Baseline | FU-1 | FU-2 | |
---|---|---|---|
Subjects, n | 20* | 20 | 16 |
CEP (deaths) | 16 (4) | 10 (4) | |
Age, years | 49.2 ± 15.1 | 49.8 ± 14.9 | 50.1 ± 14.4 |
Sex (females), % (n) | 70 (14) | 70 (14) | 81 (13) |
WHO Class | 2.1 ± 0.7 | 2.3 ± 0.7 | 2.2 ± 0.7 |
6 min walking test distance, m | 404 ± 87.8 | 412 ± 77 | 420 ± 111 |
BNP, pg/ml | 90.8 [46–282] | 114 [77–245] | 113 [76–252] |
PAH etiology | |||
Idiopathic/heritable PAH, % (n) | 60 (12) | 60 (12) | 56 (9) |
Connective tissue disease related PAH, % (n) | 15 (3) | 15 (3) | 12 (2) |
Congenital heart disease related PAH, % (n) | 25 (5) | 25 (5) | 32 (5) |
PAH specific therapy | |||
Phosphodiesterase type 5 inhibitors, % (n) | 38 (10) | 10 (2) | 12 (2) |
Endothelin receptor antagonists, % (n) | 11 (3) | 15 (3) | 0 (0) |
Prostacyclins, % (n) | 20 (5) | 65 (13) | 50 (8) |
Phosphodiesteraze type 5 inhibitors + endothelin receptor antagonists, % (n) | 31 (8) | 10 (2) | 38 (6) |
Hemodynamics | |||
Systolic pulmonary artery pressure, mm Hg | 82.2 ± 29.2 | 78.2 ± 24.2 | 75.3 ± 32.3 |
Diastolic pulmonary artery pressure, mm Hg | 33.8 ± 14.3 | 31.2 ± 13.9 | 29.43 ± 14 |
Mean pulmonary artery pressure, mm Hg | 50.5 ± 18.3 | 42.8 ± 18.6^ | 32.6 ± 12.1^^ |
Pulmonary capillary wedge pressure, mm Hg | 10.6 ± 2.5 | 9.73 ± 3 | 8.8 ± 2.8 |
Pulmonary vascular resistance, Wood units | 8.9 ± 5.7 | 7.3 ± 4.7^ | 5.3 ± 2.8^^ |
Cardiac index, L/min/m2 | 2.5 ± 0.4 | 2.9 ± 0.4^ | 2.9 ± 0.6^^ |
Right atrium pressure, mm Hg | 8.6 ± 3.6 | 8.1 ± 5.3 | 8 ± 5.3 |
RV parameters (MRI) | |||
RV ejection fraction, % | 45.1 ± 9.6 | 52.4 ± 12.9^ | |
RV EDV/BSA, mL/m2 | 113.2 ± 24.5 | 106 ± 27 | |
RV ESV/BSA, mL/m2 | 62.7 ± 22.7 | 50 ± 11 | |
RV mass/BSA, g/m2 | 39.9 ± 13.9 | 39.2 ± 14.6 | |
RV compacted myocardium thickness, mm | 5.7 ± 1.5 | 5.2 ± 1.3 | |
Pulmonary arterial compliance, mL/mm Hg | 2.4 ± 1.8 | 3.2 ± 2.4^ | |
Right ventricle stroke work index, g*m*m2/beat | 20.6 ± 8.4 | 18.2 ± 7.5 | |
Myocardial metabolism (PET) | |||
SUVRV | 2.5 [1.4–5.5] | 3.92 [1.6–8.1] | |
SUVLV | 3.5 [2.1–6.6] | 5.7 [4.8–8.9] | |
SUVRV/SUVLV ratio | 0.94 [0.48–1.38] | 0.63 [0.41–1.16] |