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Aortic dilatation and aortopathy in congenital heart diseases

Keyhan Sayadpour Zanjani1, Koichiro Niwa

  • 1Department of Pediatric Cardiology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. sayadpour@tums.ac.ir

Journal of Cardiology
|November 27, 2012
PubMed

Insights

Congenital heart disease survivors face aortic dilatation, a complex condition termed "aortopathy." This review covers its history, pathophysiology, clinical features, and management strategies for improved patient outcomes.

Area of Science:

  • Cardiology
  • Vascular Biology
  • Congenital Heart Disease Research

Background:

  • Longer survival rates in congenital heart disease (CHD) patients highlight the significance of late complications.
  • Aortic dilatation is a critical late complication in both repaired and unrepaired CHD, potentially leading to severe outcomes like aneurysm, dissection, and rupture.
  • This aortic dilatation represents a complex pathophysiological abnormality, termed 'aortopathy,' involving the aortic root, regurgitation, and ventricular dysfunction.

Purpose of the Study:

  • To provide a comprehensive review of aortic dilatation and aortopathy in congenital heart disease.
  • To elucidate the history, pathophysiology, and clinical manifestations of this complex lesion.
  • To summarize current evaluation and management strategies for aortopathy in CHD patients.

Main Methods:

  • Literature review focusing on aortic dilatation and aortopathy in congenital heart disease.
  • Synthesis of information regarding the history, pathophysiology, clinical features, evaluation, and management.
  • Analysis of the complex interplay between anatomical and pathophysiological abnormalities.

Main Results:

  • Aortic dilatation in CHD is a complex entity, 'aortopathy,' extending beyond simple anatomical changes.
  • The pathophysiology involves aortic regurgitation and ventricular dysfunction, with mechanisms only partially understood.
  • This condition requires careful evaluation and management due to its progressive nature and potential for severe complications.

Conclusions:

  • Aortopathy is an emerging concept in congenital heart disease, crucial for understanding late complications.
  • Further research is needed to fully elucidate the pathophysiology of aortopathy.
  • Effective evaluation and management strategies are essential for improving long-term outcomes in CHD survivors with aortopathy.

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