MioAtlas
G5: Cardiomyopathies

Arrhythmogenic right ventricular cardiomyopathy

Hereditary genetic cardiomyopathy characterized by progressive fibrofatty replacement of right ventricular free wall myocardium (preferentially in the 'triangle of dysplasia': RVOT, apex, and subtricuspid region).

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Definition

Hereditary genetic cardiomyopathy characterized by progressive fibrofatty replacement of right ventricular free wall myocardium (preferentially in the 'triangle of dysplasia': RVOT, apex, and subtricuspid region), generating regional and global RV structural abnormalities and predisposing to severe ventricular arrhythmias of right ventricular origin and sudden cardiac death [C1] [E3] [G5-14] [G5-15].

Severity criteria

  • Epsilon wave (Major depolarization criterion): Presence of epsilon wave, defined as a distinct low-amplitude, high-frequency signal between the end of the QRS complex and the onset of the T wave in right precordial leads (V1, V2, or V3) [E3] [G5-15].
  • T-wave inversion (Major repolarization criterion): Inverted T waves (T-wave inversion in V1 to V3) in right precordial leads (V1, V2, and V3, or beyond) in individuals over 14 years of age in the absence of complete right bundle branch block [E3] [G5-14].
  • Terminal activation delay (Minor criterion): Terminal activation duration of the S wave (TAD) ≥ 55 ms measured from the nadir of the S wave to the end of the QRS complex in V1–V3 [E3] [G5-14].
  • RV dysfunction and dilation (Major imaging criteria):
    • Right ventricular fractional area change (RV FAC) ≤ 33% [E3] [G5-14].
    • Indexed RV end-diastolic volume (RVEDVi) ≥ 110 mL/m² (in men) or ≥ 100 mL/m² (in women) by cardiac magnetic resonance [E3] [G5-14].
    • Regional akinesia, dyskinesia, or aneurysms of the RV free wall associated with global dilation [E3] [G5-14].
  • Ventricular arrhythmias: Sustained or non-sustained monomorphic ventricular tachycardia with left bundle branch block (LBBB) morphology and superior or inferior axis (RV origin) [E3] [G5-14].

Findings by layer

Hemodynamics

  • Right ventricular fractional area change (right ventricular fractional shortening): ≤ 33% (right ventricular systolic dysfunction) [E3] [G5-14].
  • Left ventricular dimensions and function: Preserved in early stages; pulmonary capillary wedge pressure and LVEF are usually normal (left ventricular ejection fraction > 55%) [C1] [E3].
  • Right atrial pressure: Normal or slightly elevated depending on the degree of dilation and associated tricuspid regurgitation [E3].

Rhythm

  • Normal sinus rhythm as baseline rhythm [E3] [G5-14].
  • Monomorphic ventricular tachycardia: With left bundle branch block morphology and frequent ventricular premature beats originating from right-sided chambers [E3] [G5-14].

ECG

  • Epsilon wave: Low-amplitude notch visible at the end of the QRS complex in leads V1 to V3 caused by delayed transmural activation in islands of infiltrated myocardium [E3] [G5-15].
  • Right precordial T-wave inversion (T-wave inversion in V1 to V3): Negative and asymmetric T waves in V1, V2, and V3 [E3] [G5-14].
  • Prolongation of QRS terminal activation duration (TAD ≥ 55 ms) [E3] [G5-14].

Heart sounds and murmurs

  • Second heart sound (S2): Widely split S2 (due to prolonged systolic ejection of the dilated, dysfunctional RV) [G1-11] [G5-14].
  • Right ventricular gallop: Occasional right ventricular third (S3) or fourth (S4) sound at the lower left sternal border [G1-12] [G1-13].
  • Tricuspid regurgitation murmur: Soft holosystolic murmur in tricuspid area that typically increases with inspiration (Rivero-Carvallo sign) if annular dilation of the RV is present [G1-14] [G5-14].

Arterial pulse

  • Normal carotid and peripheral pulse in sinus rhythm [B4].

Jugular venous pulse

  • Generally normal in early stages; prominent 'v' wave or elevation of mean venous pressure when tricuspid regurgitation and overt right ventricular failure ensue [B3] [G5-14].

Maneuvers

  • Deep inspiration: Augmentation of functional tricuspid regurgitation murmur and right-sided sounds (positive Rivero-Carvallo maneuver) [B1] [G5-14].

Simulator severity grades

Simulator severity grades: Single grade
Baseline rhythm: Normal Sinus Rhythm

Also known as: Arrhythmogenic right ventricular cardiomyopathy · Arrhythmogenic right ventricular dysplasia · Arrhythmogenic cardiomyopathy · ARVD · ARVC

Other pathologies in this group

Sources

  1. [B1] Bedside diagnosis of systolic murmurs . N Engl J Med (1988) . DOI: 10.1056/NEJM198806163182404 PubMed: 2897627
  2. [B3] The jugular venous pressure revisited . Cleve Clin J Med (2013) . DOI: 10.3949/ccjm.80a.13039 PubMed: 24085809
  3. [B4] The Carotid Pulse . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250154
  4. [C1] 2023 ESC Guidelines for the management of cardiomyopathies . Eur Heart J (2023) . DOI: 10.1093/eurheartj/ehad194 PubMed: 37622657
  5. [E3] Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria . Circulation (2010) . DOI: 10.1161/CIRCULATIONAHA.108.840827 PubMed: 20172911
  6. [G1-11] The Second Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250183
  7. [G1-12] The Third Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250184
  8. [G1-13] The Fourth Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250185
  9. [G1-14] Systolic Murmurs . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250186
  10. [G5-14] HRS expert consensus statement on evaluation, risk stratification, and management of arrhythmogenic cardiomyopathy . Heart Rhythm (2019) . DOI: 10.1016/j.hrthm.2019.05.007 PubMed: 31078652
  11. [G5-15] Epsilon waves: Milestones in the discovery and progress . Ann Noninvasive Electrocardiol (2018) . DOI: 10.1111/anec.12571 PubMed: 29978588