Table 2– Participant characteristics: item reduction phase
Patients n226
Females157 (70)
Age years55.6±14
Aetiology of PH
 Group 1: PAH
  Idiopathic89 (40)
  Congenital heart disease50 (22)
  Connective tissue disease43 (19)
  Heritable3 (1)
  Portal hypertension1 (0.5)
 Group 2: PH left heart disease1 (0.5)
 Group 3: PH lung1 (0.5)
 Group 4: CTEPH36 (16)
 Group 5: neurofibromatosis and sarcoidosis2 (1)
PH therapy
 Monotherapy125 (55)
 Dual therapy78 (35)
 Triple therapy11 (5)
 Not recorded12 (5)
 Inhaled iloprost20 (9)
i.v. iloprost8 (4)
i.v. treprostinil5 (2)
i.v. epoprostenol4 (2)
Continuous or as needed oxygen use87 (38)
6MWD m#336±130
Employment status
 Full-time work25 (11)
 Part-time work31 (14)
 Student2 (1)
 Unemployed/retired168 (74)
WHO functional class
 I3 (1)
 II73 (32)
 III115 (51)
 IV34 (15)
MLHFQ modified for PH47.8±25
Hospital Anxiety and Depression scale
Dyspnoea-12 questionnaire
  • Data are presented as n (%) or mean±sd, unless otherwise stated. PH: pulmonary hypertension; PAH: pulmonary arterial hypertension; CTEPH: chronic thromboembolic PH; 6MWD: 6-min walk distance; WHO: World Health Organization; MLHFQ: Minnesota Living with Heart Failure Questionnaire. #: n=98, patients attending the Sheffield Pulmonary Vascular Disease Unit, Sheffield, UK (n=66) underwent exercise testing with the incremental shuttle walking test and did not perform the 6MWD.