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Echocardiography plays a role in assessing cardiac health and detecting heart conditions, with various types providing critical insights for diagnosis and treatment.
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Ventricular Angiography: A Forgotten Diagnostic Tool?

Georgiana Pintea Bentea1, Brahim Berdaoui1, Sophie Samyn1

  • 1Department of Cardiology, CHU Brugmann, 1020 Brussels, Belgium.

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|July 13, 2024
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Summary
This summary is machine-generated.

This case study highlights arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosed at age 76, the latest documented presentation. Right ventricular angiography confirmed ARVC, revealing characteristic "pile d'assiettes" imaging.

Keywords:
arrhythmogenic right ventricular cardiomyopathydiagnostic imageright ventricular angiography

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Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Genetics

Background:

  • Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart muscle disease.
  • It typically affects younger individuals, leading to ventricular arrhythmias and heart failure.
  • Late-onset ARVC is rare, posing diagnostic challenges.

Purpose of the Study:

  • To report a unique case of arrhythmogenic right ventricular cardiomyopathy (ARVC) with a very late presentation.
  • To emphasize the diagnostic utility of right ventricular angiography in challenging ARVC cases.
  • To contribute to the understanding of ARVC's varied clinical manifestations.

Main Methods:

  • Clinical presentation of acute decompensated right heart failure and ventricular tachycardia.
  • Diagnostic workup including ECG, echocardiography, coronary angiography, cardiac MRI, signal-averaged ECG, and RV angiography.
  • Characteristic ECG findings (epsilon wave, inverted T waves) and imaging features were analyzed.

Main Results:

  • A 76-year-old male presented with symptoms and ECG findings suggestive of ARVC.
  • Cardiac imaging revealed significant right ventricular dilation, dysfunction, and fibro-fatty replacement.
  • Right ventricular angiography demonstrated the pathognomonic "pile d'assiettes" sign, confirming ARVC.

Conclusions:

  • Arrhythmogenic right ventricular cardiomyopathy (ARVC) can present atypically late in life.
  • Right ventricular angiography remains a crucial tool for diagnosing ARVC, especially in complex or late-onset cases.
  • This case represents the oldest documented age of ARVC presentation, expanding the known spectrum of the disease.