title: Inoperable chronic thromboembolic pulmonary hypertension treated with riociguat: A case study.
abstract: A 51-year-old woman presented with a one-year history of progressive dyspnea, WHO functional class III-IV and exercise-related syncope. Transthoracic echocardiography and computed tomography pulmonary angiography were performed, leading to a diagnosis of pulmonary arterial hypertension. She was referred to our pulmonary hypertension unit, where a complete study was performed, including ventilation/perfusion scan, which was consistent with chronic thromboembolic pulmonary hypertension. Risk factors for this condition were excluded and therapeutic options were evaluated. Imaging studies showed distal pulmonary disease so pulmonary endarterectomy was rejected. Further therapeutic options were evaluated and the patient was subsequently enrolled in an open-label uncontrolled trial with riociguat. After one year of treatment, significant improvement in functional class, 6-minute walk test and NT-proBNP were seen, without significant secondary effects.
authors.affiliation: Department of Cardiology, Institute of Heart Sciences (ICICOR), Hospital Clinico Universitario, Valladolid, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Ortiz-Bautista
authors.affiliation: Multidisciplinary Pulmonary Hypertension Unit, Cardiology Service, Hospital Universitario 12 de Octubre, CIBERES, Madrid, Spain.
authors.forename: Nuria
authors.initials: N
authors.lastname: Ochoa-Parra
authors.affiliation: Multidisciplinary Pulmonary Hypertension Unit, Cardiology Service, Hospital Universitario 12 de Octubre, CIBERES, Madrid, Spain.
authors.forename: Paula
authors.initials: P
authors.lastname: Navas-Tejedor
authors.affiliation: Multidisciplinary Pulmonary Hypertension Unit, Cardiology Service, Hospital Universitario 12 de Octubre, CIBERES, Madrid, Spain.
authors.forename: Laura
authors.initials: L
authors.lastname: Morán-Fernández
authors.affiliation: Multidisciplinary Pulmonary Hypertension Unit, Cardiology Service, Hospital Universitario 12 de Octubre, CIBERES, Madrid, Spain. Electronic address: mgomezsanchez@salud.madrid.org.
authors.forename: Miguel Ángel
authors.initials: MÁ
authors.lastname: Gómez-Sánchez
mesh_terms: D002908:Chronic Disease; D015444:Exercise; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D008875:Middle Aged; D011720:Pyrazoles / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*
chemical_list: D011720:Pyrazoles; D011743:Pyrimidines; C542595:riociguat
keywords: Chronic thromboembolic pulmonary hypertension; Hipertensão pulmonar tromboembólica crónica; Perfil de segurança do riociguat; Safety profile of riociguat; Tratamento com riociguat; Treatment with riociguat