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ARVD ARVD Dr.M.Esmaeilzadeh. ARVD ARVD Definition Definition ARVD also known as arrhythmogenic RV cardiomyopathy (ARVC), is a primary heart muscle disease.

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Presentation on theme: "ARVD ARVD Dr.M.Esmaeilzadeh. ARVD ARVD Definition Definition ARVD also known as arrhythmogenic RV cardiomyopathy (ARVC), is a primary heart muscle disease."— Presentation transcript:

1 ARVD ARVD Dr.M.Esmaeilzadeh

2 ARVD ARVD Definition 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. 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 ARVD Pathobiology Pathobiology 1-Disontogenetic theory: 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 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. The term dysplasia (which means mal- development) is in agreement with this view. Dr.M.Esmaeilzadeh

4 ARVD ARVD 2-Degenerative theory: 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. 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 ARVD 3-Inflammatory theory: 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. 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. ARVD is difficult to diagnose and usually requires many different cardiac tests. There is no gold standard. There is no gold standard.

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

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

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

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

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13 ARVD Right ventricular parietal block 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. 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 ARVD ARVD late potentials in a 16 year-old child with non-sustained VT (Major criterion) Dr.M.Esmaeilzadeh

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16 ARVD Diagnostic criteria ARVD Diagnostic criteria III. ECG repolarization abnormalities III. ECG repolarization abnormalities Minor Minor Inverted T waves in right precordial leads Inverted T waves in right precordial leads (V2 and V3) in people aged >12 years (V2 and V3) in people aged >12 years and in the absence of RBBB. and in the absence of RBBB. Dr.M.Esmaeilzadeh

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

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

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

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

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

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

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

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

27 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

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

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

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33 ARVD 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

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

37 ARVD ARVD Natural history and progression 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 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. 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. 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 RVOT Tachycardia ARVD 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 (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 84% sensitivity and 100% specificity 84% sensitivity and 100% specificity.Epsilon Wave V1-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; literature is conflicting increased signal intensity of RV free wall; wall motion abnormalities with CINE MRI wall motion abnormalities with CINE MRI Response to Therapy Acute Vagal Maneouvres Adenosine, Beta-blockers Verapamil Verapamil Chronic Beta-blockers or verapamil +/- class one Chronic Beta-blockers or verapamil +/- class one antiarrhythmic drugs antiarrhythmic drugs Sotalol Amiodrone+/- Beta blockers Sotalol Amiodrone+/- Beta blockers.RF Ablation Usually Curative Seldom Curative; may modify substrate to permit AA drugs effective Arrhythmias or different AA drugs effective Arrhythmias or different morphology tend to occur morphology tend to occur Dr.M.Esmaeilzadeh

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

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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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