Determinação da prevalência de hipertensão pulmonar pelo cateterismo cardíaco em pacientes com esclerose sistêmica

Abstract

Systemic sclerosis (ES) is a generalized autoimmune disorder of small arteries, microvessels and connective tissue, characterized by fibrosis and vascular obliteration in the skin and organs, especially the lungs, heart and digestive tract. Pulmonary involvement is frequent in ES patients, with pulmonary interstitial disease being the most frequent (80%) and pulmonary arterial hypertension (PAH) one of the most severe manifestations. PAH is defined hemodynamically by right heart catheterization (RHC) when the mean pulmonary artery pressure (mPAP) is > 25mmHg at rest. 8% to 12% of ES patients have PAH using RHC as diagnosis. The research aims to evaluate the prevalence of PAH in ES patients. Therefore a non- randomized cross-sectional study of patients with ES from a single center, classified by ES diagnosis criteria, using as screening step the clinical data association (telangiectasia) and complementary tests [percentage ratio of vital capacity forced by the capacity to Carbon monoxide (FVC / DLCO), presence of anticentromeric antibody, uric acid dosage, NT-proBNP dosage, cardiac electrical axis deviation to right] and transthoracic echocardiogram (ECOTT). If the patients achieved 300 points for clinical data and complementary methods and PSAP > 35mmHg, the patients underwent cardiac catheterization. The data were evaluated by Fischer's exact test, chi-square test, analysis of variance and Bland-Altman. Eighty three cases of ES were screened, 20 of which fulfilled the selection criteria and performed cardiac catheterization, of which 10 had HP. 5 had PAH. The sensitivity 66.67% (CI 47,19-82,71), the specificity of the proposed method was 83.33% (CI 71.48-91.71), the positive predictive value was 66.67% (CI 51,83-78,80) and the negative predictive value of 83.33% (CI 74.85-89.36). In all patients with PAH the FVC / DLCO ratio was above 1.7. We came to the conclusion that the prevalence of PH and PAH is comparable with the international literature.