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Table 1 Summarized of the treatment course of the PVOD patient

From: Good response to PAH-targeted drugs in a PVOD patient carrying Biallelic EIF2AK4 mutation

Time of Admission

2015.7

2016.3

2017.3

2018.1

2018.4

SaO2(pre-treatment, 21% O2)

88

89

90

88

89

SaO2(%) (post-treatment, 21% O2)

96

95

96

94

96

NT-proBNP (ng/ml, pre-treatment

3590

5080

24,389

9423

3957

NT-proBNP (ng/ml, post-treatment

980

746

890

1020

920

Functional class (NYHA, pre-treatment)

III

III

III

IV

III

Functional class (NYHA, post-treatment)

II

II

II

II

II

PAH-targeted drugs (in hospital)

Bosentan

Ambrisentan+Sildenafil

Ambrisentan+Sildenafil

Ambrisentan+Sildenafil+Treprostinil injection

Ambrisentan+Sildenafil+Treprostinil injection

PAH-targeted drugs (discharge)

Bosentan (changed to Ambrisentan, because of liver dysfunction 4 months later), Ambrisentan

Ambrisentan+Sildenafil

Ambrisentan+Sildenafil

Ambrisentan+Sildenafil

Ambrisentan+Sildenafil

  1. Note: SaO2 oxygen saturation, NT-proBNP N-terminal pro-brain natriuretic peptide, NYHA New York Heart Association