The present invention provides a high dose of macitentan (INN), i.e. propylsulfamic acid [5-(4-bromo-phenyl)-, for use in the treatment and/or prevention of chronic thromboembolic pulmonary hypertension (CTEPH)- 6-[2-(5-Bromo-pyrimidin-2-yloxy)-ethoxy]-pyrimidin-4-yl]-amide or a pharmaceutically acceptable salt, solvate, hydrate or morphological form thereof (morphological form), or a high dose of aprocitentan (aprocitentan). Furthermore, the present invention relates to the use of a high dose of macitentan or aprocitentan for the manufacture of a medicament for the treatment and/or prophylaxis of CTEPH, as well as CTEPH comprising administering to a patient a high dose of macitentan or aprocitentan. It relates to a method for the treatment and/or prevention of The present invention also provides a dosage regimen for the treatment and/or prophylaxis of CTEPH, as well as macitentan or aprocitentan and one or more phosphodiesterase type 5 (PDE5) inhibitors, prostacyclin analogs, pro to the use of a stacyclin receptor agonist or a soluble guanylate cyclase stimulator. The present invention also relates to a pharmaceutical composition for the treatment of CTEPH, comprising a high dose of macitentan or aprocitentan.
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