Table 1.

Demographics and clinical characteristics of 19 probands and relatives with pathogenic or likely pathogenic variants in the desmoplakin gene (DSP).

Characteristic	Relatives	Probands	p-Value
	N = 8	N = 11
Age at Diagnosis (years), median (IQR)	35.5 (31.0–48.0)	42.0 (30.0–48.0)	0.53
Sex (%)			1.00
Female	5 (62%)	7 (64%)
Male	3 (38%)	4 (36%)
Race/Ethnicity (%)			1.00
Hispanic Latinx/White	1 (12%)	1 (9%)
White	7 (88%)	9 (82%)
Unknown	0 (0%)	1 (9%)
Symptoms at Initial Assessment (%)
NYHA Class			0.11
I	5 (62%)	6 (55%)
II	1 (12%)	5 (45%)
III	2 (25%)	0 (0%)
Dyspnea	2 (25%)	1 (9%)	0.55
Chest Pain	2 (25%)	1 (9%)	0.55
Palpitations	5 (62%)	3 (27%)	0.18
Presyncope	1 (12%)	2 (18%)	1.00
Syncope	1 (12%)	0 (0%)	0.42
Edema	0 (0%)	0 (0%)	1.00
Family History of SCD (%)	—	5 (45%)
Family History of DCM (%)	—	5 (45%)

Abbreviations: NYHA = New York Heart Association; SCD = sudden cardiac death; DCM = dilated cardiomyopathy.