Table 3

RV Geometry and Basal Deformation

Controls
(n = 84)
Type IType IIType III
Mutation Carriers
(n = 12)
Optimal Simulations
Activation Delay
Mutation Carriers
(n = 35)
Optimal Simulations
Activation Delay
Mutation Carriers
(n = 37)
Optimal Simulations
Activation Delay
p Value
None30 ms60 msNone30 ms60 msNone30 ms60 msAll GroupsMutation Carriers
CMR
 RV-EDV, ml181 ± 36169 ± 55181183188208 ± 86197200206247 ± 85§217219222<0.0020.039
 RVEF, %54 ± 455 ± 854535248 ± 1349494736 ± 12§454444<0.001<0.001
 Stroke volume, ml97 ± 1988 ± 239795 ± 179785 ± 19970.0680.154
 Heart rate, beats/min59 ± 868 ± 195963 ± 115961 ± 13590.1520.374
Echocardiography
 Onset of shortening, ms52 (25–72)60 (38–76)6292114119 (99–141)§118124138218 (128–293)§130132142<0.001<0.001
 Systolic peak strain, %−24.7 ± 4.8−23.9 ± 5.3−21.1−22.6−23.9−16.4 ± 3.5§−17.2−17.8−16.7−5.3 ± 5.3§−6.1−6.5−6.1<0.001<0.001
 Post-systolic index, %2 (0–4)1 (0–3)00115 (8–21)§10142544 (22–78)§354252<0.001<0.001

Values are mean ± SD, n, or median (IQR).

Abbreviations as in Table 1.

  • p values are for differences among mutation carriers and controls or among mutation carriers only.

  • CMR data were available in a subgroup of 35 control subjects.

  • RV-EDV and RVEF of the type I simulation without activation delay have been fitted to the average values measured in the controls.

  • § p < 0.05 versus age-and sex-matched controls (Bonferroni correction).

  • Stroke volume and heart rate in all type I, type II, and type III simulations were set to the average values measured in the controls.