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Implantation of Total Artificial Heart in Congenital Heart Disease
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Congenital Heart Block

Erick Jimenez1, Matthew Ambrose1, Bradley Clark1

  • 1Division of Pediatric Cardiology, Department of Pediatrics, University of Minnesota - Masonic Children's Hospital, 2450 Riverside Avenue S, Minneapolis, MN 55454, USA.

Clinics in Perinatology
|November 13, 2025
PubMed

Insights

Congenital heart block (CHB) is a rare fetal condition affecting the heart's electrical system. Early diagnosis and multidisciplinary care, including new therapies, improve outcomes for affected pregnancies and neonates.

Area of Science:

  • Cardiology
  • Maternal-Fetal Medicine
  • Pediatric Electrophysiology

Background:

  • Congenital heart block (CHB) is a rare fetal arrhythmia caused by conduction system disruptions.
  • Often associated with maternal autoantibodies (e.g., anti-Ro/SSA, anti-La/SSB) or congenital heart defects.
  • Leads to significant fetal morbidity and mortality if untreated.

Purpose of the Study:

  • To provide a comprehensive overview of congenital heart block (CHB).
  • To examine current diagnostic strategies and emerging therapeutic interventions.
  • To highlight the importance of multidisciplinary management for improved fetal and neonatal outcomes.

Main Methods:

  • Review of current literature on CHB pathophysiology, diagnosis, and management.
  • Emphasis on advances in fetal echocardiography for early detection.
  • Discussion of pharmacological (e.g., hydroxychloroquine, IVIG) and interventional (pacemaker implantation) strategies.

Main Results:

  • Fetal echocardiography is crucial for diagnosing CHB and assessing cardiac function.
  • Maternal hydroxychloroquine prophylaxis shows promise in preventing CHB development or progression.
  • Multimodal therapies, including corticosteroids and IVIG, offer options for refractory cases.
  • Pacemaker implantation is a viable option for symptomatic neonates.

Conclusions:

  • Early and accurate diagnosis of CHB is critical for timely intervention.
  • A multidisciplinary approach involving maternal-fetal medicine specialists, pediatric cardiologists, and electrophysiologists is essential.
  • Advances in prenatal and postnatal management offer improved prognoses for CHB-affected infants.

Keywords:
Congenital heart blockFetal arrhythmiaMaternal autoantibodiesNeonatal lupusPacemaker therapy

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