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[Double discordance in adults]

Maria Jimenez1

  • 1Clinique Saint-Augustin, 114, avenue d'Ares, 33000 Bordeaux, France. mjimenez@fr.oleane.com

Presse Medicale (Paris, France : 1983)
|April 22, 2011
PubMed

Insights

Double discordance, a complex congenital heart defect, often allows long-term survival, especially in isolated cases. However, associated anomalies significantly impact patient outcomes and necessitate lifelong monitoring.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Congenital Heart Disease

Context:

  • Double discordance (DD) represents a complex congenital heart anomaly, accounting for 0.5-1% of all congenital heart diseases.
  • While isolated forms of DD are compatible with long-term survival, approximately 90% of patients present with additional cardiac abnormalities.
  • These associated anomalies, including ventricular septal defects (VSD), atrioventricular (AV) block, and left ventricular outflow tract obstruction, critically influence the clinical course.

Purpose:

  • To review the natural history and long-term management of double discordance.
  • To highlight the importance of monitoring the systemic right ventricle function and associated anomalies.
  • To emphasize the necessity of lifelong follow-up for rhythm disturbances, right ventricular ejection fraction, and tricuspid regurgitation.

Summary:

  • Double discordance is a rare congenital heart defect with variable outcomes.
  • Long-term survival is achievable, particularly in isolated DD, but frequently complicated by coexisting anomalies.
  • The natural history is primarily determined by the performance of the systemic right ventricle and the presence of other defects.

Impact:

  • This review underscores the critical need for comprehensive, lifelong surveillance in patients with double discordance.
  • Understanding the impact of associated anomalies is crucial for optimizing management strategies.
  • Regular evaluation of cardiac function and rhythm is essential for improving the quality of life and prognosis in this patient population.

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