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
	RBBB (complete and incomplete)	No	0
	R in aVL ≥11 mV	Yes	1
	R in aVL ≥11 mV	No	0
	Inferior ST-segment depression	Yes	3
	Inferior ST-segment depression	No	0

Algorithm for step-by–step analysis of the ECG for AFD

Conclusion

Independent predictors of AFD diagnosis

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

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.