Pulmonary Hypertension in Portugal: First Data from a Nationwide Registry

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.

Gender disparities in pulmonary hypertension at a tertiary centre in Cameroon

“…This is similar to reports from Nigeria, [12] Australia [5] and Portugal, [21] where most patients present with features of advanced HF. This late presentation is probably due to nonspecific symptoms of PH in the early stages, as well as limited availability of diagnostic tools.…”

“…[20] The mean age was, however, higher than that reported from a Portuguese PH registry. [21] While relatively younger age appears to be a striking feature of our cohort of SSA patients with PH, it should be noted that it is also a common finding in patients with HF, with relatively young patients being affected (median age 55 years). [22] One likely explanation is the over-representation of group 2 PH caused by left heart disease in our cohort, whether as a result of left HF or valvular heart disease, which usually occurs at a younger age.…”

Pulmonary arterial hypertension in a multi‐ethnic Asian population: Characteristics, survival and mortality predictors from a 14‐year follow‐up study

“…Most patients presented before symptomology was advanced. Only 32.4% of patients were in WHO Class III or IV, compared with between 69% and 75% in the Spanish, Portuguese, French and REVEAL registries . Early identification of disease is likely due to an efficient referral system and accessibility to tertiary health care in a small country.…”

“…While there is a wealth of data generated from large PAH registries in the United States and Europe, data from Asia, especially South‐East Asia, are sorely lacking. Woefully, it is also in this part of the world where accessibility and affordability of PAH‐specific therapy is an issue.…”

Medical management of chronic thromboembolic pulmonary hypertension

“…Single centres and registries have described the management of CTEPH: examples are summarised in table 3 [10,[43][44][45][46][47][48]. The patients reported may be postoperative, technically inoperable, those who refuse surgery and those with operable disease but who are not suitable for PEA because of comorbid conditions.…”