Table 2

Prognostic pulmonary arterial hypertension risk stratification scores of the study population

ModelScore format
(x/x/x …)
1-year3-year5-year
SPAHR/COMPERA—(SPAHR equation)n (169)Baselinen (604)Follow-upn (450)Follow-upn (352)Follow-up
 3–11 parameters, original SPAHR/COMPERA(1/2/3)16928/120/21604261/300/43450245/192/13352227/120/5
 3–11 parameters, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)16928/55/65/21604261/182/118/43450245/136/56/13352227/87/33/5
 ¤ 3–11 parameter, original SPAHR/COMPERA, new cut-offs(1/2/3)16928/119/22604259/302/43450244/193/13352226/121/5
 ¤ 3–11 parameters, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)16928/54/65/22604259/180/122/43450244/135/58/13352226/87/34/5
Bi-parametric SPAHR/COMPERA—(SPAHR equation)
 NT-proBNP and WHO-FC, original SPAHR/COMPERA(1/2/3)12510/48/67474149/225/100347145/168/34276141/117/18
 ¤ NT-proBNP and WHO-FC, original SPAHR/COMPERA, new cut-offs(1/2/3)12510/41/74474149/212/113347145/160/42276141/112/23
 NT-proBNP and WHO-FC, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)12510/15/33/67474149/136/89/100347145/117/51/34276141/87/30/18
 ¤ NT-proBNP and WHO-FC, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)12510/14/27/74474149/124/88/113347145/107/53/42276141/80/32/23
 NT-proBNP and 6MWD, original SPAHR/COMPERA(1/2/3)1136/37/70462112/220/130350112/183/55282111/140/31
 ¤ NT-proBNP and 6MWD, original SPAHR/COMPERA, new cut-offs(1/2/3)1136/31/76462112/203/147350112/170/68282111/132/39
 NT-proBNP and 6MWD, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)1136/11/26/70462112/104/116/130350112/94/89/55282111/84/56/31
 ¤ NT-proBNP and 6MWD, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)1136/10/21/76462112/100/103/147350112/90/80/68282111/80/52/39
 6MWD and WHO-FC, original SPAHR/COMPERA(1/2/3)13610/92/34508147/296/65383145/218/20302142/154/6
 6MWD and WHO-FC, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)13610/21/31/74508147/191/105/65383145/157/61/20302142/115/39/6
COMPERA 2.0
 COMPERA 2.0(1/2/3/4)11311/15/68/19447154/134/114/45337151/111/62/12270150/82/35/3
 Bi-parameter (NT-proBNP and 6MWD)(1/2/3/4)1136/14/32/61462112/135/95/120350112/120/64/54282111/103/39/29
 Bi-parameter (NT-proBNP and WHO-FC)(1/2/3/4)12510/14/29/72474149/124/91/110347145/107/53/42276141/80/32/23
 Bi-parameter (6MWD and WHO-FC)(1/2/3/4)13610/21/31/74508147/191/105/65383145/157/61/20302142/115/39/6
ModelScore format
(x/x/x …)
1-year3-year5-year
SPAHR/COMPERA—(SPAHR equation)n (169)Baselinen (604)Follow-upn (450)Follow-upn (352)Follow-up
 3–11 parameters, original SPAHR/COMPERA(1/2/3)16928/120/21604261/300/43450245/192/13352227/120/5
 3–11 parameters, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)16928/55/65/21604261/182/118/43450245/136/56/13352227/87/33/5
 ¤ 3–11 parameter, original SPAHR/COMPERA, new cut-offs(1/2/3)16928/119/22604259/302/43450244/193/13352226/121/5
 ¤ 3–11 parameters, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)16928/54/65/22604259/180/122/43450244/135/58/13352226/87/34/5
Bi-parametric SPAHR/COMPERA—(SPAHR equation)
 NT-proBNP and WHO-FC, original SPAHR/COMPERA(1/2/3)12510/48/67474149/225/100347145/168/34276141/117/18
 ¤ NT-proBNP and WHO-FC, original SPAHR/COMPERA, new cut-offs(1/2/3)12510/41/74474149/212/113347145/160/42276141/112/23
 NT-proBNP and WHO-FC, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)12510/15/33/67474149/136/89/100347145/117/51/34276141/87/30/18
 ¤ NT-proBNP and WHO-FC, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)12510/14/27/74474149/124/88/113347145/107/53/42276141/80/32/23
 NT-proBNP and 6MWD, original SPAHR/COMPERA(1/2/3)1136/37/70462112/220/130350112/183/55282111/140/31
 ¤ NT-proBNP and 6MWD, original SPAHR/COMPERA, new cut-offs(1/2/3)1136/31/76462112/203/147350112/170/68282111/132/39
 NT-proBNP and 6MWD, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)1136/11/26/70462112/104/116/130350112/94/89/55282111/84/56/31
 ¤ NT-proBNP and 6MWD, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)1136/10/21/76462112/100/103/147350112/90/80/68282111/80/52/39
 6MWD and WHO-FC, original SPAHR/COMPERA(1/2/3)13610/92/34508147/296/65383145/218/20302142/154/6
 6MWD and WHO-FC, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)13610/21/31/74508147/191/105/65383145/157/61/20302142/115/39/6
COMPERA 2.0
 COMPERA 2.0(1/2/3/4)11311/15/68/19447154/134/114/45337151/111/62/12270150/82/35/3
 Bi-parameter (NT-proBNP and 6MWD)(1/2/3/4)1136/14/32/61462112/135/95/120350112/120/64/54282111/103/39/29
 Bi-parameter (NT-proBNP and WHO-FC)(1/2/3/4)12510/14/29/72474149/124/91/110347145/107/53/42276141/80/32/23
 Bi-parameter (6MWD and WHO-FC)(1/2/3/4)13610/21/31/74508147/191/105/65383145/157/61/20302142/115/39/6

¤ indicates new NT-proBNP cut-offs.22

6MWD, 6-min walking distance; AUC, area under the ROC curve; COMPERA, Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension; NT-proBNP, N-terminal prohormone of brain natriuretic peptide; SPAHR, Swedish Pulmonary Arterial Hypertension Registry; WHO-FC, World Health Organization Functional Class.

Table 2

Prognostic pulmonary arterial hypertension risk stratification scores of the study population

ModelScore format
(x/x/x …)
1-year3-year5-year
SPAHR/COMPERA—(SPAHR equation)n (169)Baselinen (604)Follow-upn (450)Follow-upn (352)Follow-up
 3–11 parameters, original SPAHR/COMPERA(1/2/3)16928/120/21604261/300/43450245/192/13352227/120/5
 3–11 parameters, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)16928/55/65/21604261/182/118/43450245/136/56/13352227/87/33/5
 ¤ 3–11 parameter, original SPAHR/COMPERA, new cut-offs(1/2/3)16928/119/22604259/302/43450244/193/13352226/121/5
 ¤ 3–11 parameters, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)16928/54/65/22604259/180/122/43450244/135/58/13352226/87/34/5
Bi-parametric SPAHR/COMPERA—(SPAHR equation)
 NT-proBNP and WHO-FC, original SPAHR/COMPERA(1/2/3)12510/48/67474149/225/100347145/168/34276141/117/18
 ¤ NT-proBNP and WHO-FC, original SPAHR/COMPERA, new cut-offs(1/2/3)12510/41/74474149/212/113347145/160/42276141/112/23
 NT-proBNP and WHO-FC, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)12510/15/33/67474149/136/89/100347145/117/51/34276141/87/30/18
 ¤ NT-proBNP and WHO-FC, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)12510/14/27/74474149/124/88/113347145/107/53/42276141/80/32/23
 NT-proBNP and 6MWD, original SPAHR/COMPERA(1/2/3)1136/37/70462112/220/130350112/183/55282111/140/31
 ¤ NT-proBNP and 6MWD, original SPAHR/COMPERA, new cut-offs(1/2/3)1136/31/76462112/203/147350112/170/68282111/132/39
 NT-proBNP and 6MWD, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)1136/11/26/70462112/104/116/130350112/94/89/55282111/84/56/31
 ¤ NT-proBNP and 6MWD, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)1136/10/21/76462112/100/103/147350112/90/80/68282111/80/52/39
 6MWD and WHO-FC, original SPAHR/COMPERA(1/2/3)13610/92/34508147/296/65383145/218/20302142/154/6
 6MWD and WHO-FC, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)13610/21/31/74508147/191/105/65383145/157/61/20302142/115/39/6
COMPERA 2.0
 COMPERA 2.0(1/2/3/4)11311/15/68/19447154/134/114/45337151/111/62/12270150/82/35/3
 Bi-parameter (NT-proBNP and 6MWD)(1/2/3/4)1136/14/32/61462112/135/95/120350112/120/64/54282111/103/39/29
 Bi-parameter (NT-proBNP and WHO-FC)(1/2/3/4)12510/14/29/72474149/124/91/110347145/107/53/42276141/80/32/23
 Bi-parameter (6MWD and WHO-FC)(1/2/3/4)13610/21/31/74508147/191/105/65383145/157/61/20302142/115/39/6
ModelScore format
(x/x/x …)
1-year3-year5-year
SPAHR/COMPERA—(SPAHR equation)n (169)Baselinen (604)Follow-upn (450)Follow-upn (352)Follow-up
 3–11 parameters, original SPAHR/COMPERA(1/2/3)16928/120/21604261/300/43450245/192/13352227/120/5
 3–11 parameters, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)16928/55/65/21604261/182/118/43450245/136/56/13352227/87/33/5
 ¤ 3–11 parameter, original SPAHR/COMPERA, new cut-offs(1/2/3)16928/119/22604259/302/43450244/193/13352226/121/5
 ¤ 3–11 parameters, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)16928/54/65/22604259/180/122/43450244/135/58/13352226/87/34/5
Bi-parametric SPAHR/COMPERA—(SPAHR equation)
 NT-proBNP and WHO-FC, original SPAHR/COMPERA(1/2/3)12510/48/67474149/225/100347145/168/34276141/117/18
 ¤ NT-proBNP and WHO-FC, original SPAHR/COMPERA, new cut-offs(1/2/3)12510/41/74474149/212/113347145/160/42276141/112/23
 NT-proBNP and WHO-FC, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)12510/15/33/67474149/136/89/100347145/117/51/34276141/87/30/18
 ¤ NT-proBNP and WHO-FC, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)12510/14/27/74474149/124/88/113347145/107/53/42276141/80/32/23
 NT-proBNP and 6MWD, original SPAHR/COMPERA(1/2/3)1136/37/70462112/220/130350112/183/55282111/140/31
 ¤ NT-proBNP and 6MWD, original SPAHR/COMPERA, new cut-offs(1/2/3)1136/31/76462112/203/147350112/170/68282111/132/39
 NT-proBNP and 6MWD, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)1136/11/26/70462112/104/116/130350112/94/89/55282111/84/56/31
 ¤ NT-proBNP and 6MWD, updated SPAHR, divided intermediate risk, new cut-offs(1/1.75/2.25/3)1136/10/21/76462112/100/103/147350112/90/80/68282111/80/52/39
 6MWD and WHO-FC, original SPAHR/COMPERA(1/2/3)13610/92/34508147/296/65383145/218/20302142/154/6
 6MWD and WHO-FC, updated SPAHR, divided intermediate risk(1/1.75/2.25/3)13610/21/31/74508147/191/105/65383145/157/61/20302142/115/39/6
COMPERA 2.0
 COMPERA 2.0(1/2/3/4)11311/15/68/19447154/134/114/45337151/111/62/12270150/82/35/3
 Bi-parameter (NT-proBNP and 6MWD)(1/2/3/4)1136/14/32/61462112/135/95/120350112/120/64/54282111/103/39/29
 Bi-parameter (NT-proBNP and WHO-FC)(1/2/3/4)12510/14/29/72474149/124/91/110347145/107/53/42276141/80/32/23
 Bi-parameter (6MWD and WHO-FC)(1/2/3/4)13610/21/31/74508147/191/105/65383145/157/61/20302142/115/39/6

¤ indicates new NT-proBNP cut-offs.22

6MWD, 6-min walking distance; AUC, area under the ROC curve; COMPERA, Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension; NT-proBNP, N-terminal prohormone of brain natriuretic peptide; SPAHR, Swedish Pulmonary Arterial Hypertension Registry; WHO-FC, World Health Organization Functional Class.

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