Chest
Volume 141, Issue 2, February 2012, Pages 363-373
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Original Research
Pulmonary Vascular Disease
Sex Differences in the Diagnosis, Treatment, and Outcome of Patients With Pulmonary Arterial Hypertension Enrolled in the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management

https://doi.org/10.1378/chest.10-3114Get rights and content

Background

Pulmonary arterial hypertension (PAH) is a life-threatening disease that affects more women than men. The reasons for the female preponderance are unclear, and there are limited data available for men with PAH.

Methods

Data from the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) were analyzed to explore sex differences among patients with PAH with regard to 2-year survival from enrollment and 5-year survival from time of diagnosis.

Results

The data set included 2,318 women and 651 men. More women had PAH associated with connective tissue disease (P < .001), and more men had portopulmonary hypertension (P < .001) and HIV-associated PAH (P < .001). More women had congenital heart disease-associated PAH (P = .017), thyroid disease (P < .001), and depression reported (P ≤ .001). At diagnosis, men had higher mean pulmonary artery pressure (53 ± 14 vs 51 ± 14.3 mm Hg; P = .013) and mean right atrial pressure (10 ± 6 vs 9 ± 6 mm Hg; P = .031). Women had better survival estimates for 2 years from enrollment and for 5 years from diagnosis. Stratifying by age showed that survival from enrollment was similar between men and women aged < 60 years at enrollment, whereas men aged ≥ 60 years have lower survival rates compared with women aged ≥ 60 years.

Conclusions

Our findings highlight similarities and differences between men and women with PAH, raising questions for future exploration regarding the role of hormones and sex in causation and survival in PAH.

Trial registry

ClinicalTrials.gov; No.: NCT00370214; URL: www.clinicaltrials.gov

Section snippets

Study Design and Population

The REVEAL Registry design and baseline characteristics have been previously described.7, 8 REVEAL Registry is a 55-center (university-affiliated and community hospitals), observational, prospective, US-based study of approximately 3,500 patients with newly and previously diagnosed PAH (group 1 pulmonary hypertension) aged ≥ 3 months at diagnosis. Patients were consecutively enrolled from March 2006 through September 11, 2007 and are followed for at least 5 years from time of enrollment. The

Patient Characteristics

Of the 2,969 patients meeting the analysis criteria, 2,318 (78%) were women. The mean ± SD age at enrollment was 49 ± 18 years for men and 51 ± 16 years for women. Demographic characteristics are provided in Table 1. The percentage of patients with IPAH, FPAH, and APAH was similar for males and females. There were no significant sex differences in cause at diagnosis among patients with IPAH (P = .85), FPAH (P = .23), or APAH. (P = .71). However, among APAH subtypes, more women had CTD (P <

Discussion

Consistent with other PAH registries, the majority of patients in the REVEAL Registry are female. Both IPAH and APAH had similar proportions of men and women. However, among the APAH subgroups, there were clear differences. More men have APAH due to HIV or PoPH, and more women have CTD-APAH and CHD-APAH. More men reported using or having used recreational drugs, whereas the use of appetite suppressants has a female predominance. Reported depression, obesity, and thyroid disorders appeared as

Conclusions

The size of the REVEAL Registry afforded us an opportunity to look at male patients with PAH and compare them to females with PAH. Our findings highlight the similarities and differences between sexes and raise questions for future exploration into the role of hormones in causation and survival in PAH. The data also provide reassurance that the diagnosis and treatment of PAH is similar between men and women.

Acknowledgments

Author contributions: Dr Shapiro: contributed to the study design; collection, analysis, and interpretation of data; drafting and critical review of the manuscript; and has seen and approved the final version.

Ms Traiger: contributed to the study design; collection, analysis, and interpretation of data; drafting and critical review of the manuscript; and has seen and approved the final version.

Ms Turner: contributed to the study design; collection, analysis, and interpretation of data; drafting

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Funding/Support: Funding for the REVEAL Registry is provided by Actelion Pharmaceuticals US, Inc.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestpubs.org/site/misc/reprints.xhtml).

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