Chest
Volume 147, Issue 2, February 2015, Pages 495-501
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Original Research: Pulmonary Vascular Disease
Pulmonary Arterial Hypertension in the Southern Hemisphere

https://doi.org/10.1378/chest.14-1036Get rights and content

BACKGROUND

Pulmonary arterial hypertension (PAH) is a rare and ultimately fatal disorder of the pulmonary vasculature. There is increasing interest in the worldwide characteristics of patients with PAH, although data coming from the Southern Hemisphere remain scarce. The objective of this study was to describe a cohort of incident patients with PAH from a large reference center in Brazil.

METHODS

: All consecutive patients who received a diagnosis of PAH by right-sided heart catheterization between 2008 and 2013 were included in the study.

RESULTS

: A total of 178 patients with newly diagnosed PAH were enrolled in the study (mean age, 46 years; female/male ratio, 3.3:1; 45.5% in New York Heart Association functional class III or IV). Idiopathic PAH (IPAH), connective tissue disease (CTD), and schistosomiasis-associated PAH (Sch-PAH) accounted for 28.7%, 25.8%, and 19.7% of all cases, respectively. The patients were treated with phosphodiesterase type 5 inhibitors (66%), endothelin receptor antagonists (27%), or a combination of both (5%). For the PAH group as a whole, the estimated survival rate 3 years after diagnosis was 73.9%. The prognosis for the patients with CTD was worse than that for the patients with IPAH and Sch-PAH (P = .03).

CONCLUSIONS

: The distribution of PAH causes and the baseline characteristics in our registry clearly differ from the previously published European and US-based registries. These differences highlight the importance of regional registries and also raise questions regarding the need to better account for such differences in future clinical trials.

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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