Table 3.

Clinical Manifestations of ARVC in 48 Subjects at High Risk Who Received an ICD and 58 Matched Control Subjects From 11 Families With ARVC Linked to 3p25

	ICD Cohort (n = 48)			Control Cohort (n = 58)			Chi-Square 1 df, p Value
	No. With Manifestation	No. With Documentation	%	No. With Manifestation	No. With Documentation	%
Antiarrhythmic medications*
Class 1	10	44	23	11	42	26	ns
Sotalol	13	44	33	6	31	13	ns
Amiodarone	11	44	27	5	30	17	ns
Symptoms
Palpitations	35	44	79	15	32	47	0.003
Presyncope	29	44	66	12	32	37	0.01
Syncope	15	44	34	5	33	15	ns
Chest pain	14	44	32	18	35	51	ns
Heart failure	6	47	13	10	31	32	0.04
Abnormal clinical tests†
12-lead ECG‡	33	48	69	32	33	97	0.002
SAECG§	17	31	55	5	12	42	ns
Echocardiogram¶	24	42	57	20	25	80	ns
Holter monitor||	33	42	79	20	24	83	ns
MRI**	4	23	23	0	4	0	ns
Catheterization††	0	18	0	3	15	20	0.05
Autopsy‡‡	0	0	0	16	17	94

^*Class 1 medications: mexilitine, propafenone, procainamide, quinidine, and phenytoin. The larger denominator for the class 1 medication takes into account those with no records because they never visited a doctor.

^†Abnormalities on clinical tests.

^‡Electrocardiogram (ECG) T-wave inversion, septal Q waves, poor R-wave progression in V₁, V₂, and V₃, or presence of PVCs (17, 56).

^§Signal-averaged ECG (SAECG): >115 ms QRS duration (17).

^¶Echocardiogram: >112% Left ventricular dilatation (54), any right ventricular dilatation, focal or global hypokinesis, or akinesis of any cardiac wall (17, 57–59).

^||Holter monitor, >1000 PVCs in 24 h, documented runs of VT of >4 beats (17,60).

^**Magnetic resonance imaging (MRI): evidence of fatty/fibrous infiltrate or ventricular thinning (61–63).

^††Cardiac catheterization: any critical coronary artery disease.

^‡‡Autopsy: evidence of fat and fiber in the myocardium (17,64).

ARVC = arrhythmogenic right ventricular cardiomyopathy; ICD = implantable cardioverter-defibrillator.