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6.3.3.2.3 Macitentan. Macitentan is an oral, dual ERA that has

been found to increase exercise capacity and reduce a composite endpoint of clinical worsening in patients with PAH.167 While no liver toxicity has been shown, a reduction in Hb to ≤8 g/dL was observed in 4.3% of patients receiving 10 mg of macitentan.167 6.3.3.3 Phosphodiesterase 5 inhibitors and guanylate cyclase

stimulators Stimulating soluble guanylate cyclase (sGC) by nitric oxide results in production of the intracellular second messenger cyclic guanosine monophosphate (cGMP) (Figure 7). This pathway is controlled by a

Risk (3 strata)

( T able 16)

T

T

Initi a l ER A

R

R + P DE5i ther a p y

p

p (Cl ass I ) I niti a l E RA + PD E 5 i

a n d i. v .

v v /s . c . P

. C A b (Cl ass I Ia ) I niti a l ora l mono th er a p y

p

p

with P D E 5i or E R A

R

R (Cl ass I Ia )

A d d i. v .

v v o

. r s

r . c . P

. C A and/o r

e va l ua te

a a f o

f f r l u n g t rans pl an t a ti

a o n

(Cl ass I Ia )

R e g ul ar f o

f f ll o w- u p assess m e nt a n d i nd i v i dua li z e d t h e r a p y

p

p

Re g ul a r f o

f f ll o w-u p ass e ss me n t

( T ab

T

T l e 17 )

L o w or intermediate High

Risk (4 strata)

( T able 18)

T

T

C o nti n ue initi a l ther a p y

p

p

(Cl ass I )

L o w Intermediate-l o w Intermediate-high

or high

O R

A d d P R A

R R (Cl ass I I a ) Swit c h f r o m

P D E 5i t o s GC s

(Cl ass I I b )

T r T T eatment of patients with I/H/D- P AH or

P P P AH-CTD

P P

Diagnosis confirmed at PH cent r e , vaso

, r eactivity testing negati v e

General measu r es th r oughout

the course of the disease

(ReCo T able 5)

T T (Class I)

Patient without ca r diopulmona r y comorbidities a Patient with

ca r diopulmona r y comorbidities a

All risk cate g ories

Figure 9 Evidence-based pulmonary arterial hypertension treatment algorithm for patients with idiopathic, heritable, drug-associated, and connective tissue disease-associated pulmonary arterial hypertension. DLCO, Lung diffusion capacity for carbon monoxide; ERA, endothelin receptor antagonist; I/ H/D-PAH, idiopathic, heritable, or drug-associated pulmonary arterial hypertension; i.v., intravenous; PAH-CTD, PAH associated with connective tissue disease; PCA, prostacyclin analogue; PDE5i, phosphodiesterase 5 inhibitor; PH, pulmonary hypertension; PRA, prostacyclin receptor agonist; ReCo, recommendation; s.c., subcutaneous; sGCs, soluble guanylate cyclase stimulator. aCardiopulmonary comorbidities are conditions associated with an increased risk of left ventricular diastolic dysfunction, and include obesity, hypertension, diabetes mellitus, and coronary heart disease; pulmonary comorbidities may include signs of mild parenchymal lung disease and are often associated with a low DLCO (,45% of the predicted value). bIntravenous epoprostenol or i.v./s.c. treprostinil. Downloaded from academic.oup.com/eurheartj/article/43/38/3618/6673929 by guest on 07 October 2026