Introduction
Methods
Study population
Original Task Force Criteria | Revised Task Force Criteria |
---|---|
A | |
I. Global or regional dysfunction and structural alterations; Imaging | |
Major | Major |
-Severe dilatation and reduction of RV ejection fraction with no LV impairment -Localized RV aneurysms -Severe segmental dilatation of the RV | By 2D echo: Regional RV akinesia, dyskinesia or aneurysm and 1 of the following (end diastole and corrected for body size (Parasternal long axis /BSA)): - Parasternal long axis RVOT > 32 mm Parasternal long axis /BSA > 19 mm/m2 - Parasternal short axis RVOT > 36 mm Parasternal short axis /BSA > 21 mm/m2 -Fractional area change < 33% By CMR: Regional RV akinesia or dyskinesia or dyssynchrounous RV contraction and 1 of the following: -Ratio of RV EDV to BSA > 110 ml/m2 (male) or > 100 ml/m2 (female) -RV ejection fraction < 40% |
Minor | Minor |
-Mild global RV dilatation and/or ejection fraction reduction with normal LV -Mild segmental dilatation of the RV -Regional RV hypokinesis | By 2D echo: Regional RV akinesia or dyskinesia and 1 of the following (end diastole and corrected for body size (Parasternal long axis /BSA)) -Parasternal long axis RVOT > 29 to < 32 mm Parasternal long axis /BSA > 16 to < 19 mm/m2 -Parasternal short axis RVOT > 32 to 36 mm Parasternal short axis /BSA > 18 to < 21 mm/m2 -RV Fractional area change > 33% to < 40% By MRI: Regional RV akinesia or dyskinesia or dyssynchrounous RV contraction and 1 of the following: -Ratio of RV EDV to BSA > 100 to < 110 ml/m2 (male) or > 90 to < 100 ml/m2 (female) -RV ejection fraction > 40% to < 45% |
B | |
V. Arrhythmias | |
Major | Major |
-Non-sustained or sustained ventricular tachycardia of left bundle-branch morphology with superior axis (negative or indeterminate QRS in leads II, III, and aVF and positive in lead aVL) | |
Minor | Minor |
-Left bundle-branch block-type ventricular tachycardia (sustained and non-sustained) -Frequent ventricular extra-systoles (1000 per 24 h) | -Non-sustained or sustained ventricular tachycardia of RV outflow configuration, left bundle-branch block morphology with inferior axis (positive QRS in leads II, III, and aVF and negative in lead aVL) or of unknown axis - > 500 ventricular extra-systoles per 24 h |
C | |
VI. Family history | |
Major | Major |
-Familial disease confirmed at necropsy or surgery | -ARVC confirmed in a first-degree relative who meets current Task Force criteria -ARVC confirmed pathologically at autopsy or surgery in a first-degree relative -Identification of a pathogenic mutation categorized as associated or probably associated with ARVC in the patient |
Minor | Minor |
-Family history of premature sudden death (35 years of age) due to suspected ARVC -Familial history (clinical diagnosis based on present criteria) | -History of ARVC in a first-degree relative in whom it is not possible or practical to determine whether the family member meets current Task Force criteria -Premature sudden death (35 years of age) due to suspected ARVC in a first-degree relative -ARVC confirmed pathologically or by current Task Force Criteria in second-degree relative |
CMR protocol
Volumetric volume and function assessment Utilising cine CMR
CMR flow calculation
Image analysis
Statistical analysis
Results
Demographics
First Degree Relative with ARVC | First Degree Relative with premature death | Ventricular Tachycardia | Ventricular Extra-systoles | P value Family History vs Arrhythmia Group | |
---|---|---|---|---|---|
n (%) | 16 (33.3%) | 8 (16.7%) | 13 (27.1%) | 11 (22.9%) | |
Age, years | 41.1 +/− 18.9 | 40.3 +/− 19.3 | 49.3 +/−13.1 | 55.4 +/− 9.2 | 0.015 |
Sex, Female, n (%) | 11 (68.8%) | 4 (50%) | 6 (46.1%) | 6 (54.5%) | 0.561 |
Treatment, n (%) | |||||
Beta Blockers | 0 | 0 | 2 (15.4%) | 2 (18.2%) | 0.109 |
Calcium Channel Blockers | 0 | 0 | 1 (7.7%) | 0 | 1.000 |
Electrical Ablation, n (%) | 0 | 0 | 3 (23.1%) | 0 | 0.234 |
Pathogenic mutation associated with ARVC, n (%) | |||||
-PKP2 | 4 (25%) | 0 | 0 | 0 | 0.109 |
Major Criteria from Another Category, n (%) | 16 (100%) | 0 | 13 (100%) | 0 | 0.556 |
Minor Criteria from Another Category, n (%) | 0 | 8 (100%) | 0 | 11 (100%) | 0.556 |
Index CMR | |||||
RV Dilatation, n (%) | 4 (25%) | 4 (50%) | 6 (46.2%) | 4 (36.6%) | 0.7661 |
RV Dyskinesia, n (%) | 12 (75%) | 4 (50%) | 7 (53.8%) | 7 (63.4%) | 0.7661 |
2010 Major Imaging, n (%) | 1 (6.3%) | 2 (25%) | 1 (7.7%) | 2 (18.2%) | 1.000 |
2010 Minor Imaging, n (%) | 15 (93.7%) | 6 (75%) | 12 (92.3%) | 9 (81.8%) | 1.000 |
CMR imaging results
First Degree Relative with ARVC N = 16 | First Degree Relative with premature death N = 8 | Ventricular Tachycardia N = 13 | Ventricular Extra-systoles N = 11 | P value Family History vs Arrhythmia Group | |
---|---|---|---|---|---|
Serial CMR | |||||
Change in RVEDVI, ml | −4.4 +/− 7.2 | −6.8 +/− 6.8 | −12.6 +/− 17.1 | −11.2 +/− 8.7 | 0.099 |
Change in RVESVI, ml | −3.4 +/− 7.9 | −4.2+/− 3.9 | −8.3 +/− 13.3 | −7.2 +/− 5.2 | 0.268 |
Change in RVEF, ml | + 1.3 +/− 7.2 | + 3.1+/− 5.7 | − 4.5 +/− 11.2 | −3.5 +/− 4.5 | 0.438 |
2010 CMR Major Criteria, n (%) | 1 (6.3%) | 1 (12.5%) | 0 | 0 | 0.489 |
2010 CMR Minor Criteria, n (%) | 0 | 0 | 0 | 0 | N/A |