Table 2

Follow-up of vasoactive therapy effect

PatientTherapy at diagnosisWHO-FC at PH diagnosisTherapy adjustmentsObserved follow-up (months)Description outcomeWHO-FC at end follow-upFinal outcome
1S2S↑: 18 months
S↑: 36 months
36Slow progression to WHO-FC 33Alive
2S3S + I: 12 months16Remains at WHO-FC 3 after iloprost therapy3Alive
3B2B + S: 7 months
B + S + T: 24 months
36Slow progression to WHO-FC 3
6-MWT at diagnosis 420 m, at 8 months 459 m, at 28 months 390m
4Death due to heart failure after 36 months
6B3B↑: 48 months48With bosentan no PH after 12 months, after 48 months progressive dyspnoeaNAAlive
7S2Stop S: 8 months24Exacerbation of PM after 8 months, ↑ prednisone and start tacrolimus4Death due to respiratory failure after 24 months
8S3NA0Unknown due to transfer to other hospitalNANA
9A3Diuretics: 2 months12Short hospitalisation after 2 months due to heart failure, after 12 months stable in WHO-FC 33Alive
  • A, ambrisentan; B, bosentan; I, iloprost inhalation; S, sildenafil; T, treprostinil; ↑, increase of dose; WHO-FC, WHO functional class; 6-MWT, 6 min walking test; PM, polymyositis; NA, not available.