• Rev Invest Clin · Jan 2018

    Pulmonary Vasoreactivity and Phenotypes in Pulmonary Arterial Hypertension Associated to Connective Tissue Diseases.

    • José Luis Hernández-Oropeza, Tatiana Sofía Rodríguez-Reyna, Diego Luis Carrillo-Pérez, José de Jesús Rodríguez-Andoney, René Narváez-David, Yesenia Salado-Morales, Eduardo Rivero-Sigarroa, Guillermo Domínguez-Cherit, and Tomás Pulido-Zamudio.
    • Department of Cardiopulmonary Disease, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico.
    • Rev Invest Clin. 2018 Jan 1; 70 (2): 82-7.

    BackgroundPulmonary arterial hypertension (PAH) is a fatal complication in patients with connective tissue disease (CTD).ObjectiveThe objective of the study was to study the prognostic value of the acute pulmonary vasoreactivity test with inhaled iloprost and its association with clinical deterioration in a tertiary care academic medical center.MethodsWe conducted a prospective study of patients with CTD and the diagnosis of PAH established by right heart catheterization. Patients were classified into classic responders, partial responders, and non-responders. The association of the pulmonary response and clinical deterioration was analyzed.ResultsWe enrolled 25 patients (mean age of 47 ± 13.4 years); 88% were female. The most frequent rheumatologic diagnosis was systemic lupus erythematosus, in 16 (64%) patients. Seventy-two percent of patients were classified as non-responders, and 28% were partial responders. Patients with a partial response had lower right atrial pressure values (5.1 ± 3.1 vs. 8.5 ± 3.2, p = 0.01) and greater systolic pulmonary arterial pressure (87.6 ± 8.1 vs. 72.4 ± 16.2, p = 0.02), compared with non-responders. Non-responders had a tendency for a shorter time to clinical deterioration than partial responders (17.8 vs. 41.1 months, p = 0.052).ConclusionsPatients with a partial response to the acute pulmonary vasodilator test with inhaled iloprost had a longer clinical deterioration-free period than non-responders.Copyright: © 2017 SecretarÍa de Salud.

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