Table 2

Univariate survival analysis

Survivalp ValueFreedom from death or transplantationP Value
Unadjusted HR (95% CI)Unadjusted HR (95% CI)
Demographic and historic
 Prevalent group0.83 (0.27 to 2.50)0.730.45 (0.18 to 1.16)0.10
 Age at diagnosis0.92 (0.81 to 1.05)0.210.95 (0.87 to 1.05)0.33
 Female gender8.97 (1.18 to 68.32)0.031.80 (0.71 to 4.59)0.22
 Weight z-score0.65 (0.47 to 0.89)<0.010.73 (0.57 to 0.95)0.02
 Height z-score0.73 (0.54 to 0.99)0.040.78 (0.61 to 1.02)0.07
 Syncope0.65 (0.18 to 2.34)0.510.79 (0.29 to 2.15)0.65
 WHO2.35 (1.04 to 5.30)0.042.79 (1.41 to 5.50)<0.01
Electrocardiographic:
 P pulmonale0.71 (0.25 to 2.05)0.531.23 (0.54 to 3.00)0.57
 QRS-duration0.99 (0.95 to 1.03)0.561.00 (0.97 to 1.03)0.84
 RV strain1.63 (0.56 to 4.70)0.371.83 (0.78 to 4.30)0.16
 RVH0.91 (0.20 to 4.10)0.901.00 (0.30 to 3.40)1.00
Echocardiographic
 ASD2.57 (0.90 to 7.33)0.082.34 (1.02 to 5.39)0.05
 Max TR velocity1.68 (0.90 to 3.15)0.111.82 (1.10 to 3.02)0.02
 RV dilatation score1.70 (0.87 to 3.31)0.121.73 (1.01 to 2.96)0.04
 RV function score1.10 (0.56 to 2.05)0.841.29 (0.76 to 2.19)0.34
Haemodynamic
 RAP1.02 (0.85 to 1.23)0.841.07 (0.93 to 1.24)0.32
 PAP1.00 (0.98 to 1.03)0.691.01 (0.99 to 1.03)0.32
 SAP0.97 (0.91 to 1.02)0.250.96 (0.92 to 1.01)0.12
 CI0.92 (0.50 to 1.66)0.770.67 (0.44 to 1.08)0.10
 PVRI1.01 (0.97 to 1.06)0.631.04 (1.01 to 1.07)0.03
Acute vasodilator response
 PAP1.01 (0.98 to 1.05)0.401.02 (1.00 to 1.05)0.05
 PVRI1.01 (0.94 to 1.08)0.871.06 (1.01 to 1.10)0.01
 ARNo deaths0.12*No events0.05*
  • Boldface highlights the stastically significant p values throughout tables 1 & 2.

  • * Log-rank test for equality of survival functions.

  • AR, acute responder; ASD, inter-atrial communication; CI, cardiac index; mPAP, mean pulmonary artery pressure; mSAP, mean systemic arterial pressure; PVRI, pulmonary vascular resistance index; RAP, mean right atrial pressure.