Table 3

 Secondary PH clinical summary

PatientSexAge (years)DiagnosisMean PAP (mm Hg)PVR (units⋅m2)Treatment duration (months)WHO classSix minutes walkWeight (kg)
BaselineChangeBaseline% ChangeBaseline% Change
*Taking epoprostenol at start; †repaired truncus arteriosus with interrupted left pulmonary artery and residual ventricular septal defect (VSD).
AVSD, atrioventricular septal defect; CLD, chronic lung disease; CT, connective tissue; DORV, double outlet right ventricle; E, Eisenmenger’s syndrome; pAVSD, partial atrioventricular septal defect; po, postoperative; SAH, systemic arterial hypertension; TA, truncus arteriosus; TAPVD, total anomalous pulmonary venous drainage; TGA, transposition of the great arteries.
1F1.7AVSD (E)571222III−18.718
2M2.7TGA/Rastelli3736.122IV01055
3F4.2AVSD, po384.916III−12407615.316
4F4.3AVSD, po207III01511
5M5.8TGA, VSD Switch579.412III−1110183.513.46.7
6F10.1AVSD (E)8336.917III08012527.912
7F11.3TA,VSD (E)†3818.711IV−121024.518.519
8M14.2TAPVD, po5313.319IV−13103625.89.26
9M14.3TGA/Senning518.118III−12908259.317
10M15.1DORV (E)7217.311III080127.544.17.5
11*F15.2TGA/Senning5318.416IV−1160187.542.17.7
12F15.7pAVSD, po4913.27III04007.5314.5
13F0.6CLD2IV06.825
14F1.9CLD3210.119III−19.30
15M2.4CLD784912III−11229
16*M3.3Systemic sclerosis + SAH4388III09.6−6.7
17M7.3Diffuse interstitial lung disease + SAH4819.16IV033.57.2
18M8.1Cutis marmorata438.255III−131233.319.922.4
19F12.25CT disorder/pulmonary fibrosis + SAH9IV–132.819.6
20F16HIV8022.16III0340−5.844.44.95