Role of Ecg in Differential Diagnosis of Anderson - Fabry Disease with HCM
Study objective and method
Determine independent ECG predictors of AFD |
Validate an ECG scoring system for differential diagnosis of AFD |
Results
Anderson–Fabry ECG score
Score calculation |
PR interval | <120 ms | 1 |
120- 199 ms | 0 | ||
≥200 ms | 2 | ||
QRS duration | <100 ms | 0 | |
100–119 ms | 1 | ||
120–139 ms | 2 | ||
≥140 ms | 3 | ||
RBBB (complete and incomplete) | Yes | 2 | |
No | 0 | ||
R in aVL ≥11 mV | Yes | 1 | |
No | 0 | ||
Inferior ST-segment depression | Yes | 3 | |
No | 0 |
Algorithm for step-by–step analysis of the ECG for AFD
Conclusion
Independent predictors of AFD diagnosis |
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Short PR interval |
Prolonged QRS duration |
RBBB |
R in aVL ≥1.1 mV |
Inferior ST-segment depression |
A stepwise ECG scoring system |
Good diagnostic performance |
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Discriminates unexplained LVH | Providing clinical and therapeutic implications |
AFD: Anderson–Fabry disease; aVF: Augmented vector foot; aVL: Augmented vector left; ECG: Electrocardiogram; HCM: Hypertrophic cardiomyopathy; LVH: Left ventricular hypertrophy; MS: Millisecond; mV: Millivolt; RBBB: Right bundle branch block.