Table 3.

Cardiac MRI Functional and Fibrosis data

	Controls	ALMS		ALMS
			ECV < ULN	ECV > ULN	ECV > ULN
				NO LGE	LGE
Number n	26	26	12	5	9
Mean global ECV	0.25 ± 0.01	0.30 ± 0.05*	0.25 ± 0.02	0.31 ± 0.03a	0.34 ± 0.05b
Global pre contrast T1 (ms)	970 ± 11	949 ± 35	934 ± 34	952 ± 33	972 ± 35
Global post contrast T1 (ms)	522 ± 58	465 ± 61*	472 ± 54	440 ± 47	437 ± 43
Diffuse LGE n	0	9	0	0	9
LVEDV (ml)	133 ± 29	105 ± 23**	107 ± 31	100 ± 13	108 ± 25
LVEDV index (ml/m2)	69 ± 12	57 ± 11**	57 ± 13	56 ± 11	58 ± 13
LVESV(ml)	43 ± 13	38 ± 15	37 ± 15	32 ± 8	43 ± 19
LVESV index (ml/m2)	22 ± 6	20 ± 8	20 ± 7	18 ± 5	23 ± 10
LVEF (%)	68 ± 5	66 ± 8	67 ± 7	69 ± 5	62 ± 11
LV mass (g)	115 ± 31	104 ± 27	106 ± 27	87 ± 13	112 ± 34
LV mass index (g/m2)	60 ± 14	56 ± 12	56 ± 10	48 ± 8	60 ± 16
LV mass/ Height2.7 (g/m2.7)	25 ± 6	29 ± 7*	28 ± 6	24 ± 6	32 ± 9
LA volume (ml/m2)	33 ± 8	35 ± 15	29 ± 8	40 ± 14	39 ± 15
MAPSE (mm)	17 ± 2	12 ± 2*	13 ± 2	13 ± 3	11 ± 3
Global long SS (%)	14.7 ± 2.3	11.2 ± 2.6**	12.9 ± 1.0	10.9 ± 2.0a	9.6 ± 1.9b
Global long SSR (s−1)	0.83 ± 0.15	0.57 ± 0.11**	0.63 ± 0.16	0.53 ± 0.11a	0.52. ± 0.15b
Global long ESR (s−1)	0.62 ± 0.15	0.49 ± 0.18*	0.54 ± 0.15	0.44 ± 0.19	0.41 ± 0.19

EDV end-diastolic volume, ESV end-systolic volume, EF ejection fraction, LA left atrium, LGE late gadolinium enhancement, long; longitudinal, LV left ventricle, MAPSE mitral annular plane systolic excursion, RV right ventricle, TAPSE tricuspid annular plane systolic excursion, SS systolic strain, SR systolic strain rate, ESR early diastolic strain rate, ULN ECV above the upper limit (0.279) observed in matched control

^a p < 0.05, Bonferroni post-hoc tests for differences ECV above upper limit of normal controls vs. increased ECV no LGE

^b p <0.05, Bonferroni post-hoc tests for differences ECV above upper limit of normal controls vs. increased ECV with LGE

ANOVA * p <0.05, ** <0.01, ALMS vs. controls