Table 1. Comparison of study parameters... | American Society of Hematology

Table 1.

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
Parameter	All (n = 24)	Yes (n = 10)	No (n = 14)	P
Demographics
Baseline age in years	46.5 (⁠ $39.3 - 59$ ⁠)	44.5 (⁠ $38.3 - 63$ ⁠)	48 (⁠ $39.5 - 57$ ⁠)	.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 (⁠ $100 - 685$ ⁠)	470 (⁠ $420 - 500$ ⁠)	486.5 (⁠ $402.5 - 537.5$ ⁠)	.711
Right heart catheterization (baseline)
mPAP in mm Hg	41.5 (⁠ $35 - 48$ ⁠)	45.5 (⁠ $35 - 50.5$ ⁠)	40.5 (⁠ $33.8 - 47.5$ ⁠)	.437
sPAP in mm Hg	63 (⁠ $53 - 86$ ⁠)	60 (⁠ $51 - 83$ ⁠)	66 (⁠ $49 - 88.5$ ⁠)	.563
Cardiac index in l/min/ $m^{2}$	3.4 (⁠ $2.7 - 4.7$ ⁠)	2.9 (⁠ $2.7 - 5$ ⁠)	3.8 (⁠ $2.5 - 4.6$ ⁠)	.877
PVR in dyn-s- ${cm}^{- 5}$	500.9 (⁠ $406.1 - 828.1$ ⁠)	611.9 (⁠ $453.9 - 918.9$ ⁠)	470.9 (⁠ $398.6 - 651.6$ ⁠)	.267
Vasoreactivity*, %	26.7	42.9	12.5	.282
Laboratory
Hemoglobin in g/dL
Baseline	9.2 (⁠ $8.8 - 9.9$ ⁠)	9.7 (⁠ $8.8 - 10.1$ ⁠)	9.2 (⁠ $8.7 - 9.6$ ⁠)	.522
Change from baseline	$+ 0.3$ (⁠ $- 0.3 - 0.6$ ⁠)	$+ 0.4$ (⁠ $0.0 - 0.9$ ⁠)	$+ 0.3$ (⁠ $- 0.9 - 0.6$ ⁠)	.414
Serum ferritin in ng/mL
Baseline	694 (⁠ $412.5 - 1250.9$ ⁠)	550 (⁠ $344.6 - 820.7$ ⁠)	985.9 (⁠ $598.2 - 1730.2$ ⁠)	.077
Change from baseline	$- 100.2$ (⁠ $- 334.1 - 174.7$ ⁠)	$- 56.6$ (⁠ $- 265.8 - 210.5$ ⁠)	$- 166.5$ (⁠ $- 400.6 - 174.7$ ⁠)	.710
Echocardiography
LVEF in %
Baseline	60 (⁠ $55 - 65$ ⁠)	60 (⁠ $56.5 - 66$ ⁠)	60 (⁠ $55 - 65.5$ ⁠)	.926
Change from baseline	0 (⁠ $- 5 - 5$ ⁠)	$+ 2$ (⁠ $- 5 - 5$ ⁠)	$- 0.5$ (⁠ $- 5.5 - 3.4$ ⁠)	.711
TRV in m/s
Baseline	3.8 (⁠ $3.4 - 4.5$ ⁠)	3.7 (⁠ $3.4 - 4.7$ ⁠)	4.0 (⁠ $3.4 - 4.5$ ⁠)	.752
Change from baseline	$+ 0.3$ (⁠ $- 0.9 - 0.9$ ⁠)	$+ 0.3$ (⁠ $0.3 - 0.3$ ⁠)	$- 0.7$ (⁠ $- 1.47 - 0.7$ ⁠)	.400
sPAP in mm Hg
Baseline	68.5 (⁠ $49.3 - 90$ ⁠)	65 (⁠ $48 - 91.3$ ⁠)	71 (⁠ $49 - 90.5$ ⁠)	.931
Change from baseline	$- 2$ (⁠ $- 36 - 2.8$ ⁠)	$+ 6.5$ (⁠ $- 7.5 - 25.5$ ⁠)	$- 21.5$ (⁠ $- 42 - 0.8$ ⁠)	.024
TAPSE in cm
Baseline	20 (⁠ $18 - 23.3$ ⁠)	19.3 (⁠ $17.5 - 21.5$ ⁠)	22 (⁠ $19 - 25$ ⁠)	.235
Change from baseline	$+ 1$ (⁠ $- 3.8 - 4.5$ ⁠)	$+ 1$ (⁠ $- 4.5 - 3.9$ ⁠)	$+ 1$ (⁠ $- 4 - 5$ ⁠)	.661
Right atrium area in ${cm}^{2}$
Baseline	31 (⁠ $23 - 32$ ⁠)	32 (⁠ $23 - 32$ ⁠)	31 (⁠ $19.5 - 32$ ⁠)	.630
Change from baseline	$- 4$ (⁠ $- 7 - 1$ ⁠)	0 (⁠ $0 - 0$ ⁠)	$- 4$ (⁠ $- 7 - 1$ ⁠)	1.000

Parameter	All (n = 24)	PAH-related mortality		P
Parameter	All (n = 24)	Yes (n = 10)	No (n = 14)	P
Demographics
Baseline age in years	46.5 (⁠ $39.3 - 59$ ⁠)	44.5 (⁠ $38.3 - 63$ ⁠)	48 (⁠ $39.5 - 57$ ⁠)	.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 (⁠ $100 - 685$ ⁠)	470 (⁠ $420 - 500$ ⁠)	486.5 (⁠ $402.5 - 537.5$ ⁠)	.711
Right heart catheterization (baseline)
mPAP in mm Hg	41.5 (⁠ $35 - 48$ ⁠)	45.5 (⁠ $35 - 50.5$ ⁠)	40.5 (⁠ $33.8 - 47.5$ ⁠)	.437
sPAP in mm Hg	63 (⁠ $53 - 86$ ⁠)	60 (⁠ $51 - 83$ ⁠)	66 (⁠ $49 - 88.5$ ⁠)	.563
Cardiac index in l/min/ $m^{2}$	3.4 (⁠ $2.7 - 4.7$ ⁠)	2.9 (⁠ $2.7 - 5$ ⁠)	3.8 (⁠ $2.5 - 4.6$ ⁠)	.877
PVR in dyn-s- ${cm}^{- 5}$	500.9 (⁠ $406.1 - 828.1$ ⁠)	611.9 (⁠ $453.9 - 918.9$ ⁠)	470.9 (⁠ $398.6 - 651.6$ ⁠)	.267
Vasoreactivity*, %	26.7	42.9	12.5	.282
Laboratory
Hemoglobin in g/dL
Baseline	9.2 (⁠ $8.8 - 9.9$ ⁠)	9.7 (⁠ $8.8 - 10.1$ ⁠)	9.2 (⁠ $8.7 - 9.6$ ⁠)	.522
Change from baseline	$+ 0.3$ (⁠ $- 0.3 - 0.6$ ⁠)	$+ 0.4$ (⁠ $0.0 - 0.9$ ⁠)	$+ 0.3$ (⁠ $- 0.9 - 0.6$ ⁠)	.414
Serum ferritin in ng/mL
Baseline	694 (⁠ $412.5 - 1250.9$ ⁠)	550 (⁠ $344.6 - 820.7$ ⁠)	985.9 (⁠ $598.2 - 1730.2$ ⁠)	.077
Change from baseline	$- 100.2$ (⁠ $- 334.1 - 174.7$ ⁠)	$- 56.6$ (⁠ $- 265.8 - 210.5$ ⁠)	$- 166.5$ (⁠ $- 400.6 - 174.7$ ⁠)	.710
Echocardiography
LVEF in %
Baseline	60 (⁠ $55 - 65$ ⁠)	60 (⁠ $56.5 - 66$ ⁠)	60 (⁠ $55 - 65.5$ ⁠)	.926
Change from baseline	0 (⁠ $- 5 - 5$ ⁠)	$+ 2$ (⁠ $- 5 - 5$ ⁠)	$- 0.5$ (⁠ $- 5.5 - 3.4$ ⁠)	.711
TRV in m/s
Baseline	3.8 (⁠ $3.4 - 4.5$ ⁠)	3.7 (⁠ $3.4 - 4.7$ ⁠)	4.0 (⁠ $3.4 - 4.5$ ⁠)	.752
Change from baseline	$+ 0.3$ (⁠ $- 0.9 - 0.9$ ⁠)	$+ 0.3$ (⁠ $0.3 - 0.3$ ⁠)	$- 0.7$ (⁠ $- 1.47 - 0.7$ ⁠)	.400
sPAP in mm Hg
Baseline	68.5 (⁠ $49.3 - 90$ ⁠)	65 (⁠ $48 - 91.3$ ⁠)	71 (⁠ $49 - 90.5$ ⁠)	.931
Change from baseline	$- 2$ (⁠ $- 36 - 2.8$ ⁠)	$+ 6.5$ (⁠ $- 7.5 - 25.5$ ⁠)	$- 21.5$ (⁠ $- 42 - 0.8$ ⁠)	.024
TAPSE in cm
Baseline	20 (⁠ $18 - 23.3$ ⁠)	19.3 (⁠ $17.5 - 21.5$ ⁠)	22 (⁠ $19 - 25$ ⁠)	.235
Change from baseline	$+ 1$ (⁠ $- 3.8 - 4.5$ ⁠)	$+ 1$ (⁠ $- 4.5 - 3.9$ ⁠)	$+ 1$ (⁠ $- 4 - 5$ ⁠)	.661
Right atrium area in ${cm}^{2}$
Baseline	31 (⁠ $23 - 32$ ⁠)	32 (⁠ $23 - 32$ ⁠)	31 (⁠ $19.5 - 32$ ⁠)	.630
Change from baseline	$- 4$ (⁠ $- 7 - 1$ ⁠)	0 (⁠ $0 - 0$ ⁠)	$- 4$ (⁠ $- 7 - 1$ ⁠)	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.

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