ARVDARVD

Dr.M.Esmaeilzadeh

ARVDARVD

DefinitionDefinition ARVD also known as arrhythmogenic RV ARVD also known as arrhythmogenic RV

cardiomyopathy (ARVC), is a cardiomyopathy (ARVC), is a primaryprimary heart muscle disease of heart muscle disease of unknown etiologyunknown etiology characterized by a progressive loss of characterized by a progressive loss of myocardium, with a peculiar myocardium, with a peculiar fatty or fatty or fibrofatty replacementfibrofatty replacement, that accounts for , that accounts for the onset of the onset of cardiac electrical instability. cardiac electrical instability.

Dr.M.Esmaeilzadeh

ARVDARVD

PathobiologyPathobiology 1-Disontogenetic theory:1-Disontogenetic theory:

The absence of myocardium is considered The absence of myocardium is considered as a consequence of a as a consequence of a congenital aplasia congenital aplasia or hypoplasia of the RV wallor hypoplasia of the RV wall, leading to a , leading to a parchment like appearance.parchment like appearance.

The term ‘ dysplasia’’ (which means mal-The term ‘ dysplasia’’ (which means mal-development) is in agreement with this view.development) is in agreement with this view.

Dr.M.Esmaeilzadeh

ARVDARVD

2-Degenerative theory:2-Degenerative theory:

The loss of myocardium is a consequence The loss of myocardium is a consequence of of progressive myocytes deathprogressive myocytes death due to due to some metabolic or ultra-structural defect. some metabolic or ultra-structural defect. FamilialFamilial occurrence suggests a genetic occurrence suggests a genetic disease with disease with autosomal dominantautosomal dominant transmission and variable expression and transmission and variable expression and penetrance. The term ‘penetrance. The term ‘’myocardial ’myocardial dystrophydystrophy’’’’ is in agreement with this view. is in agreement with this view.

Dr.M.Esmaeilzadeh

ARVDARVD

3-Inflammatory theory: 3-Inflammatory theory:

The fibrofatty replacement is viewed as a The fibrofatty replacement is viewed as a healing process in the setting of healing process in the setting of chronic chronic myocarditismyocarditis. Thus, an infectious and/or . Thus, an infectious and/or immune myocardial reaction might intervene immune myocardial reaction might intervene in the etiology and pathogenesis of the in the etiology and pathogenesis of the disease.disease.

Dr.M.Esmaeilzadeh

ARVDARVDDiagnosis Diagnosis

ARVD is difficult to diagnose and usually ARVD is difficult to diagnose and usually requires many different cardiac tests.requires many different cardiac tests.

There is no gold standard.There is no gold standard.

ARVD ARVD DiagnosisDiagnosis 2 major criteria2 major criteria 1 major + 2 minor criteria 1 major + 2 minor criteria 4 minor criteria4 minor criteria

Dr.M.Esmaeilzadeh

ARVD ARVD Diagnostic criteria Diagnostic criteria

I. Familial historyI. Familial history  

MajorMajor   Familial disease confirmed at necroscopy or surgery.Familial disease confirmed at necroscopy or surgery.

MinorMinor   Family history of premature SCD (<35 years) due to Family history of premature SCD (<35 years) due to

suspected ARVD.  suspected ARVD.  Family history (clinical diagnosis based on present Family history (clinical diagnosis based on present

criteria). criteria).

Dr.M.Esmaeilzadeh

ARVDARVDDiagnostic criteria Diagnostic criteria

II. ECG depolarization/conduction II. ECG depolarization/conduction abnormalitiesabnormalities  

MajorMajor   Epsilon wavesEpsilon waves Localized prolongation (>110 ms) of the QRS Localized prolongation (>110 ms) of the QRS

complex (parietal block) in right precordial complex (parietal block) in right precordial leads (V1-V3). leads (V1-V3).

  MinorMinor   Late potentials seen on signal averaged Late potentials seen on signal averaged

electrocardiography. electrocardiography.

Dr.M.Esmaeilzadeh

ARVDARVD Epsilon waves,Epsilon waves, (Major criterion) which which

are reproducible small deflections seen are reproducible small deflections seen just beyond the QRS complex in lead V1 just beyond the QRS complex in lead V1 or V2.or V2.

Dr.M.Esmaeilzadeh

ARVDARVD Right ventricular parietal blockRight ventricular parietal block (Major criterion) as as

evidenced by a QRS duration in V1 + V2 + V3  that evidenced by a QRS duration in V1 + V2 + V3  that is longer than that in leads V4, V5, V6 by a ratio of is longer than that in leads V4, V5, V6 by a ratio of 1/2.1/2.

Dr.M.Esmaeilzadeh

ARVDARVDlate potentials in a 16 year-old child with non-sustained VT

(Major criterion) 

Dr.M.Esmaeilzadeh

ARVDARVDDiagnostic criteria Diagnostic criteria

III. ECG repolarizationIII. ECG repolarization abnormalitiesabnormalities    

MinorMinor   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

ARVDARVD Typical inverted T-waves in the right precordial leads up to V4.

(Minor criterion)

Dr.M.Esmaeilzadeh

ARVDARVDDiagnostic criteria Diagnostic criteria

IV. Tissue characteristics of wallsIV. Tissue characteristics of walls

MajorMajor   Fibro fatty replacement of myocardium Fibro fatty replacement of myocardium

on endomyocardial biopsy. on endomyocardial biopsy.

Dr.M.Esmaeilzadeh

ARVDARVD     Diagnostic criteria (from McKenna et al)Diagnostic criteria (from McKenna et al)

   

V. Global and/or regional dysfunction and V. Global and/or regional dysfunction and structural alterationsstructural alterations  

MajorMajor   Severe Severe dilatation and reduction of RV ejection fractiondilatation and reduction of RV ejection fraction with no with no

(or only mild) left ventricular impairment.  (or only mild) left ventricular impairment.  Localized RV Localized RV aneurysms aneurysms (Akinetic or dyskinetic areas with (Akinetic or dyskinetic areas with

diastolic bulging).  diastolic bulging).  Severe Severe segmental dilatationsegmental dilatation of the RV. of the RV.

MinorMinor   Mild global RV dilatation and/or ejection fraction reduction Mild global RV dilatation and/or ejection fraction reduction

with normal left ventricle.  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

ARVD ARVD Diagnostic criteria Diagnostic criteria

VI. ArrhythmiasVI. Arrhythmias    

MinorMinor   Sustained/ non-sustained VT(LBBB type)Sustained/ non-sustained VT(LBBB type)

documented on the electrocardiography, Holter documented on the electrocardiography, Holter monitoring or during exercise testing. monitoring or during exercise testing.

Frequent ventricular extrasystolesFrequent ventricular extrasystoles (> 1,000/24 (> 1,000/24 h on Holter monitoring). h on Holter monitoring). 

Dr.M.Esmaeilzadeh

ARVDARVDA monomorphic ventricular tachycardia with LBBB morphology

(Minor criterion)

Dr.M.Esmaeilzadeh

ARVDARVDAngiography of the RV (LAO view): anterior bulging ofinfundibulum (arrow) in a child with non-sustained VT

  (Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVDAngiography of the RV (lateral view): sub tricuspid bulging (arrow)

(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVDApical 4-ch echocardiogram: Dyskinesia of the RV apex (arrows)

(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVDShort axis nuclear MR

Diffuse bright signal from the RV wall, suggestive for myocardial fatty replacement

(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVDFatty 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

ARVDARVDConventional angiogram of the right ventricle

heavy trabeculations and aneurysmal bulges of the RVOT(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVDAxial T1-weighted black blood spin-echo imageAxial T1-weighted black blood spin-echo image

Extensive transmural fatty replacement of the RV and RVOTExtensive transmural fatty replacement of the RV and RVOT

(Major criterion)(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVD 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 RVOTDiffuse thinning and fatty replacement of the RV and RVOT

(Major criterion)(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVD  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

ARVDARVDCross section of the heart specimen showing diffuse involvement of the Cross section of the heart specimen showing diffuse involvement of the

RV free wall with anterior and sub-tricuspid RV free wall with anterior and sub-tricuspid aneurysmsaneurysms

(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVD Histology of the RV aneurysm: transmural Histology of the RV aneurysm: transmural fibro-fatty replacementfibro-fatty replacement

of the atrophic myocardium accounting for a thin wall of the atrophic myocardium accounting for a thin wall

(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVD Endomyocardial biopsy: extensive fibrous replacement of myocardium

(Major criterion)

Dr.M.Esmaeilzadeh

ARVDARVD Natural history and progression Natural history and progression 1-Early clinically 1-Early clinically ‘’concealed ‘’‘’concealed ‘’ phase with or without phase with or without

minor arrhythmias (SCD may be the first minor arrhythmias (SCD may be the first manifestation, sport restriction is mandatory).manifestation, sport restriction is mandatory).

2-2-’’Overt electrical heart disorder’’’’Overt electrical heart disorder’’,, with severe with severe arrhythmias and impending cardiac arrestarrhythmias and impending cardiac arrest

3-Final stage of 3-Final stage of ‘’ biventricular pump failure’’‘’ biventricular pump failure’’ mimicking dilated cardiomyopathy with mimicking dilated cardiomyopathy with cardiomegaly, CHF, and the risk of cardiomegaly, CHF, and the risk of thromboembolic complication.thromboembolic complication.

Dr.M.Esmaeilzadeh

ARVDARVD Differential diagnosisDifferential diagnosis The major condition which needs to be The major condition which needs to be

differentiated from ARVD is idiopathic ventricular differentiated from ARVD is idiopathic ventricular tachycardia arising from the outflow tract. The tachycardia arising from the outflow tract. The ventricular tachycardia can be exactly the same, ventricular tachycardia can be exactly the same, but there is no structural abnormality of the heart, but there is no structural abnormality of the heart, unlike the situation in ARVD where commonly unlike the situation in ARVD where commonly there is dilation of the ventricle. there is dilation of the ventricle.

Right ventricular outflow tract tachycardia (RVOT) Right ventricular outflow tract tachycardia (RVOT) is more common than ARVD and occurs in is more common than ARVD and occurs in youngyoung, , otherwise otherwise healthy peoplehealthy people. The treatment is either . The treatment is either with medications or with catheter ablation. with medications or with catheter ablation.

Dr.M.Esmaeilzadeh

ARVDARVD RVOT TachycardiaRVOT Tachycardia ARVDARVD..Family History of ArrhythmiaFamily History of Arrhythmiaor Sudden Cardiac Deathor Sudden Cardiac Death No Frequently Yes No Frequently Yes .Arrhythmias .Arrhythmias PVBs, NSVT or SVT PVBs, NSVT or SVT (at rest or with exercise ) Same (at rest or with exercise ) Same .Sudden Cardiac Death.Sudden Cardiac Death Rare 1% per year Rare 1% per year .Frontal Plane QRS .Frontal Plane QRS Positive in leads III , AVF, negative in lead AVL Inferior or Superior Positive in leads III , AVF, negative in lead AVL Inferior or Superior ..T-wave MorphologyT-wave Morphology T wave upright V2-V5 T wave inverted beyond V1 T wave upright V2-V5 T wave inverted beyond V1..Parietal BlockParietal Block QRS duration <110 msec in V1, V2 or V3 QRS duration > 110 msec QRS duration <110 msec in V1, V2 or V3 QRS duration > 110 msec     84% sensitivity and 100% specificity 84% sensitivity and 100% specificity ..Epsilon WaveEpsilon Wave V1-V3V1-V3 Absent Present 30% Absent Present 30%.Signal Averaged ECG.Signal Averaged ECG Normal Usually Abnormal Normal Usually Abnormal .Echocardiogram .Echocardiogram Normal Increased RV size and/or wall motion abnormalities Normal Increased RV size and/or wall motion abnormalities ..RV VentriculogramRV Ventriculogram Usually Normal Usually Abnormal Usually Normal Usually Abnormal .MRI.MRI Usually Normal, but data in 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 TherapyResponse to Therapy Acute Vagal Maneouvres Acute Vagal Maneouvres

Adenosine, Beta-blockers Adenosine, Beta-blockers Verapamil Verapamil Chronic Beta-blockers or verapamil +/- class oneChronic Beta-blockers or verapamil +/- class one antiarrhythmic drugsantiarrhythmic drugs Sotalol Amiodrone+/- Beta blockersSotalol Amiodrone+/- Beta blockers.RF Ablation.RF Ablation Usually Curative Seldom Curative; may modify substrate to permit Usually Curative Seldom Curative; may modify substrate to permit AA drugs effective Arrhythmias or different AA drugs effective Arrhythmias or different morphology tend to occurmorphology tend to occur

Dr.M.Esmaeilzadeh

Uhl's AnomalyUhl's Anomaly

DefinitionDefinition Partial/total absence of the RV myocardiumPartial/total absence of the RV myocardium,,

termed termed parchment heartparchment heart since the parietal since the parietal myocardium is paper thin and translucent since the myocardium is paper thin and translucent since the endocardium is in apposite with the epicardium endocardium is in apposite with the epicardium without intervening muscle.without intervening muscle.

Dr.M.Esmaeilzadeh

Uhl's AnomalyUhl's Anomaly

PresentationPresentation --Cyanosis, dyspnea and Cyanosis, dyspnea and right-sided right-sided

failurefailure, usually in infancy or early , usually in infancy or early childhood.childhood.

-No genetic basis-No genetic basis

Dr.M.Esmaeilzadeh

Uhl's AnomalyUhl's Anomaly

Dr.M.Esmaeilzadeh

Uhl's AnomalyUhl's Anomaly

Dr.M.Esmaeilzadeh

Uhl's AnomalyUhl's Anomaly

Dr.M.Esmaeilzadeh

Uhl's AnomalyUhl's Anomaly

Dr.M.Esmaeilzadeh

Uhl's AnomalyUhl's Anomaly

Dr.M.Esmaeilzadeh