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ARVD

ARVD. Dr.M.Esmaeilzadeh. ARV D. Definition

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ARVD

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  1. ARVD Dr.M.Esmaeilzadeh

  2. ARVD Definition ARVD also known as arrhythmogenic RV cardiomyopathy (ARVC), is a primary heart muscle disease of unknown etiology characterized by a progressive loss of myocardium, with a peculiar fatty or fibrofatty replacement, that accounts for the onset of cardiac electrical instability. Dr.M.Esmaeilzadeh

  3. ARVD Pathobiology 1-Disontogenetic theory: The absence of myocardium is considered as a consequence of a congenital aplasia or hypoplasia of the RV wall, leading to a parchment like appearance. The term ‘ dysplasia’’ (which means mal-development) is in agreement with this view. Dr.M.Esmaeilzadeh

  4. ARVD 2-Degenerative theory: The loss of myocardium is a consequence of progressive myocytes death due to some metabolic or ultra-structural defect. Familial occurrence suggests a genetic disease with autosomal dominant transmission and variable expression and penetrance. The term ‘’myocardial dystrophy’’ is in agreement with this view. Dr.M.Esmaeilzadeh

  5. ARVD 3-Inflammatory theory: The fibrofatty replacement is viewed as a healing process in the setting of chronic myocarditis. Thus, an infectious and/or immune myocardial reaction might intervene in the etiology and pathogenesis of the disease. Dr.M.Esmaeilzadeh

  6. ARVD Diagnosis • ARVD is difficult to diagnose and usually requires many different cardiac tests. • There is no gold standard.

  7. ARVD Diagnosis • 2 major criteria • 1 major + 2 minor criteria • 4 minor criteria Dr.M.Esmaeilzadeh

  8. ARVD Diagnostic criteria I. Familial history Major • Familial disease confirmed at necroscopy or surgery. Minor • Family history of premature SCD (<35 years) due to suspected ARVD.  • Family history (clinical diagnosis based on present criteria). Dr.M.Esmaeilzadeh

  9. ARVDDiagnostic criteria II. ECG depolarization/conduction abnormalities Major • Epsilon waves • Localized prolongation (>110 ms) of the QRS complex (parietal block) in right precordial leads (V1-V3). Minor • Late potentials seen on signal averaged electrocardiography. Dr.M.Esmaeilzadeh

  10. ARVD • Epsilon waves,(Major criterion) which are reproducible small deflections seen just beyond the QRS complex in lead V1 or V2. Dr.M.Esmaeilzadeh

  11. ARVD • Right ventricular parietal block(Major criterion) as evidenced by a QRS duration in V1 + V2 + V3  that is longer than that in leads V4, V5, V6 by a ratio of 1/2. Dr.M.Esmaeilzadeh

  12. ARVD late potentials in a 16 year-old child with non-sustained VT (Major criterion) Dr.M.Esmaeilzadeh

  13. ARVDDiagnostic criteria III. ECG repolarizationabnormalities Minor • Inverted T waves in right precordial leads (V2 and V3) in people aged >12 years and in the absence of RBBB. Dr.M.Esmaeilzadeh

  14. ARVD Typical inverted T-waves in the right precordial leads up to V4. (Minor criterion) Dr.M.Esmaeilzadeh

  15. ARVDDiagnostic criteria IV. Tissue characteristics of walls Major • Fibro fatty replacement of myocardium on endomyocardial biopsy. Dr.M.Esmaeilzadeh

  16. ARVDDiagnostic criteria (from McKenna et al) V. Global and/or regional dysfunction and structural alterations Major • Severe dilatation and reduction of RV ejection fraction with no (or only mild) left ventricular impairment.  • Localized RV aneurysms (Akinetic or dyskinetic areas with diastolic bulging).  • Severe segmental dilatation of the RV. Minor • Mild global RV dilatation and/or ejection fraction reduction with normal left ventricle.  • Mild segmental dilatation of the RV.  • Regional RV hypokinesis. Dr.M.Esmaeilzadeh

  17. ARVD Diagnostic criteria VI. Arrhythmias Minor • Sustained/ non-sustained VT(LBBB type) documented on the electrocardiography, Holter monitoring or during exercise testing. • Frequent ventricular extrasystoles (> 1,000/24 h on Holter monitoring).  Dr.M.Esmaeilzadeh

  18. ARVD A monomorphic ventricular tachycardia with LBBB morphology (Minor criterion) Dr.M.Esmaeilzadeh

  19. ARVD Angiography of the RV (LAO view): anterior bulging of infundibulum (arrow) in a child with non-sustained VT (Major criterion) Dr.M.Esmaeilzadeh

  20. ARVD Angiography of the RV (lateral view): sub tricuspid bulging (arrow) (Major criterion) Dr.M.Esmaeilzadeh

  21. ARVD Apical 4-ch echocardiogram: Dyskinesia of the RV apex (arrows) (Major criterion) Dr.M.Esmaeilzadeh

  22. ARVD Short axis nuclear MR Diffuse bright signal from the RV wall, suggestive for myocardial fatty replacement (Major criterion) Dr.M.Esmaeilzadeh

  23. ARVD Fatty form of ARVD (Major criterion) An intermediate-weighted fast spin echo image in the axial plane (left) shows the thickening and replacement of the right ventricular anterior wall by fatty tissue. The same sequence with fat suppression (right) shows the loss of signal in the right ventricular anterior wall, confirming the fatty nature of these changes. Dr.M.Esmaeilzadeh

  24. ARVD Conventional angiogram of the right ventricle heavy trabeculations and aneurysmal bulges of the RVOT (Major criterion) Dr.M.Esmaeilzadeh

  25. ARVD Axial T1-weighted black blood spin-echo image Extensive transmural fatty replacement of the RV and RVOT (Major criterion) Dr.M.Esmaeilzadeh

  26. ARVD Axial T1-weighted black blood spin-echo image Diffuse thinning and fatty replacement of the RV and RVOT (Major criterion) Dr.M.Esmaeilzadeh

  27. ARVD  Axial balanced cine fast-field-echo images Dilated right ventricle (RV) (Minor criterion) Dr.M.Esmaeilzadeh

  28. ARVD Cross section of the heart specimen showing diffuse involvement of the RV free wall with anterior and sub-tricuspid aneurysms (Major criterion) Dr.M.Esmaeilzadeh

  29. ARVD Histology of the RV aneurysm: transmural fibro-fatty replacement of the atrophic myocardium accounting for a thin wall (Major criterion) Dr.M.Esmaeilzadeh

  30. ARVD Endomyocardial biopsy: extensive fibrous replacement of myocardium (Major criterion) Dr.M.Esmaeilzadeh

  31. ARVD Natural history and progression 1-Early clinically ‘’concealed ‘’ phase with or without minor arrhythmias (SCD may be the first manifestation, sport restriction is mandatory). 2-’’Overt electrical heart disorder’’, with severe arrhythmias and impending cardiac arrest 3-Final stage of ‘’ biventricular pump failure’’ mimicking dilated cardiomyopathy with cardiomegaly, CHF, and the risk of thromboembolic complication. Dr.M.Esmaeilzadeh

  32. ARVD Differential diagnosis • The major condition which needs to be differentiated from ARVD is idiopathic ventricular tachycardia arising from the outflow tract. The ventricular tachycardia can be exactly the same, but there is no structural abnormality of the heart, unlike the situation in ARVD where commonly there is dilation of the ventricle. • Right ventricular outflow tract tachycardia (RVOT) is more common than ARVD and occurs in young, otherwise healthy people. The treatment is either with medications or with catheter ablation. Dr.M.Esmaeilzadeh

  33. ARVD RVOT TachycardiaARVD .Family History of Arrhythmia or Sudden Cardiac Death No Frequently Yes .Arrhythmias PVBs, NSVT or SVT (at rest or with exercise ) Same .Sudden Cardiac Death Rare 1% per year .Frontal Plane QRS Positive in leads III , AVF, negative in lead AVL Inferior or Superior .T-wave Morphology T wave upright V2-V5 T wave inverted beyond V1 .Parietal Block QRS duration <110 msec in V1, V2 or V3 QRS duration > 110 msec 84% sensitivity and 100% specificity .Epsilon WaveV1-V3 Absent Present 30% .Signal Averaged ECG Normal Usually Abnormal .Echocardiogram Normal Increased RV size and/or wall motion abnormalities .RV Ventriculogram Usually Normal Usually Abnormal .MRI Usually Normal, but data in literature is conflicting increased signal intensity of RV free wall; wall motion abnormalities with CINE MRI Response to Therapy Acute Vagal Maneouvres Adenosine, Beta-blockers Verapamil Chronic Beta-blockers or verapamil +/- class one antiarrhythmic drugs Sotalol Amiodrone+/- Beta blockers .RF Ablation Usually Curative Seldom Curative; may modify substrate to permit AA drugs effective Arrhythmias or different morphology tend to occur Dr.M.Esmaeilzadeh

  34. Uhl's Anomaly Definition Partial/total absence of the RV myocardium, termed parchment heart since the parietal myocardium is paper thin and translucent since the endocardium is in apposite with the epicardium without intervening muscle. Dr.M.Esmaeilzadeh

  35. Uhl's Anomaly Presentation -Cyanosis, dyspnea and right-sided failure, usually in infancy or early childhood. -No genetic basis Dr.M.Esmaeilzadeh

  36. Uhl's Anomaly Dr.M.Esmaeilzadeh

  37. Uhl's Anomaly Dr.M.Esmaeilzadeh

  38. Uhl's Anomaly Dr.M.Esmaeilzadeh

  39. Uhl's Anomaly Dr.M.Esmaeilzadeh

  40. Uhl's Anomaly Dr.M.Esmaeilzadeh

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