Contemporary characteristics and outcomes of adults with familial dilated cardiomyopathy listed for heart transplantation

Table 1 Baseline characteristics by etiology n (%)

	NICM (n = 8416)	FDCM (n = 677)	P value	ICM (n = 8301)	P value
Age at listing	50.9 ± 12.3	43.9 ± 13.5	< 0.001	58.5 ± 8.1	< 0.001
Male gender	6113 (72.6)	444 (65.6)	< 0.001	7212 (86.9)	< 0.001
Ethnicity			< 0.001		< 0.001
White	4609 (54.8)	444 (65.6)		6411 (77.2)
Black	2776 (33.0)	159 (23.5)		976 (11.8)
Hispanic	705 (8.4)	54 (8.0)		575 (6.9)
Asian	223 (2.6)	14 (2.1)		269 (3.2)
Other or unknown	103 (1.2)	6 (0.9)		70 (0.8)
Initial status			< 0.001		0.956
1A	1918 (22.8)	147 (21.7)		1823 (22.0)
1B	4023 (47.8)	273 (40.3)		3415 (41.1)
2	2227 (26.5)	238 (35.2)		2831 (34.1)
7	248 (2.9)	19 (2.8)		232 (2.8)
Therapies
Inotropes	2947 (35)	233 (34.4)	0.769	2386 (28.7)	0.002
ECMO	52 (0.6)	4 (0.6)	1.0	87 (1.0)	0.320
IABP	395 (4.7)	21 (3.1)	0.056	403 (4.9)	0.038
Mechanical ventilation	141 (1.7)	10 (1.5)	0.875	209 (2.5)	0.118
LVAD	2104 (25.1)	124 (18.4)	< 0.001	2116 (25.6)	< 0.001
BiVAD	153 (1.8)	8 (1.2)	0.288	157 (1.9)	0.233
TAH	50 (0.6)	9 (1.3)	0.039	31 (0.4)	0.002
ICD	6985 (83.5)	562 (83.8)	0.914	6652 (80.9)	0.073
Laboratory values
Creatinine	1.4 ± 1.0	1.3 ± 0.7	0.008	1.4 ± 0.9	< 0.001
Albumin	3.7 ± 0.7	3.8 ± 0.6	0.001	3.7 ± 0.7	0.001
Bilirubin	1.1 ± 1.8	1.1 ± 1.0	0.578	1.0 ± 2.0	0.540
PRA class I	7.1 ± 18.6	7.2 ± 18.5	0.892	5.7 ± 16.2	0.084
PRA class II	4.8 ± 15.7	5.2 ± 16.2	0.645	3.4 ± 12.6	0.012
Hemodynamics
PA systolic pressure (mmHg)	44.3 ± 13.9	42.7 ± 13.2	0.004	44.8 ± 15.2	0.001
PA diastolic pressure (mmHg)	22.1 ± 8.9	21.7 ± 8.7	0.266	20.8 ± 8.6	0.013
PA mean pressure (mmHg)	30.5 ± 10.2	29.7 ± 9.8	0.052	29.8 ± 10.5	0.821
PCWP (mmHg)	20.5 ± 9.0	20.4 ± 8.5	0.735	19.7 ± 8.9	0.081
CO (L/min)	4.3 ± 1.4	4.1 ± 1.3	0.011	4.5 ± 1.3	< 0.001

NICM: Non-ischemic cardiomyopathy; FDCM: Familial dilated cardiomyopathy; ICM: Ischemic cardiomyopathy; UNOS: United network for organ sharing; ECMO: Extracoroporeal membrane oxygenation; IABP: Intraaortic balloon pump; VAD: Ventricular assist device; LVAD: Left ventricular assist device; BiVAD: Biventricular assist device; TAH: Total artificial heart; ICD: Implantable cardioverter-defibrillator; PA: Pulmonary artery; PCWP: Pulmonary capillary wedge pressure; CO: Cardiac output.

Table 2 Wait-list outcomes by etiology

Outcome	FDCM vs NICM	FDCM vs ICM
	HR (95%CI), P value	HR (95%CI), P value
Waitlist mortality	0.720 [0.507-1.023], P = 0.067	0.609 [0.429-0.864], P = 0.005
Delisting due to deterioration	0.499 [0.319-0.781], P = 0.002	0.387 [0.248-0.604], P < 0.001
Waitlist mortality or delisting due to deterioration	0.617 [0.468-0.813], P = 0.001	0.501 [0.381-0.659], P = 0.001
Delisting due to improvement	0.277 [0.131-0.588], P = 0.001	0.347 [0.163-0.735], P = 0.006
Transplant	1.248 [1.135-1.373], P < 0.001	1.183 [1.076-1.302], P = 0.001

NICM: Non-ischemic cardiomyopathy; FDCM: Familial dilated cardiomyopathy.

Table 3 Determinants of wait-list mortality or delisting

	Univariable HR (95%CI)	P value	Multivariable HR (95%CI)	P value
Age at listing		0.082
Gender		0.413
Ethnicity		0.712
UNOS listing status		< 0.001		0.001
1b vs 1a	0.431 [0.231-0.805]		0.606 [0.305-1.204]
2 vs 1a	0.160 [0.073-0.350]		0.176 [0.073-0.424]
7 vs 1a	0.852 [0.252-2.886]		1.326 [0.373-4.707]
Inotropes		0.110
ECMO		0.229
IABP	3.987 [1.575-10.090]	0.004		0.124
Mechanical Ventilation	4.294 [1.333-13.831]	0.015	3.694 [1.022-13.360]	0.046
VAD		0.009		0.519
LVAD vs no VAD	1.084 [0.540-2.179]
BiVAD vs no VAD	7.636 [2.342-24.900]
TAH vs no VAD	1.724 [0.236-12.600]
ICD		0.392
Creatinine	1.275 [1.142-1.422]	< 0.001	1.377 [1.211-1.566]	< 0.001
PASP	1.022 [1.002-1.043]	0.033		0.169
PADP		0.067
PAMP		0.097
PCWP		0.387
CO		0.093
List year		0.282

UNOS: United network for organ sharing; ECMO: Extracoroporeal membrane oxygenation; IABP: Intraaortic balloon pump; VAD: Ventricular assist device; LVAD: Left ventricular assist device; BiVAD: Biventricular assist device; ICD: Implantable cardioverter-defibrillator; PCWP: Pulmonary capillary wedge pressure; CO: Cardiac output.

Table 4 Post-transplantation outcomes n (%)

	NICM	FDCM	P-value	ICM	P value
Stroke	114 (2.3)	6 (1.4)	0.239	147 (3.0)	0.051
Permanent pacemaker	171 (3.4)	16 (3.6)	0.785	164 (3.3)	0.681
Treated for rejection	496 (9.8)	51 (11.4)	0.283	416 (8.4)	0.034
Dialysis	476 (9.5)	43 (9.7)	0.866	507 (10.2)	0.806
LOS (d)	19 ± 22	17.3 ± 13.1	0.105	20.7 ± 25.4	0.006

NICM: Non-ischemic cardiomyopathy; FDCM: Familial dilated cardiomyopathy; ICM: Ischemic cardiomyopathy; LOS: Length of stay.