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ARVC: Diagnostic challenges
Dr Shankar Sadagopan
Southampton University Hospitals
UK
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Learning Objectives
A. Diagnosis using Revised Task Force Criteria
B. Diagnostic modalities outside Task Force Criteria
C. Quiz
D. Differential diagnosis
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Introduction
• Genetics: Mutations in genes that encode constituents of intercalated discs of cardiomyocytes
• Histological hallmark: Cardiomyocyte loss and replacement of fibrous/ fibro-fatty tissues
• Characteristics: Arrhythmias, SCD & Progressive heart failure
• Prevalence: Estimated 1 in 5000 (M:F- 3:1)
• 10% of unexplained SCD in <69 yrs of age
• Median age of onset of symptoms: 29 yrs of age
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Diagnosis using revised TFC
• Integration of data from 1. Imaging techniques 2. Depolarisation abnormalities on ECG 3. Repolarisation abnormalities on ECG 4. Arrhythmias 5. Family history 6. Tissue diagnosis
• Diagnostic groups
– Possible: M(1) OR m(2) – Borderline: M(1)+m(1) OR m(3) – Definite: M(2) OR M(1)+m(2) OR m(4)
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Imaging
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Imaging by Echo
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Imaging Criteria
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Imaging by Angiography
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MRI
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Revised TFC in Children
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Depolarisation abnormalities
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Depolarisation changes: Epsilon
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Depolarisation changes & Epsilon
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Depolarisation changes: SAECG
• Late Potentials> 1 of 3 features
1. Filtered QRS duration fQRS ≥114 ms
2. Low Amplitude Signal duration (duration of terminal QRS), 40 mV: LAS40 ≥ 38 ms
3. Root-mean-square voltage of terminal 40 ms: RMS40≤20 mV
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SAECG: Serial testing
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Depolarisation changes: TAD
Frank I. Marcus et al.
Circulation. 2010
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Repolarisation changes
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Repolarisation changes: TWI • Seen in 32-50% of ARVC
– Sensitivity 47%
– Specificity 96%
• Seen in 1-3% of normal young patients
• Seen in 20-30% of RVOT VT
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Electrical abnormalities
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Arrhythmias
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Arrhythmias
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Family history
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Genetics
• Inherited as Autosomal dominant trait in up to 50% of cases
• Incomplete penetrance
• Variable clinical expression
HRS/EHRA Expert Consensus Statement on the State of Genetic Testing for the Channelopathies and Cardiomyopathies
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Family history & Progression
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Tissue Diagnosis: Myocardial Bx
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Tissue diagnosis
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Useful modalities outside TFC
• Tpe assessment
• Isoproterenol challenge
• Exercise Stress Test
• EPS, Voltage maps for scar assessment
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Repolarisation change: Tpe
(Tpeak-Tend)
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Isoproterenol testing
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ETT in Asymptomatic carriers
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EPS & Voltage maps- abnormal
endocardium
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Quiz-1: Athlete referred for pre-
participation evaluation
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Quiz-2: Healthy Athlete from Africa
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Quiz-3: Olympic screening
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Quiz-4: Athlete referred for
screening
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Quiz-5
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Differential Diagnosis
• BrS overlap
• RVOT VT (Malignant conversion)
• Race
• Athletes
• Congenital abnormalities and Acquired heart diseases
• Early repolarisation
• Sarcoidosis
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BrS overlap
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RVOT VT- Malignant conversion
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Athlete’s Heart
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Summary
• Integration of 6 domains of TFC in diagnosis
• Importance of serial monitoring
• Electrical changes precede structural changes
• Newer modalities outside TFC
• Differential diagnosis
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Thanks for your
attention