Pulmonary hypertension: Real-world data from a Portuguese expert referral centre

Gomes, A.; Cruz, Célia; Rocha, Jaime; Ricardo, Miguel; Vicente, Margarida; Melo, Alzira; Santos, Mário; Carvalho, L.; Gonçalves, Fabienne; Reis, Abílio

doi:10.1016/j.pulmoe.2018.02.003

Cited by 8 publications

(12 citation statements)

References 35 publications

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“…The estimated 1-year survival in this cohort of CTEPH patients was 96.6%, which is high compared to other previous studies of the CTEPH population (including the recently published international registry of CTEPH) [6,[16][17][18][19], which did not appear to be driven by survivor bias in prevalent cases (in fact, prevalent cases had lower estimated survival). Nonetheless, there are other smaller studies that show comparable 1-year survival estimates and, importantly, show comparable disease characteristics such as disease severity measured by WHO FC [20].…”

Section: Discussioncontrasting

confidence: 49%

Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry

et al. 2020

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BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive vascular disease with poor prognosis if left untreated. This study aims to assess the patient characteristics, treatment approach and clinical and survival outcomes for CTEPH patients enrolled in the Systematic Prospective Follow Up for Better Understanding of Clinical Characteristics of Patients with Pulmonary Hypertension Disease (SAUDIPH) registry.MethodsThis study presents a subanalysis of CTEPH patients enrolled in the SAUDIPH registry. This registry enrolled patients with pulmonary hypertension, established through right heart catheterisation, under clinical management at a specialised tertiary care centre. Patients received standard care during the period of the registry.ResultsAt the time of this analysis, 64 CTEPH patients were enrolled in the registry. Mean age at diagnosis was 39.7 years and there was a female predominance (67.6%). At baseline, most patients were in World Health Organization functional classes III or IV (70.1%). At the last follow-up visit, most patients (63.2%) had undergone endarterectomy, showing significant improvement in disease severity from baseline. Patients who underwent endarterectomy showed numerically higher (p=0.126) probability of survival at 1 year (97.5%) versus those who did not undergo endarterectomy (94.4%).ConclusionPatients were diagnosed at relatively young age, but still showed high disease severity, suggesting delay in diagnosis. Patients who underwent surgical treatment showed substantial improvements in clinical and haemodynamic parameters, while the remaining patients tended to show disease progression. The 96.6% 1-year cumulative probability of survival was high compared to previous studies.

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Section: Discussioncontrasting

confidence: 49%

Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry

et al. 2020

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“…[21][22][23] However, in terms of other PAH etiologies the percentage is lower than in most other registries and is probably related to low overall physician awareness of PAH in these patients. 8,9,13,18,19,24 Furthermore, in accordance with other PAH cohorts, dyspnea and fatigue were the most common symptoms at baseline. However, our population had more favorable clinical characteristics at diagnosis, since half of patients were in WHO II FC ( Table 5).…”

Section: Discussionsupporting

confidence: 78%

“…21–23 However, in terms of other PAH etiologies the percentage is lower than in most other registries and is probably related to low overall physician awareness of PAH in these patients. 8,9,13,18,19,24…”

Section: Discussionmentioning

confidence: 99%

“…Despite major advances in pharmacotherapy of PAH in the last 15 years, 6 we still lack thorough knowledge about the pathophysiology and the epidemiology of PAH. Although large national and international registries 7–15 have so far provided useful information on the epidemiology, the diagnosis, and management of patients with PAH, regional differences certainly exist regarding patients' characteristics, diagnostic workup, and treatment availability. Moreover, since the registries include a mix of PAH patients with advanced age and various comorbidities, there is a scientific need for more epidemiological data from different countries, with different healthcare organization and financial background, in order to obtain a more comprehensive view of PAH worldwide.…”

Section: Introductionmentioning

confidence: 99%

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Epidemiology and initial management of pulmonary arterial hypertension: real‐world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE)

Arvanitaki

Boutsikou²,

Anthi

et al. 2019

Pulm. circ.

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Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.

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“…Some previous studies also included prevalent cases but were conducted earlier (closing enrolment in 2008). [8][9][10]17,26 More recent studies show comparable disease severity at diagnosis, 12,13,18,20,22,27 reflecting the impact of better referral networks established over the years. In the last follow-up visit, patients were more likely to be in WHO FC I/II, indicating a reduction in disease severity.…”

Section: Tablementioning

confidence: 99%

Pulmonary hypertension in Saudi Arabia: First data from the SAUDIPH registry with a focus on pulmonary arterial hypertension

et al. 2020

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Background and objective This study presents the first results of ‘SAUDIPH’ registry, aiming to assess patient characteristics, treatment approach and clinical and survival outcomes in patients with PAH. Methods The registry enrolled patients with Group 1 and Group 4 PH under clinical management in a specialized tertiary care centre from 2004 to 2018. Changes from baseline to last follow‐up visit were assessed. Results A total of 222 patients were enrolled, and Group 1 PH was the most frequent aetiology (57.7%). Mean age at diagnosis was 32 years. mPAP was 55.0 mm Hg and was higher for Group 1 PH (59.0 mm Hg, P < 0.001). At the last visit, most patients were on specific therapy (83.7%) and 30% shifted from FC III/IV to FC I/II. NT‐proBNP improved by 29.2% in the overall population. The 1‐, 3‐ and 5‐year cumulative probabilities of survival were 95.6% (95% CI: 91.5–99.9%), 89.2% (95% CI: 82.1–96.9%) and 74.6% (95% CI: 59.4–93.7%), respectively. CHD‐PAH demonstrated the best survival among Group 1 PAH with 1‐, 3‐ and 5‐year cumulative probability of 100%, 100%, and 75.0% (95% CI: 42.6–100), respectively. Conclusion PAH was the most frequent aetiology and patients were younger at diagnosis compared to other cohorts. Most patients showed improvement in FC and NT‐proBNP. The estimated 1‐year survival was better than previous studies, possibly reflecting wider use of combination therapy and the high prevalence of CHD‐PAH.

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Pulmonary hypertension: Real-world data from a Portuguese expert referral centre

Cited by 8 publications

References 35 publications

Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry

Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry

Epidemiology and initial management of pulmonary arterial hypertension: real‐world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE)

Pulmonary hypertension in Saudi Arabia: First data from the SAUDIPH registry with a focus on pulmonary arterial hypertension

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