Pulmonary Vasoreactivity and Phenotypes in Pulmonary Arterial Hypertension Associated to Connective Tissue Diseases
2018
Background: Pulmonary arterial hypertension (PAH) is a fatal complication in patients with connective tissue disease (CTD).
Objective: The 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. Methods: We 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. Results: We 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). Conclusions: Patients with a partial response to the acute pulmonary vasodilator
test with inhaled iloprost had a longer clinical deterioration-free period than non-responders. (REV INVES CLIN. 2018;70:82-7)
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