Chest
Original Research: Pulmonary Vascular DiseasePulmonary Arterial Hypertension in the Southern Hemisphere
Section snippets
Materials and Methods
From January 2008 to December 2013, a total of 178 consecutive patients with PAH, diagnosed according to the Nice classification,15 were enrolled in this study. PAH diagnosis was established in accordance with current international guidelines.4 The date of the first right-sided heart catheterization was defined as the date of diagnosis.9 Other causes of pulmonary hypertension were excluded by echocardiography, pulmonary function testing, chest CT scans, and ventilation-perfusion scans. The
Results
A total of 178 patients with PAH were included in the study. Figure 1 shows the proportion of the patients in each PAH subgroup. IPAH, connective tissue disease (CTD), and schistosomiasis-associated PAH (Sch-PAH) represented the most prevalent subgroups, accounting for 28.7%, 25.8%, and 19.7% of all cases, respectively. From the 46 patients with CTD-PAH, 23 patients had systemic sclerosis, 14 patients had systemic lupus erythematosus, five patients had mixed CTD, and the remaining four patients
Discussion
To our knowledge, the present study represents the first prospective registry including only incident cases of PAH in Brazil. Data coming from the Southern Hemisphere are scarce, making it difficult to compare these data directly to the published registries from the United States and Europe.5, 6, 7, 8, 9, 10, 12, 14, 16, 17 Our study allowed the comparison not only in terms of baseline characteristics but also in terms of long-term prognosis.
Our study confirmed that schistosomiasis is an
Acknowledgments
Author contributions: R. S. takes full responsibility for the whole content of the manuscript. J. L. A., F. G., C. J., C. J. C. d. S. F., L. T. K. M., B. D., and S. H. collected the data; J. L. A., F. G., M. H., and R. S. analyzed the data and drafted the manuscript; and M. H. and R. S. planned the study.
Financial/nonfinancial disclosures: The authors have reported to CHEST the following conflicts of interest: Dr Souza has received lecture fees and/or has participated in advisory boards for
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FUNDING/SUPPORT: The authors have reported to CHEST that no funding was received for this study.
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