Table 1.
AMCs (n=47) | UMCs (n=35) | P value | |
---|---|---|---|
Females | 35 (74.5) | 15 (48.4) | 0.029 |
Age, yr | |||
At diagnosis | 36.4 ± 14.1 | n/a | n/a |
At present or at death | n/a | 60.5 ± 17.3 | n/a |
NMD+ mutations | 27 | 20 | |
NMD− mutations | 20 | 15 | |
Baseline hemodynamic data at diagnosis, mean | |||
Right atrial pressure, mmHg | 10.3 ± 6.0 | n/a | |
Mean PAP, mmHg | 61.3 ± 12.0 | n/a | |
Cardiac index, L•min•m−2 | 1.90 ± 0.73 | n/a | |
Indexed PVR, U•m2 | 19.3 ± 12.7 | n/a |
Abbreviations: AMC, affected mutation carrier with HPAH; UMC, unaffected mutation carrier; NMD, nonsense mediated decay; PAP, pulmonary artery pressure; CI, cardiac index; PVR, pulmonary vascular resistance; n/a, not available
Data are presented as n (%) or mean ±SD, unless otherwise stated. A chi square test was used for P value calculation.