019; 54 (01) 87-95
  • 10 Warnes CA. Transposition of the great arteries. Circulation 2006; 114 (24) 2699-2709
  • 11 The clinical spectrum of autoimmune congenital heart block - PubMed. Accessed October 24, 2024 at: https://pubmed-ncbi-nlm-nih-gov.accesdistant.sorbonne-universite.fr/25800217/
  • 12 Song MK, Kim NY, Bae EJ. et al. Long-term follow-up of epicardial pacing and left ventricular dysfunction in children with congenital heart block. Ann Thorac Surg 2020; 109 (06) 1913-1920
  • 13 Van den Berg NWE, Slieker MG, van Beynum IM. et al. Fluorinated steroids do not improve outcome of isolated atrioventricular block. Int J Cardiol 2016; 225: 167-171
  • 14 Ciardulli A, D'Antonio F, Magro-Malosso ER. et al. Maternal steroid therapy for fetuses with immune-mediated complete atrioventricular block: a systematic review and meta-analysis. J Matern Fetal Neonatal Med 2019; 32 (11) 1884-1892
  • 15 Donofrio MT, Moon-Grady AJ, Hornberger LK. et al; American Heart Association Adults With Congenital Heart Disease Joint Committee of the Council on Cardiovascular Disease in the Young and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and Council on Cardiovascular and Stroke Nursing. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation 2014; 129 (21) 2183-2242
  • 16 Komori A, Ayusawa M, Kato M, Nakamura T, Takahashi S. Congenital complete atrioventricular block with pulmonary hypertension. Pediatr Int 2017; 59 (10) 1095-1096
  • 17 Maltret A, Morel N, Levy M. et al. Pulmonary hypertension associated with congenital heart block and neonatal lupus syndrome: a series of four cases. Lupus 2021; 30 (02) 307-314
  • 18 Doti PI, Escoda O, Cesar-Díaz S. et al. Congenital heart block related to maternal autoantibodies: descriptive analysis of a series of 18 cases from a single center. Clin Rheumatol 2016; 35 (02) 351-356
  • 19 Minimally Invasive Epicardial Pacemaker Implantation in Neonates with Congenital Heart Block - PubMed. Accessed January 13, 2025 at: https://pubmed-ncbi-nlm-nih-gov.accesdistant.sorbonne-universite.fr/28876373/