Table 3.

Baseline clinical characteristics of FLNC truncating mutations carriers divided according to study outcomes.

	D/HT/LVAD			Non-arrhythmic death/HT/LVAD			SCD/MVA
	Outcome Yes	Outcome No	p-value	Outcome Yes	Outcome No	p-value	Outcome Yes	Outcome No	p-value
Age (years)	45±18	42±14	0.485	43±20	42±14	0.943	45±11	42±16	0.424
Male %	53	53	0.976	46	54	0.594	56	62	0.687
Caucasian %	95	100	0.061	92	100	0.018	100	98	0.540
Proband (%)	79	35	0.001	77	39	0.011	65	37	0.021
Variant mapping %			0.689			0.610			0.458
ABD	5	6		0	7		4	7
ROD1	42	49		46	47		44	48
ROD2	53	41		54	42		43	43
Z-disk	21	17	0.659	15	18	0.816	17	18	0.970
Dimerization	0	4		0	4		9	2
Family history cardiomyopathy %	63	86	0.023	54	86	0.006	74	84	0.297
Family history SCD %	32	58	0.046	31	56	0.100	52	52	0.963
Phenotype %			0.120			0.367			0.185
DCM	63	46		69	46		48	50
ARVC	0	5		0	4		9	1
ALVC	37	21		31	24		35	21
Biventricular	0	1		0	1		0	2
Minor phenotype	0	14		0	12		9	11
Unaffected	0	14		0	12		0	15
HR (bpm)	74±23	71±20	0.668	74±26	71±20	0.718	70±17	72±22	0.615
AF %	32	14	0.076	32	15	0.186	30	13	0.065
LBBB %	22	5	0.017	25	6	0.026	4	10	0.407
NYHA 3–4 %	42	10	0.002	46	13	0.004	13	20	0.479
NYHA 1 %	37	77	0.001	23	76	<0.001	74	66	0.466
Negative anterior T waves %	32	19	0.220	23	21	0.875	26	20	0.523
PVCs/24h	3987±8473	3054±4899	0.704	652±364	3476±5673	0.331	4642±6958	2773±4979	0.371
Positive Late Potentials %	5	9	0.593	8	8	0.938	4	10	0.427
NSVT %	75	41	0.038	67	45	0.230	82	34	0.001
Echo LVEF (%)	30±12	46±15	<0.001	27±12	45±15	<0.001	40±14	43±16	0.360
Echo LVEF <50 %	100	55	0.001	100	58	0.005	73	61	0.328
Echo LVEF ≦35 %	65	22	0.001	67	25	0.004	36	29	0.513
Echo LVEDD (mm)	67±6	54±9	<0.001	67±5	55±9	<0.001	59±11	56±9	0.279
Echo MWT (mm)	9±2	9±2	0.966	9±2	9±2	0.973	10±1	9±2	0.170
Echo LVH (%)	8	11	0.863	10	11	0.929	16	9	0.358
Echo RVFAC (%)	34±13	39±10	0.231	35±14	38±10	0.504	34±9	39±11	0.114
Echo RVFAC<35 %	62	29	0.063	67	30	0.072	42	32	0.520
Echo RV WMA %	20	6	0.277	33	6	0.086	15	5	0.229
Echo MR %	69	39	0.044	78	39	0.030	50	42	0.556
CMR LGE % **	50	59	0.729	33	60	0.367	63	57	0.782

D/HT/LVAD=death heart transplant/left ventricular assist device, non-SCD/HT/LVAD=non sudden cardiac death/heart transplant/left ventricular assist device, SCD/MVA=sudden cardiac death/major ventricular arrhythmias, ABD=active binding domain, SCD=sudden cardiac death, DCM=dilated cardiomyopathy, ARVC=arrhythmogenic right ventricular cardiomyopathy, ALVC=arrhythmogenic left-dominant cardiomyopathy, CPK=creatine phosphokinase, HR=heart rate, AF=atrial fibrillation, LBBB=left bundle branch block, NYHA=New York heart association, PVC=premature ventricular complex, NSVT=non-sustained ventricular tachycardia, LVEF=left ventricular ejection fraction, LVEDD=left ventricular end-diastolic volume, MWT=maximum wall thickness, LVH=left ventricular hypertrophy, RVFAC=right ventricle fractional area change, WMA=wall motion abnormalities, MR=mitral regurgitation, CMR=cardiac magnetic resonance imaging, LGE=late gadolinium enhancement.Values are reported as mean±standard deviation, median and interquartile range or percentage as appropriate.

^**among the 43 pts with available CMR.