TABLE 2

Clinical data at baseline and during follow-up

BaselineFollow-up#Changep-value
nmean±sdnmean±sdmean±sd
6MWD91403.2±111.466388±125−21.8±79.30.039
NT-proBNP pg·mL−195216±26677396±1068188±10300.005
Pulmonary function testing
 FVC L942.9±1.0752.7±1.0−0.1±0.30.005
 FVC %9691.2±23.77689.2±24.6−1.1±11.9ns
 FEV1 L942.2±0.8742.0±0.8−0.1±0.3<0.001
 FEV1 %9585.7±22.97582.6±21.7−1.3±11.8ns
DLCO mmol·min−1·kPa−1937.5±4.0683.9±1.5−4.0±4.0<0.001
DLCO %9548.9±10.87046.3±11.8−3.1±8.2<0.001
KCO mmol·min−1·kPa−1·L−1932.5±1.5691.5±1.1−1.3±1.9<0.001
KCO %9371.4±16.96967.7±17.1−5.1±23.00.011
 TLC %8385.8±22.37089.7±22.92.7±11.4ns
 RV %8485.8±33.16394.4±34.25.5±23.1ns
Echocardiography
 LA mm9329.1±6.47230±70.5±6.4ns
 IVC mm8214.0±3.76413±6−2.0±6.8ns
 IVS mm9210.1±1.84010±20.0±3.2ns
 RA cm28912.1±3.77212.7±4.00.2±4.2ns
 RVD mm8529.1±5.64231±73.8±7.60.01
 RV cm28814.5±4.37213.2±3.7−1.2±4.10.043
 LV-EDD mm9244.1±5.54242±8−1.5±8.5ns
 LV-ESD mm9127.0±5.14227±70.0±7.0ns
 TRV m·s−1882.4±0.4632.6±0.40.1±0.50.019
 TAPSE mm8922.5±4.67722±5−0.4±6.1ns
 sPAP mmHg8729.1±7.16332±93.1±9.10.017
Right heart catheterisation
 mPAP mmHg9617.1±4.07122±63.8±5.5<0.001
 PAWP mmHg968.5±3.37111±31.9±4.3<0.001
 TPG mmHg968.5±2.9719±30.1±2.6ns
 CO L·min−1965.3±1.2715.1±1.1−0.1±0.9ns
 PVR dynes96135±55.471182±11843.5±96.10.001

6MWD: 6-min walking distance; NT-proBNP: N-terminal pro-brain natriuretic peptide; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; DLCO: diffusing capacity of the lung for carbon monoxide; KCO: transfer coefficient of the lung for carbon monoxide; TLC: total lung capacity; RV: residual volume; LA: left atrium; IVC: inferior vena cava; IVS: interventricular septum; RA: right atrium; RVD: right ventricular diameter; RV: right ventricle; LV: left ventricle; EDD: end-diastolic diameter; ESD: end-systolic diameter; TRV: tricuspid regurgitation velocity; TAPSE: tricuspid annular plane systolic excursion; sPAP: systolic pulmonary arterial pressure; mPAP: mean pulmonary arterial pressure; PAWP: pulmonary arterial wedge pressure; TPG: transpulmonary gradient; CO: cardiac output; PVR: pulmonary vascular resistance; ns: nonsignificant. #: follow-up after 3 years.