Table 3

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

BaselineFollow-upP value
Cardiac MRI: left ventricle
 LVEDV (mL)171±36169±380.57
 LVESV (mL)54±2052±200.41
 SV (mL)117±22117±230.98
 EF (%)69±770±60.55
 LVM (g)152±54147±550.06
 Native septal T1 (ms)1060±471049±420.06
 ECV fraction (%)30.0±4.529.5±4.00.06
 Total myocardial volume (mL)145±52140±530.05
 ECM volume (mL)44±1842±170.04
 Cellular volume (mL)101±3699±370.11
 Global longitudinal strain (%)−18.3±3.4−19.4±3.40.03
 PCr/ATP ratio1.27±0.441.4±0.390.46
Cardiac MRI: left atrium
 LAESV (mL)75.8±43.665.8±40.90.04
 LAEDV (mL)124.4±51.3114.6±47.30.18
 Total EF (%)41.3±8.345.5±12.20.07
 LA expansion index0.73±0.220.94±0.540.09
 Total strain (%)20.0±3.921.5±5.00.04
 Peak systolic strain rate (−1)0.75±130.83±0.190.11
Echocardiographic data
 E/A ratio1.11±0.671.10±0.500.84
 Mean S’ velocity (cm/s)6.9±2.58.1±1.90.03
 Mean E’ velocity (cm/s)6.8±3.47.0±2.50.71
 Mean A’ velocity (cm/s)7.4±3.18.4±2.70.11
 Mitral deceleration time (ms)295±79265±470.11
  • ECV fraction was calculated as ECV = (1haematocrit) 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.