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. 2023 Jan 1;13(1):e12193.
doi: 10.1002/pul2.12193. eCollection 2023 Jan.

Risk assessment validation in patients with pulmonary arterial hypertension: Data from a Southern Brazil registry (RESPHIRAR study)

Fernanda Brum Spilimbergo  1 , Roger Pirath Rodrigues  2 , Marcelo Credidio Dias-Pinto  3 , Daniela Cavalet Blanco  4 , Gláucia Maria Barbieri  5 , Marina Andrade-Lima  6 , Ariovaldo Leal Fagundes  7 , Marcelo Basso Gazzana  8 , Gabriela Roncato  1 , Marcelo Martins Mello  1 , Guilherme Watte  1 , Taís Silveira Assmann  1 , Cássia Ferreira Braz Caurio  1 , Rogerio Souza  9 , Gisela Martina Bohns Meyer  1
Affiliations

Risk assessment validation in patients with pulmonary arterial hypertension: Data from a Southern Brazil registry (RESPHIRAR study)

Fernanda Brum Spilimbergo et al. Pulm Circ. .

Abstract

Pulmonary arterial hypertension (PAH) is a severe and progressive disease characterized by increased pulmonary vascular resistance, ultimately leading to right heart failure and death. Registries are a valuable tool in the research of rare conditions such as PAH. Moreover, the risk assessment strategy has been validated in European and North American registries and has been reported to provide an accurate prediction of mortality and the clinical advantage of reaching low-risk status. However, there is no available data from Brazil. Thus, the aim of the present study was to describe the characteristics of a sample of PAH from Southern Brazil and to retrospectively validate the risk assessment at our population. The RESPHIRAR is a retrospective and multicentric registry on pulmonary hypertension. With a join collaboration from nine centers in Southern Brazil, demographics, clinical presentation, and hemodynamics data of PAH were collected between 2007 and 2017. Moreover, the REVEAL 2.0 and REVEAL 2.0 Lite risk assessments were validated in our population. Overall, 370 PAH patients were included in the present study. Patients were predominantly female (78.5%) and had a mean age of 41.8 ± 18.8 years. Most patients (33.4%) had idiopathic PAH, 30.2% had PAH associated with congenital heart disease, and 23.5% had PAH associated with connective tissue disease. The low-risk group showed significantly lower mortality than the intermediated- or high-risk group at diagnosis (p < 0.05). In conclusion, our data suggest that REVEAL 2.0 and REVEAL 2.0 Lite risk assessments can predict mortality risk in PAH patients in Southern Brazil.

Keywords: Southern Brazil registry; pulmonary arterial hypertension; risk assessment.

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Conflict of interest statement

Gláucia Maria Barbieri reports speaker and sponsorships in scientific events by Janssen and Bayer. Ariovaldo Leal Fagundes reports lectures to GlaxoSmithKline, Pfizer, and AstraZeneca. Scientific consultancy fee from Ache, Boehringer Ingelheim, and Chiesi. Roger Pirath Rodrigues reports lectures to Janssen, Bayer, Astra‐Zeneca, GSK. Rogerio Souza reports lecture and consultancy fees from Acceleron, Bayer, and Merck. Gisela Martina Bohns Meyer reports lecture and consultancy fees from Aceeleron, Bayer, and GSK. The remaining authors declare no conflict of interest. Conflict of interest outside the submitted work.

Figures

Figure 1
Figure 1
Characteristics of PAH sample at baseline. (a) Common signals and symptoms and (b) common comorbidities. PAH, pulmonary arterial hypertension.
Figure 2
Figure 2
Kaplan−Meier estimated of 5‐year survival from data of diagnosis in PAH patients. (a) Overall PAH population. (b) PAH subgroups. PAH, pulmonary arterial hypertension.
Figure 3
Figure 3
Kaplan−Meier estimated of 5‐year survival from data of diagnosis in PAH patients. (a) REVEAL Lite 2.0. (b) REVEAL 2.0. PAH, pulmonary arterial hypertension.
See this image and copyright information in PMC

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