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ARVD Dr.M.Esmaeilzadeh.

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Presentation on theme: "ARVD Dr.M.Esmaeilzadeh."— Presentation transcript:

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


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


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