Table 1.

Comparison of study parameters in patients who died due to pulmonary arterial hypertension and those who did not

ParameterAll (n = 24)PAH-related mortalityP
Yes (n = 10)No (n = 14)
Demographics     
 Baseline age in years 46.5 (39.35944.5 (38.36348 (39.557.709 
 Male, % 50 40 57.1 .680 
 Thalassemia diagnosis, %     
  Thalassemia major 41.7 40 42.9 1.000 
  Thalassemia intermedia 58.3 60 57.1  
 Splenectomized, % 91.7 100 85.7 .493 
Functional status (baseline)     
 NYHA class, %     
  I or II 34.8 44.4 28.6 .657 
  III or IV 65.2 55.6 71.4  
 6-MWD in m 480 (100685470 (420500486.5 (402.5537.5.711 
Right heart catheterization (baseline)     
 mPAP in mm Hg 41.5 (354845.5 (3550.540.5 (33.847.5.437 
 sPAP in mm Hg 63 (538660 (518366 (4988.5.563 
 Cardiac index in l/min/m2 3.4 (2.74.72.9 (2.753.8 (2.54.6.877 
 PVR in dyn-s-cm5 500.9 (406.1828.1611.9 (453.9918.9470.9 (398.6651.6.267 
 Vasoreactivity*, % 26.7 42.9 12.5 .282 
Laboratory     
 Hemoglobin in g/dL     
  Baseline 9.2 (8.89.99.7 (8.810.19.2 (8.79.6.522 
  Change from baseline +0.3 (0.30.6+0.4 (0.00.9+0.3 (0.90.6.414 
 Serum ferritin in ng/mL     
  Baseline 694 (412.51250.9550 (344.6820.7985.9 (598.21730.2.077 
  Change from baseline 100.2 (334.1174.756.6 (265.8210.5166.5 (400.6174.7.710 
Echocardiography     
 LVEF in %     
  Baseline 60 (556560 (56.56660 (5565.5.926 
  Change from baseline 0 (55+2 (550.5 (5.53.4.711 
 TRV in m/s     
  Baseline 3.8 (3.44.53.7 (3.44.74.0 (3.44.5.752 
  Change from baseline +0.3 (0.90.9+0.3 (0.30.30.7 (1.470.7.400 
 sPAP in mm Hg     
  Baseline 68.5 (49.39065 (4891.371 (4990.5.931 
  Change from baseline 2 (362.8+6.5 (7.525.521.5 (420.8.024 
 TAPSE in cm     
  Baseline 20 (1823.319.3 (17.521.522 (1925.235 
  Change from baseline +1 (3.84.5+1 (4.53.9+1 (45.661 
 Right atrium area in cm2     
  Baseline 31 (233232 (233231 (19.532.630 
  Change from baseline 4 (710 (004 (711.000 
ParameterAll (n = 24)PAH-related mortalityP
Yes (n = 10)No (n = 14)
Demographics     
 Baseline age in years 46.5 (39.35944.5 (38.36348 (39.557.709 
 Male, % 50 40 57.1 .680 
 Thalassemia diagnosis, %     
  Thalassemia major 41.7 40 42.9 1.000 
  Thalassemia intermedia 58.3 60 57.1  
 Splenectomized, % 91.7 100 85.7 .493 
Functional status (baseline)     
 NYHA class, %     
  I or II 34.8 44.4 28.6 .657 
  III or IV 65.2 55.6 71.4  
 6-MWD in m 480 (100685470 (420500486.5 (402.5537.5.711 
Right heart catheterization (baseline)     
 mPAP in mm Hg 41.5 (354845.5 (3550.540.5 (33.847.5.437 
 sPAP in mm Hg 63 (538660 (518366 (4988.5.563 
 Cardiac index in l/min/m2 3.4 (2.74.72.9 (2.753.8 (2.54.6.877 
 PVR in dyn-s-cm5 500.9 (406.1828.1611.9 (453.9918.9470.9 (398.6651.6.267 
 Vasoreactivity*, % 26.7 42.9 12.5 .282 
Laboratory     
 Hemoglobin in g/dL     
  Baseline 9.2 (8.89.99.7 (8.810.19.2 (8.79.6.522 
  Change from baseline +0.3 (0.30.6+0.4 (0.00.9+0.3 (0.90.6.414 
 Serum ferritin in ng/mL     
  Baseline 694 (412.51250.9550 (344.6820.7985.9 (598.21730.2.077 
  Change from baseline 100.2 (334.1174.756.6 (265.8210.5166.5 (400.6174.7.710 
Echocardiography     
 LVEF in %     
  Baseline 60 (556560 (56.56660 (5565.5.926 
  Change from baseline 0 (55+2 (550.5 (5.53.4.711 
 TRV in m/s     
  Baseline 3.8 (3.44.53.7 (3.44.74.0 (3.44.5.752 
  Change from baseline +0.3 (0.90.9+0.3 (0.30.30.7 (1.470.7.400 
 sPAP in mm Hg     
  Baseline 68.5 (49.39065 (4891.371 (4990.5.931 
  Change from baseline 2 (362.8+6.5 (7.525.521.5 (420.8.024 
 TAPSE in cm     
  Baseline 20 (1823.319.3 (17.521.522 (1925.235 
  Change from baseline +1 (3.84.5+1 (4.53.9+1 (45.661 
 Right atrium area in cm2     
  Baseline 31 (233232 (233231 (19.532.630 
  Change from baseline 4 (710 (004 (711.000 

All data presented as median (IQR) unless otherwise specified.

6-MWD, 6-min walk distance; LVEF, left-ventricular ejection fraction; mPAP, mean pulmonary arterial pressure; NYHA, New York Heart Association; TAPSE, tricuspid annular plane systolic excursion.

*

A positive acute response was defined as a reduction in mPAP of 10 mm Hg to reach an absolute value of 40 mm Hg with an increased or unchanged cardiac output.