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
Dr.M.Esmaeilzadeh

9. 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

10. ARVD Diagnostic 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

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

13. 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

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

16. ARVD Diagnostic criteria
III. ECG repolarization abnormalities  
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

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

18. ARVD Diagnostic criteria
IV. Tissue characteristics of walls
Major 
Fibro fatty replacement of myocardium on endomyocardial biopsy.
Dr.M.Esmaeilzadeh

19. ARVD Diagnostic 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

20. 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

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

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

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

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

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

27. 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

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

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

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

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

33. 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

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

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

37. 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

38. 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

39. ARVD Dr.M.Esmaeilzadeh or Sudden Cardiac Death No Frequently Yes
RVOT Tachycardia ARVD
.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 % per year
.Frontal Plane QRS Positive in leads III , AVF, negative in lead AVL Inferior or Superior
.T-wave Morphology T wave upright V2-V T wave inverted beyond V1
.Parietal Block QRS duration <110 msec in V1, V2 or V QRS duration > 110 msec
  % sensitivity and 100% specificity
.Epsilon Wave V1-V 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

40. 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

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

43. Uhl's Anomaly
Dr.M.Esmaeilzadeh

44. Uhl's Anomaly
Dr.M.Esmaeilzadeh

45. Uhl's Anomaly
Dr.M.Esmaeilzadeh

46. Uhl's Anomaly
Dr.M.Esmaeilzadeh

47. Uhl's Anomaly
Dr.M.Esmaeilzadeh