Table 2.

Associated Clinical Features which are Present in a Minority of Patients with Familial Dilated Cardiomyopathy

Associated Phenotype	Clinical Features	Comment	Associated Gene*
Conduction disease	Sinus arrest AV block Interventricular block	May precede DCM	DES DMD EMD LMNA SCN5A
Supraventricular arrhythmia prior to DCM	Premature atrial contraction Atrial fibrillation	Often with slow ventricular response	EMD LMNA SCN5A
Skeletal Myopathy	Limb Girdle	Proximal muscle weakness	LMNA
	Emery-Dreifuss	Contractures, skeletal myopathy and wasting	EMD, LMNA
	Myotonic Dystrophy	Myotonia, weakness, baldness and cataracts.	ZNF9, DMPK1
	Duchenne/Becker	Progressive X-linked proximal myopathy	DMD
	Myofibrillar Myopathy	Slowly progressive proximal and distal weakness	DES
Hearing loss	Sensorineural hearing loss	Hearing loss typically occurs in 1st and 2nd decade of life	EYA
Palmoplantar keratoderma	Increased thickness of the palms and soles with woolly or excessively curly hair	May precede cardiac involvement	DSP

^*Selected, incomplete list of associated disease genes.

AVB = atrioventricular block.