Table 3.

Cardiac MRI and echographic data at baseline and after 6 months of treatment with trientine

	Baseline	Follow-up	P value
Cardiac MRI: left ventricle
LVEDV (mL)	171±36	169±38	0.57
LVESV (mL)	54±20	52±20	0.41
SV (mL)	117±22	117±23	0.98
EF (%)	69±7	70±6	0.55
LVM (g)	152±54	147±55	0.06
Native septal T1 (ms)	1060±47	1049±42	0.06
ECV fraction (%)	30.0±4.5	29.5±4.0	0.06
Total myocardial volume (mL)	145±52	140±53	0.05
ECM volume (mL)	44±18	42±17	0.04
Cellular volume (mL)	101±36	99±37	0.11
Global longitudinal strain (%)	−18.3±3.4	−19.4±3.4	0.03
PCr/ATP ratio	1.27±0.44	1.4±0.39	0.46
Cardiac MRI: left atrium
LAESV (mL)	75.8±43.6	65.8±40.9	0.04
LAEDV (mL)	124.4±51.3	114.6±47.3	0.18
Total EF (%)	41.3±8.3	45.5±12.2	0.07
LA expansion index	0.73±0.22	0.94±0.54	0.09
Total strain (%)	20.0±3.9	21.5±5.0	0.04
Peak systolic strain rate (−1)	0.75±13	0.83±0.19	0.11
Echocardiographic data
E/A ratio	1.11±0.67	1.10±0.50	0.84
Mean S’ velocity (cm/s)	6.9±2.5	8.1±1.9	0.03
Mean E’ velocity (cm/s)	6.8±3.4	7.0±2.5	0.71
Mean A’ velocity (cm/s)	7.4±3.1	8.4±2.7	0.11
Mitral deceleration time (ms)	295±79	265±47	0.11

ECV fraction was calculated as ECV = (1−haematocrit) x ƛ, where ƛ is the partition coefficient. Total myocardial volume was LV mass / 1.05. The total extracellular volume (ECM) was calculated with the formula ECM=ECV x LVM/1.05, while the cellular volume was calculated as cellular volume (mL)=total myocardial volume − ECM volume.

An expanded version of this table is available in the online supplemental tables S6, S7.

ECM, extracellular matrix; ECV, extracellular volume; EF, ejection fraction; LAEDV, left atrial end diastolic volume; LAEF, left atrial ejection fraction; LVEDV, left vendricular end diastolic volume; LVESV, left ventricular end systolic volume; LVM, left ventricular mass; PCr/ATP, myocardial phosphocreatine/adenosine triphosphate; SV, stroke volume.