Imaging of Arrhythmogenic Cardiomyopathies

Arrhythmogenic cardiomyopathy is a genetic heart disease where normal heart muscle is replaced by fatty and fibrous tissue, increasing the risk of dangerous heart rhythms and death. Newer diagnostic criteria include advanced cardiac MRI findings, improving detection. Treatment includes implanted defibrillators, procedures to control abnormal rhythms, and avoiding intense sports.

Course ID: Q00811 Category:
Modality:

3.50

Satisfaction Guarantee

$37.00

Targeted CE per ARRT’s Discipline, Category, and Subcategory classification for enrollments starting after February 10, 2026:
[Note: Discipline-specific Targeted CE credits may be less than the total Category A credits approved for this course.]

Cardiac-Interventional Radiography: 1.00
Procedures: 1.00
Diagnostic and Electrophysiology Procedures: 0.75
Interventional Procedures: 0.25

Nuclear Medicine Technology: 0.50
Procedures: 0.50
Cardiac Procedures: 0.50

Registered Radiologist Assistant: 1.00
Procedures: 1.00
Thoracic Section: 1.00

Outline

  1. Introduction
  2. Genetic Basis of ACM
    1. Desmosome Structure
    2. Genetic Abnormalities of ACM
    3. Genotype-Phenotype Correlation
  3. Clinicopathologic and Imaging Manifestations of ACM
  4. Task Force Criteria for Diagnosis
    1. 1994 Task Force Criteria
    2. 2010 Task Force Criteria
  5. Challenges in Diagnosing ARVC
    1. Regional Wall Motion Abnormalities
    2. Anatomic Variants
    3. Characterization of Myocardial Tissue
    4. Limitations of Task Force Criteria
      1. Quantitative Threshold for Criteria
      2. Tissue Characterization
      3. Beyond ARVC (Diagnosing ALVC and Biventricular ACM
  6. Padua Criteria for ACM
  7. Diagnosis of ARVC
    1. Morphofunctional Abnormalities
    2. Structural Myocardial Abnormalities
  8. Diagnosis of ALVC
    1. Morphofunctional Abnormalities
    2. Structural Myocardial Abnormalities
  9. Diagnosis of Biventricular ACM
  10. Effect of Padua Criteria on Diagnosis of ACM
  11. Future Directions
  12. Management of ACM
  13. Differential Diagnosis
    1. Congenital Heart Diseases Causing RV Dilatation
    2. Sarcoidosis
    3. Myocarditis
    4. Dilated Cardiomyopathy
  14. Conclusion

Objectives

Upon completion of this course, students will:

  1. list the conditions included in the 2019 expert consensus statement definition of ACM
  2. identify the original name for ACM when it was first described
  3. list the tissue types that can be affected by desmosome abnormalities
  4. list the families of proteins that comprise desmosomes
  5. list the protein subtypes within the cadherin family of proteins
  6. identify the year when ACM was first described as a heritable disease
  7. describe the genetic variation most associated with the ARVC phenotype of ACM
  8. describe the genetic variation most associated with the ALVC phenotype of ACM
  9. list the right ventricular regions that comprise the triangle of dysplasia
  10. list the clinical and imaging findings that manifest in the hot phase of ACM
  11. describe the common initial manifestation of ACM in young patients and athletes
  12. describe the ACM patient populations at risk of dying during physical exertion
  13. list the imaging modalities that were part of the major imaging criteria from the 1994 task force
  14. identify the preferred imaging modality in the 2010 task force criteria
  15. describe the MRI technique used to quantify RV dilatation and function
  16. describe the imaging plane used for postprocessing contouring of the RV at cardiac MRI
  17. identify the imaging finding required by the task force criteria to diagnose ARVC
  18. describe the imaging plane used to evaluate wall motion abnormalities in the RV infundibulum
  19. describe the RV region where the accordion sign is usually seen
  20. list the RV anatomic variants that can be incorrectly interpreted as a wall motion abnormality
  21. identify the patient population that often exhibits physiologic fat infiltration of the RV wall
  22. identify the diagnostic criteria that include LGE at cardiac MRI
  23. describe the diagnostic test required by the task force criteria to identify cardiac fibrosis
  24. describe when the new Padua diagnostic criteria were published
  25. identify the biggest accomplishment of the Padua criteria over both task force criteria
  26. list the imaging modalities used to evaluate morphofunctional abnormalities in the Padua criteria
  27. list the imaging modalities used to evaluate structural abnormalities in the Padua criteria
  28. describe why endomyocardial biopsy is no longer routinely performed for ACM diagnosis
  29. list the categories of ARVC diagnosis in the Padua criteria
  30. identify the myocardial layers where LGE is typically seen in patients with ALVC
  31. list the possible clinical outcomes for ACM patients with higher native T1 and T2 values
  32. list the congenital heart diseases that can mimic ARVC
  33. describe the imaging tests routinely used to assess cardiac involvement of sarcoidosis
  34. compare the LGE patterns seen with sarcoidosis and ALVC
  35. identify the Padua criterion required to differentiate sarcoidosis from ALVC
  36. describe the diagnostic criteria used to assess myocarditis
  37. compare the clinical and imaging features of myocarditis and hot-phase ACM
  38. distinguish the clinical and imaging features of ALVC and dilated cardiomyopathy