Comparison of study parameters in patients who died due to pulmonary arterial hypertension and those who did not
Parameter . | All (n = 24) . | PAH-related mortality . | P . | |
---|---|---|---|---|
Yes (n = 10) . | No (n = 14) . | |||
Demographics | ||||
Baseline age in years | 46.5 ( | 44.5 ( | 48 ( | .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 ( | 470 ( | 486.5 ( | .711 |
Right heart catheterization (baseline) | ||||
mPAP in mm Hg | 41.5 ( | 45.5 ( | 40.5 ( | .437 |
sPAP in mm Hg | 63 ( | 60 ( | 66 ( | .563 |
Cardiac index in l/min/ | 3.4 ( | 2.9 ( | 3.8 ( | .877 |
PVR in dyn-s- | 500.9 ( | 611.9 ( | 470.9 ( | .267 |
Vasoreactivity*, % | 26.7 | 42.9 | 12.5 | .282 |
Laboratory | ||||
Hemoglobin in g/dL | ||||
Baseline | 9.2 ( | 9.7 ( | 9.2 ( | .522 |
Change from baseline | .414 | |||
Serum ferritin in ng/mL | ||||
Baseline | 694 ( | 550 ( | 985.9 ( | .077 |
Change from baseline | .710 | |||
Echocardiography | ||||
LVEF in % | ||||
Baseline | 60 ( | 60 ( | 60 ( | .926 |
Change from baseline | 0 ( | .711 | ||
TRV in m/s | ||||
Baseline | 3.8 ( | 3.7 ( | 4.0 ( | .752 |
Change from baseline | .400 | |||
sPAP in mm Hg | ||||
Baseline | 68.5 ( | 65 ( | 71 ( | .931 |
Change from baseline | .024 | |||
TAPSE in cm | ||||
Baseline | 20 ( | 19.3 ( | 22 ( | .235 |
Change from baseline | .661 | |||
Right atrium area in | ||||
Baseline | 31 ( | 32 ( | 31 ( | .630 |
Change from baseline | 0 ( | 1.000 |
Parameter . | All (n = 24) . | PAH-related mortality . | P . | |
---|---|---|---|---|
Yes (n = 10) . | No (n = 14) . | |||
Demographics | ||||
Baseline age in years | 46.5 ( | 44.5 ( | 48 ( | .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 ( | 470 ( | 486.5 ( | .711 |
Right heart catheterization (baseline) | ||||
mPAP in mm Hg | 41.5 ( | 45.5 ( | 40.5 ( | .437 |
sPAP in mm Hg | 63 ( | 60 ( | 66 ( | .563 |
Cardiac index in l/min/ | 3.4 ( | 2.9 ( | 3.8 ( | .877 |
PVR in dyn-s- | 500.9 ( | 611.9 ( | 470.9 ( | .267 |
Vasoreactivity*, % | 26.7 | 42.9 | 12.5 | .282 |
Laboratory | ||||
Hemoglobin in g/dL | ||||
Baseline | 9.2 ( | 9.7 ( | 9.2 ( | .522 |
Change from baseline | .414 | |||
Serum ferritin in ng/mL | ||||
Baseline | 694 ( | 550 ( | 985.9 ( | .077 |
Change from baseline | .710 | |||
Echocardiography | ||||
LVEF in % | ||||
Baseline | 60 ( | 60 ( | 60 ( | .926 |
Change from baseline | 0 ( | .711 | ||
TRV in m/s | ||||
Baseline | 3.8 ( | 3.7 ( | 4.0 ( | .752 |
Change from baseline | .400 | |||
sPAP in mm Hg | ||||
Baseline | 68.5 ( | 65 ( | 71 ( | .931 |
Change from baseline | .024 | |||
TAPSE in cm | ||||
Baseline | 20 ( | 19.3 ( | 22 ( | .235 |
Change from baseline | .661 | |||
Right atrium area in | ||||
Baseline | 31 ( | 32 ( | 31 ( | .630 |
Change from baseline | 0 ( | 1.000 |
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.