Table 1.

Clinical and cardiovascular magnetic resonance findings in healthy controls and Fabry patients without LVH

	Healthy controls (n = 76)	Fabry disease without LVH		P-value normal T1 vs. healthy controls	P-value normal T1 vs. low T1
		Normal T1 (n = 40)	Low T1 (n = 32)
Age (years)	49 ± 15	40 ± 13	43 ± 11	0.002	0.332
Male, n (%)	38 (50)	5 (13)	8 (25)	<0.001	0.222
SBP (mmHg)	122 ± 13	110 ± 11	115 ± 11	<0.001	0.155
DBP (mmHg)	76 ± 9	71 ± 8	75 ± 5	0.010	0.082
BSA (m2)	1.8 ± 0.2	1.8 ± 0.2	1.8 ± 0.2	0.448	0.753
Cardiac variant, n (%)	NA	13 (33)	10 (31)		1.000
ERT/OCT, n (%)	NA	13 (33)	12 (38)		0.804
hs-TnT (ng/L)	NA	1 (1–5)	3 (1–6)		0.693
NT-proBNP (pmol/L)	NA	8 (1–14)	6 (1–12)		0.630
CMR
LV EDVI (mL/m2)	72 ± 11	74 ± 11	72 ± 12	0.437	0.564
LVEF (%)	67 ± 4	70 ± 7	73 ± 6	0.018	0.097
LVMI (g/m2)	65 ± 13	59 ± 10	67 ± 14	0.008	0.011
MWT (mm)	9 (7–10)	8 (7–9)	10 (9–11)	0.527	0.001
Septal T1 (ms)	1029 ± 38	1000 ± 28	913 ± 35	<0.001	<0.001
BIFL T2 (ms)	48 ± 2	50 ± 4	46 ± 2	0.027	0.021
Global ECV	24 ± 3	26 ± 2	25 ± 2	0.029	0.243
LGE, n (%)	0	5/37 (14)	8/31 (26)	0.003	0.230
LV LGE (%)a	0	0.3 ± 1.1	0.7 ± 1.4	0.004	0.156
GLS (%)	−20.3 ± 2.3	−18.3 ± 2.1	−18.7 ± 2.5	<0.001	0.457
Stress MBF (mL/g per min)	3.0 ± 0.8	2.5 ± 0.7	2.5 ± 0.5	0.028	0.961

BIFL, basal inferolateral wall; BNP, brain natriuretic peptide; BSA, body surface area; CMR, cardiovascular magnetic resonance; DBP, diastolic blood pressure; ECV, extracellular volume fraction; EDVI, end-diastolic volume index; ERT, enzyme replacement therapy; GLS, global longitudinal strain; hs-TnT, high-sensitivity troponin T; LGE, late gadolinium enhancement; LVEF, left ventricular ejection fraction; LVH, left ventricular hypertrophy; LVMI, left ventricular mass index; MBF, myocardial blood flow; MWT, maximum wall thickness; NA, not available/not applicable; OCT, oral chaperone therapy; SBP, systolic blood pressure.

^aNon-normally distributed variable.