Table 1.

Differential diagnosis of Uhl's anomaly and ARVD^5,12,15

Features	Uhl's anomaly	ARVD
Morphology	RV myocardial absence	Fibrofatty replacement of RV myocardium
Distribution	Generalized	Localized
Age of presentation	Childhood	Adolescence and adulthood
Gender	Indifferent	More common in men (3:1)
Family history	No	Usual
Usual mode of presentation	Heart failure	Arrhythmias, syncope, or sudden death
Risk for sudden death	Low	High
Electrocardiography	Uncharacteristic	Localized QRS enlargement (>110 msec) in right precordial leads (V1–V3) and epsilon waves