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Multicenter Study
. 2013:2013:489574.
doi: 10.1155/2013/489574. Epub 2013 Oct 21.

Pulmonary hypertension in Portugal: first data from a nationwide registry

Rui Baptista  1 , José Meireles, Ana Agapito, Graça Castro, António Marinho da Silva, Teresa Shiang, Fabienne Gonçalves, Susana Robalo-Martins, António Nunes-Diogo, Abílio Reis
Affiliations
Multicenter Study

Pulmonary hypertension in Portugal: first data from a nationwide registry

Rui Baptista et al. Biomed Res Int. 2013.

Abstract

Introduction: Pulmonary arterial hypertension (PAH) is a rare disease that must be managed in specialized centers; therefore, the availability of epidemiological national data is critical.

Methods: We conducted a prospective, observational, and multicenter registry with a joint collaboration from five centers from Portugal and included adult incident patients with PAH or chronic thromboembolic pulmonary hypertension (CTEPH).

Results: Of the 79 patients enrolled in this study, 46 (58.2%) were classified as PAH and 33 patients (41.8%) as CTEPH. PAH patients had a mean age of 43.4 ± 16.4 years. Idiopathic PAH was the most common etiology (37%). At presentation, PAH patients had elevated right atrial pressure (RAP) (7.7 ± 5.9 mmHg) and mean pulmonary vascular resistance (11.4 ± 6.5 Wood units), with a low cardiac index (2.7 ± 1.1 L·min(-1)·m(-2)); no patient was under selective pulmonary vasodilators; however, at follow-up, most patients were on single (50%), double (28%), or triple (9%) combination vasodilator therapy. One-year survival was 93.5%, similar to CTEPH patients (93.9%), that were older (60.0 ± 12.5 years) and had higher RAP (11.0 ± 5.2 mmHg, P = 0.015).

Conclusions: We describe for the first time nationwide data on the diagnosis, management, and prognosis of PAH and CTEPH patients in Portugal. Clinical presentation and outcomes are comparable with those reported on other national registries.

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Figures

Figure 1
Figure 1
Patient selection flowchart.
Figure 2
Figure 2
Distribution of age and gender.
Figure 3
Figure 3
One-year survival in pulmonary arterial hypertension (a) and chronic thromboembolic pulmonary hypertension (b) patients.
See this image and copyright information in PMC

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