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Electrophysiologic Management and Considerations for D-transposition of the Great Arteries After Atrial Baffle Repair

Akash H Patel1, Diana Glovaci1,2, David Donaldson1,2,3

  • 1Department of Medicine, University of California Irvine Medical Center, Orange, CA, USA.

The Journal of Innovations in Cardiac Rhythm Management
|September 3, 2021
PubMed

Insights

This case study details managing sudden cardiac death risk in a young woman with D-transposition of the great arteries after Mustard repair. It emphasizes electrophysiologic assessment for this complex congenital heart condition.

Area of Science:

  • Cardiology
  • Electrophysiology
  • Congenital Heart Disease

Background:

  • D-transposition of the great arteries (dTGA) is a complex congenital heart defect.
  • The Mustard procedure is a palliative surgical repair for dTGA, creating an intra-atrial baffle.
  • Adults with repaired dTGA face unique risks, including sudden cardiac death (SCD).

Observation:

  • A 35-year-old woman with a history of dTGA status post-Mustard atrial switch repair presented for evaluation.
  • The patient underwent comprehensive electrophysiologic assessment.
  • Risk stratification for SCD was a primary focus.

Findings:

  • Electrophysiologic studies are crucial for risk assessment in adults with repaired dTGA.
  • Identifying arrhythmic substrates and risk factors can guide management strategies.
  • This case underscores the importance of tailored electrophysiologic evaluation in this specific patient population.

Implications:

  • Proactive electrophysiologic management can mitigate SCD risk in adults with complex congenital heart disease.
  • Understanding long-term sequelae of Mustard repair is vital for patient care.
  • This case contributes to the knowledge base for managing arrhythmias in adults with D-transposition of the great arteries.

Keywords:
Atrial fibrillationD-transposition of the great arteriescongenital heart diseaseintra-atrial reentrant tachycardiasudden cardiac death

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