Table 1– Overview of clinical, functional and haemodynamic features
Year of assessment
19881998200320072009–2010
NYHAIIIIIIIIII
FVC % pred6561657071
FEV1 % pred6544404540
FEV1/VC %8152495044
RV % pred51150139133171
DL,CO % pred6916<577
KCO % pred921951510
Pa,O2 at rest# kPa8.77.89.4+9.1+9.5+
6-min walk distance mNDND335378444
RVSP mmHgND40554541
Ppa mmHgNDND41ND30
Ppcw mmHgNDND13ND10
PVR dyn·s·cm−5NDND649ND300
CI L·min−1·m−2NDND1.9ND2.6
RAP mmHgNDND9ND5
SV,O2 %NDND63ND69
REF %NDND153332
TherapyNoneNasal oxygen commencedNasal oxygen, bosentan commencedNasal oxygen plus bosentanNasal oxygen plus bosentan
  • NYHA: New York Heart Association functional class; FVC: forced vital capacity; VC: vital capacity; % pred: % predicted; FEV1: forced expiratory volume in 1 s; RV: residual volume; DL,CO: transfer factor for carbon monoxide; KCO: transfer coefficient of the lung for carbon monoxide; Pa,O2: arterial oxygen tension; RVSP: right ventricular systolic pressure at echocardiography; Ppa: pulmonary arterial pressure; Ppcw: pulmonary capillary wedge pressure; PVR: pulmonary vascular resistance; CI: cardiac index; RAP: right atrial pressure; SV,O2: mixed venous oxygen saturation; REF: isotopic measurement of right systolic ejection fraction; ND: not determined. #: nasal oxygen;: air; +: 4 L·min−1.