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Pulmonary hypertension in Portugal: first data from a nationwide registry.

Baptista R, Meireles J, Agapito A, Castro G, da Silva AM, Shiang T, Gonçalves F, Robalo-Martins S, Nunes-Diogo A, Reis A - Biomed Res Int (2013)

Bottom Line: PAH patients had a mean age of 43.4 ± 16.4 years.Idiopathic PAH was the most common etiology (37%).Clinical presentation and outcomes are comparable with those reported on other national registries.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, 3000 Coimbra, Portugal ; Institute for Biomedical Imaging and Life Sciences, Faculty of Medicine of University of Coimbra, Azinhaga de Santa Comba, 3000 Coimbra, Portugal.

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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Related in: MedlinePlus

One-year survival in pulmonary arterial hypertension (a) and chronic thromboembolic pulmonary hypertension (b) patients.
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fig3: One-year survival in pulmonary arterial hypertension (a) and chronic thromboembolic pulmonary hypertension (b) patients.

Mentions: Survival data was available for all patients (Figure 3). One year after the diagnostic RHC, 5 patients were deceased. The Kaplan-Meier survival estimates for patients with PAH and CTEPH at 1 year were 93.5% and 93.9%, respectively (Log-rank P = 0.709). Unoperated CTEPH patients had a one-year survival rate of 92.9%, whereas all patients that underwent PEA survived.


Pulmonary hypertension in Portugal: first data from a nationwide registry.

Baptista R, Meireles J, Agapito A, Castro G, da Silva AM, Shiang T, Gonçalves F, Robalo-Martins S, Nunes-Diogo A, Reis A - Biomed Res Int (2013)

One-year survival in pulmonary arterial hypertension (a) and chronic thromboembolic pulmonary hypertension (b) patients.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3818811&req=5

fig3: One-year survival in pulmonary arterial hypertension (a) and chronic thromboembolic pulmonary hypertension (b) patients.
Mentions: Survival data was available for all patients (Figure 3). One year after the diagnostic RHC, 5 patients were deceased. The Kaplan-Meier survival estimates for patients with PAH and CTEPH at 1 year were 93.5% and 93.9%, respectively (Log-rank P = 0.709). Unoperated CTEPH patients had a one-year survival rate of 92.9%, whereas all patients that underwent PEA survived.

Bottom Line: PAH patients had a mean age of 43.4 ± 16.4 years.Idiopathic PAH was the most common etiology (37%).Clinical presentation and outcomes are comparable with those reported on other national registries.

View Article: PubMed Central - PubMed

Affiliation: Department of Cardiology, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Praceta Mota Pinto, 3000 Coimbra, Portugal ; Institute for Biomedical Imaging and Life Sciences, Faculty of Medicine of University of Coimbra, Azinhaga de Santa Comba, 3000 Coimbra, Portugal.

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.

Show MeSH
Related in: MedlinePlus