Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal

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

doi:10.1016/j.repc.2018.02.009

Cited by 14 publications

(7 citation statements)

References 25 publications

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“…From the results of the adjusted HR calculation for IPAH, significantly higher HRs were apparent for older age 6 10 14 15 and male patients. 6 8 14 Because mean age between different sex group showed significant differences, we analyzed HR of sex after age group adjusted.…”

Section: Discussionmentioning

confidence: 99%

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea

et al. 2022

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Background Few studies used nationwide data to assess the survival rates (SRs) and death risk for idiopathic pulmonary arterial hypertension (IPAH; ICD-10 I27.0) in Korea. Methods IPAH data (N = 9,017; female:male = 6:4) were collected from the National Health Insurance Service in Korea, from 2006 through 2017. The data consisted of primary diagnoses related to IPAH. The Kaplan–Meier method and Cox proportional-hazards analyses were carried out. Results The mean age was 62.3 (± 19.4) years, 64.2 (± 18.9) years in female and 59.4 (± 19.8) years in male ( P < 0.001). The one-, three-, five- and 10-year SRs for IPAH were 89.0%, 79.8%, 72.3% and 57.0%, respectively. The adjusted hazard ratio (HR) of IPAH was 1.81 (95% confidence interval [CI], 1.26–2.59) in 60–69 age group, 3.42 (95% CI, 2.40–4.87) in 70–79, and 7.73 (95% CI, 5.43–11.0) in 80s. Other risk factors were male, low-income status, diabetes, myocardial infarction, atrial fibrillation, ischemic stroke, hemorrhagic stroke, and malignant neoplasm. Conclusion The 10-year SR of IPAH was 57% in Korea. The HR for IPAH was significantly high in patients with older age and other risk factors.

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Section: Discussionmentioning

confidence: 99%

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea

et al. 2022

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“…We found a female-to-male ratio of 2.3 in the total group of PAH patients and 2.5 in the group of patients with IPAH/HPAH, figures similar to those found in most other PAH registries in the Western Europe. In the French and Swedish registry, this ratio was 1.9 and in the Portuguese registry 2.4 [27]. Interestingly, the female predominance was significantly lower in PAH patients enrolled to the COMPERA study, in which the female-to-male ratio was 1.5 [26].…”

Section: Age and Sex Distributionmentioning

confidence: 93%

Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

Kopeć

Kurzyna²,

Mroczek

et al. 2020

JCM

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Current knowledge of pulmonary arterial hypertension (PAH) epidemiology is based mainly on data from Western populations, and therefore we aimed to characterize a large group of Caucasian PAH adults of Central-Eastern European origin. We analyzed data of incident and prevalent PAH adults enrolled in a prospective national registry involving all Polish PAH centers. The estimated prevalence and annual incidence of PAH were 30.8/mln adults and 5.2/mln adults, respectively and they were the highest in females ≥65 years old. The most frequent type of PAH was idiopathic (n = 444; 46%) followed by PAH associated with congenital heart diseases (CHD-PAH, n = 356; 36.7%), and PAH associated with connective tissue disease (CTD-PAH, n = 132; 13.6%). At enrollment, most incident cases (71.9%) were at intermediate mortality risk and the prevalent cases had most of their risk factors in the intermediate or high risk range. The use of triple combination therapy was rare (4.7%). A high prevalence of PAH among older population confirms the changing demographics of PAH found in the Western countries. In contrast, we found: a female predominance across all age groups, a high proportion of patients with CHD-PAH as compared to patients with CTD-PAH and a low use of triple combination therapy.

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“…The group of CTEPH patients was well characterized in a large prospective international registry which enrolled patients between February 2007 and January 2009. 7 As the phenotype of CTEPH patients may be population dependent, 8 several national reports as summarized in Table 1 7,9–29 were of added value. Most of them, however, recruited patients before the marketing authorization of riociguat by the European Medicines Agency through the European Union (27/03/2014), and before the first experience with balloon pulmonary angioplasty (BPA) outside of Japan.…”

Section: Introductionmentioning

confidence: 99%

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

Kopeć

Dzikowska-Diduch

Mroczek

et al. 2021

Therapeutic Advances in Chronic Disease

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Background: Significant achievements in the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) have provided effective therapeutic options for most patients. However, the true impact of the changed landscape of CTEPH therapies on patients’ management and outcomes is poorly known. We aimed to characterize the incidence, clinical characteristics, and outcomes of CTEPH patients in the modern era of CTEPH therapies. Methods: We analyzed the data of CTEPH adults enrolled in the prospective multicenter registry. Results: We enrolled 516 patients aged 63.8 ± 15.4 years. The incidence rate of CTEPH was 3.96 per million adults per year. The group was burdened with several comorbidities. New oral anticoagulants ( n = 301; 58.3%) were preferred over vitamin K antagonists ( n = 159; 30.8%). Pulmonary endarterectomy (PEA) was performed in 120 (23.3%) patients and balloon pulmonary angioplasty (BPA) in 258 (50%) patients. PEA was pretreated with targeted pharmacotherapy in 19 (15.8%) patients, and BPA in 124 (48.1%) patients. Persistent CTEPH was present in 46% of PEA patients and in 65% of patients after completion of BPA. Persistent CTEPH after PEA was treated with targeted pharmacotherapy in 72% and with BPA in 27.7% of patients. At a mean time period of 14.3 ± 5.8 months, 26 patients had died. The use of PEA or BPA was associated with better survival than the use of solely medical treatment. Conclusions: The modern population of CTEPH patients comprises mostly elderly people significantly burdened with comorbid conditions. This calls for treatment decisions that are tailored individually for every patient. The combination of two or three methods is currently a frequent approach in the treatment of CTEPH. Clinical Trial Registration: clinicaltrials.gov/ct2/show/NCT03959748

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Long-term survival in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: Insights from a referral center in Portugal

Abstract: Our data indicate a trend toward improved survival over time of CTD-PAH and CTEPH patients treated at a Portuguese referral PH center. Earlier diagnosis, increasing use of parenteral prostanoids, and surgical treatment may further improve PH prognosis.

Cited by 14 publications

References 25 publications

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea

A Retrospective Population-Based Survival Study of Idiopathic Pulmonary Arterial Hypertension in Korea

Characterization of Patients with Pulmonary Arterial Hypertension: Data from the Polish Registry of Pulmonary Hypertension (BNP-PL)

Characteristics and outcomes of patients with chronic thromboembolic pulmonary hypertension in the era of modern therapeutic approaches: data from the Polish multicenter registry (BNP-PL)

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