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Disease characteristics and clinical outcome over two decades from the Swiss pulmonary hypertension registry.

ABSTRACT: Pulmonary hypertension (PH), especially pulmonary arterial and chronic thromboembolic pulmonary hypertension (PAH/CTEPH), are rare and progressive conditions. Despite recent advances in treatment and prognosis, PH is still associated with impaired quality of life and survival. Long-term PH-registry data provide information on the changing PH-epidemiology and may help to direct resources to patient's needs. This retrospective analysis of the Swiss Pulmonary Hypertension Registry includes patients newly diagnosed with PH (mainly PAH/CTEPH) registered from January 2001 to June 2019 at 13 Swiss hospitals. Patient characteristics (age, body mass index, gender, diagnosis), hemodynamics at baseline, treatment, days of follow-up, and events (death, transplantation, pulmonary endarterectomy, or loss to follow-up) at last visit were analyzed. Patients were stratified into four time periods according to their date of diagnosis. Survival was analyzed overall and separately for PAH/CTEPH and time periods. 1427 PH patients were included (thereof 560 PAH, 383 CTEPH). Over the years, age at baseline (mean ± SD) significantly increased from 59 ± 14 years in 2001-2005 to 66 ± 14 years in 2016-2019 (p < 0.001) while the gender distribution tended toward equality. Mean pulmonary artery pressure and pulmonary vascular resistance significantly decreased over time (from 46 ± 15 to 41 ± 11 mmHg, respectively, 9 ± 5 to 7 ± 4 WU, p < 0.001). Three-year survival substantially increased over consecutive periods from 69% to 91% (for PAH 63%-95%, for CTEPH 86%-93%) and was poorer in PAH than CTEPH independently of time period (p < 0.001). Most patients were treated with mono- or combination therapy and an increasing number of CTEPH underwent pulmonary endarterectomy (40% 2016-2019 vs. 15% 2001-2005). This long-term PH registry reveals that over two decades of observation, newly diagnosed patients are older, less predominantly female, have less impaired hemodynamics and a better survival.

SUBMITTER: Appenzeller P

PROVIDER: S-EPMC9052988 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature

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Disease characteristics and clinical outcome over two decades from the Swiss pulmonary hypertension registry.

Appenzeller Paula P Lichtblau Mona M Berlier Charlotte C Aubert John-David JD Azzola Andrea A Fellrath Jean-Marc JM Geiser Thomas T Lador Frederic F Pohle Susanne S Opitz Isabelle I Schwerzmann Markus M Stricker Hans H Tamm Michael M Saxer Stéphanie S Ulrich Silvia S

Pulmonary circulation 20220105 1

Pulmonary hypertension (PH), especially pulmonary arterial and chronic thromboembolic pulmonary hypertension (PAH/CTEPH), are rare and progressive conditions. Despite recent advances in treatment and prognosis, PH is still associated with impaired quality of life and survival. Long-term PH-registry data provide information on the changing PH-epidemiology and may help to direct resources to patient's needs. This retrospective analysis of the Swiss Pulmonary Hypertension Registry includes patients ...[more]

PMID: 35506112

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Project description:BackgroundThe treatment, genotyping, and phenotyping of patients with World Health Organization Group 1 pulmonary arterial hypertension (PAH) have evolved dramatically in the last decade.Research questionThe United States Pulmonary Hypertension Scientific Registry was established as the first US PAH patient registry to investigate genetic information, reproductive histories, and environmental exposure data in a contemporary patient population.Study design and methodsInvestigators at 15 US centers enrolled consecutively screened adults diagnosed with Group 1 PAH who had enrolled in the National Biological Sample and Data Repository for PAH (PAH Biobank) within 5 years of a cardiac catheterization demonstrating qualifying hemodynamic criteria. Exposure and reproductive histories were collected by using a structured interview and questionnaire. The biobank provided genetic data.ResultsBetween 2015 and 2018, a total of 499 of 979 eligible patients with clinical diagnoses of idiopathic PAH (IPAH) or familial PAH (n = 240 [48%]), associated PAH (APAH; n = 256 [51%]), or pulmonary venoocclusive disease/pulmonary capillary hemangiomatosis (n = 3 [1%]) enrolled. The mean age was 55.8 years, average BMI was 29.2 kg/m2, and 79% were women. Mean duration between symptom onset and diagnostic catheterization was 1.9 years. Sixty-six percent of patients were treated with more than one PAH medication at enrollment. Past use of prescription weight loss drugs (16%), recreational drugs (27%), and oral contraceptive pills (77%) was common. Women often reported miscarriage (37%), although PAH was rarely diagnosed within 6 months of pregnancy (1.9%). Results of genetic testing identified pathogenic or suspected pathogenic variants in 13% of patients, reclassifying 18% of IPAH patients and 5% of APAH patients to heritable PAH.InterpretationPatients with Group 1 PAH remain predominately middle-aged women diagnosed with IPAH or APAH. Delays in diagnosis of PAH persist. Treatment with combinations of PAH-targeted medications is more common than in the past. Women often report pregnancy complications, as well as exposure to anorexigens, oral contraceptives, and/or recreational drugs. Results of genetic tests frequently identify unsuspected heritable PAH.

Project description:Background: Hypertension is a leading cause of cardiovascular-related morbidity and mortality. Elevated blood pressure (BP) in children is related to long-term adverse health effects. Until recently, few studies have reported the secular trend and associated factors of hypertension phenotypes in Chinese children and adolescents. Methods: From the China Health and Nutrition Survey (CHNS) 1991-2015, a total of 15,143 records of children aged 7-17 years old were included. Following definitions of hypertension from the Chinese Child Blood Pressure References Collaborative Group, we estimated the prevalence of prehypertension, hypertension, stage 1 hypertension, stage 2 hypertension and its phenotypes (ISH, isolated systolic hypertension; IDH, isolated diastolic hypertension; SDH, combined systolic and diastolic hypertension). General estimation equation was used to analyze the trends in the prevalence of hypertension and hypertension phenotypes, and a multivariable logistic regression was constructed to explore the associated factors. Results: During 1991-2015, increasing trends were revealed in BP and hypertension prevalence (P < 0.001) in Chinese children and adolescents. For ISH, IDH and SDH, the age-standardized prevalence increased dramatically from 0.9 to 2.2%, from 6.2 to 14.1%, and from 1.4 to 2.9%, respectively (all P < 0.001). Adolescents aged 13-17 years (OR = 1.76, 95% CI: 1.56-1.97, P < 0.001), general obesity (OR = 2.69, 95% CI: 2.10-3.44, P < 0.001) and central obesity (OR = 1.49, 95% CI: 1.21-1.83, P < 0.001) were positively associated with hypertension, whereas the South region (OR = 0.65, 95% CI: 0.58-0.73, P < 0.001) was a negative factor. Furthermore, body mass index (BMI) and general obesity were linked to the presence of ISH, IDH and SDH. Age, waist circumference (WC) and central obesity were additionally associated with ISH, and sex, age, urban/rural setting, North/South region, WC and central obesity were additionally associated with IDH. Conclusion: BP levels and prevalence of hypertension and phenotypes increased dramatically in Chinese children and adolescents from 1991 to 2015. Regional discrepancy, demographic features, BMI, WC and overweight/obesity status were associated factors of hypertension among youths.

Project description:IntroductionThe predictive value of the risk stratification scales in elderly patients with IPAH might differ from that in younger patients. It is unknown whether young and older IPAH patients have the same survival dependence on PAH-specific therapy numbers. The aim of this study was to evaluate the prognostic relevance of risk stratification scales and PAH medication numbers in elderly IPAH patients in comparison with young IPAH patients.Materials and methodsA total of 119 patients from a prospective single-center PAH registry were divided into group I < 60 years old (n = 89) and group II ≥ 60 years old (n = 30). ESC/ERS, REVEAL, and REVEAL 2.0 risk stratification scores were assessed at baseline, as well as H2FpEF score and survival at follow-up.ResultsDuring a mean follow-up period of 2.9 years (1.63; 6.0), 42 (35.3%) patients died; at 1, 2, 3, 5, 7, and 10 years, survival was 95%, 88.6%, 78.5%, 61.7%, 48.5%, and 33.7%, respectively. No survival differences were observed between the two groups, despite the use of monotherapy in the elderly patients. The best predictive REVEAL value in elderly patients (IPAH patients ≥ 60 years) was AUC 0.73 (0.56-0.91), p = 0.03; and in patients with LHD comorbidities in the entire cohort, it was AUC 0.73 (0.59-0.87), p < 0.009. Factors independently associated with death in the entire cohort were CKD (p = 0.01, HR 0.2), the right-to-left ventricle dimension ratio (p = 0.0047, HR 5.97), and NT-proBNP > 1400 pg/mL (p = 0.008, HR 3.18).ConclusionRisk stratification in the elderly IPAH patients requires a fundamentally different approach than that of younger patients, taking into account the initial limitations in physical performance and comorbidities that interfere with current assessment scores. The REVEAL score reliably stratifies patients at any age and LHD comorbidities. The initial monotherapy seems to be reasonable in patients over 60 years. Selection tools for initial combination PAH therapy in older IPAH patients with comorbidities need to be validated in prospective observational studies.

Project description:Acupuncture has already been extensively utilized to treat high blood pressure (hypertension) in several nations. Nevertheless, the bibliometric research on the worldwide usage of acupuncture for hypertension is mostly unclear. As a result, our objective for the research aimed to investigate the present state as well as developments in the global usage of acupuncture on hypertension during the last 20 years using CiteSpace (5.8.R2). The Web of Science (WOS) database examined papers on acupuncture treatment of hypertension from 2002 to 2021. We examined the number of publications, cited journals, nations/regions, organizations, authors, cited authors, cited references, and keywords utilizing CiteSpace. The record of 296 documents was obtained between 2002 and 2021. The quantity and frequency of annual publications rose gradually. Regarding frequency and centrality of citations, Circulation and Clin Exp Hypertens (Clinical and Experimental Hypertension) scored top and second respectively. China had the most publications among countries/regions, as well as the five largest institutions were also in China. Cunzhi Liu was the most productive author, while P Li was the most referenced author. XF Zhao produced the first article inside the quantity of cited references classification. 'Electroacupuncture' had a significant frequency with centrality for the keywords, which suggested electroacupuncture is a popular treatment in this field. In the treatment of hypertension, electroacupuncture has a beneficial effect on reducing blood pressure. However, because of the many different applications of electroacupuncture frequencies in research, whether the electroacupuncture frequency is connected to the therapeutic impact should be given more significant consideration. The findings of this bibliometric analysis give an overview of the present state as well as developments of clinical studies on acupuncture for hypertensive patients during the last two decades, which could assist researchers in identifying hot subjects and exploring novel directions in further study within the field.

Project description:AimTo study demographic and clinical characteristics and to give a comparative description of the functional and hemodynamic status, profile of concomitant pathology in patients with various forms of pulmonary arterial hypertension (PAH), and chronic thromboembolic pulmonary hypertension (CTEPH) according to the Russian National Registry.MethodsDuring the period from January 01, 2012, till January 01, 2019, 1105 patients aged >18 years with verified diagnosis of PAH and CTEPH, who were subsequently observed at 15 PH expert centers of the Russian Federation in the 52 provinces, are included in the Russian registry on the basis of the Federal State Budgetary Institution of Cardiology of the Ministry of Healthcare of Russia. All newly diagnosed patients (n = 727) were entered into the registry database (NCT03707561). A comparative analysis of demographic and clinical characteristics, profile of concomitant pathology, and parameters of a comprehensive examination of patients was performed.ResultsAmong newly diagnosed patients, 67% had PAH and 28.3% had CTEPH. In the PAH group, 40.9% of patients had idiopathic arterial PAH (IPAH), 36.6% had PAH associated with simple congenital heart disease (PAH-CHD), 19.3% had PAH associated with systemic connective tissue disease (PAH-CTD), 1.8% had portal pulmonary hypertension (PoPH), 0.6% had PAH associated with HIV infection (PAH-HIV), 0.4% had heritable PAH (HPAH), and 0.4% had drug/toxin-induced PAH. At the time of diagnosis, PAH patients were younger than patients with CTEPH (45.2 ± 14.9; 52.6 ± 15.3 years, respectively) (p < 0.05). At the time of diagnosis, 71% PAH and 77% CTEPH patients had WHO FC III/IV. Mean (±SD) 6MWD was significantly less in CTEPH vs. the PAH group 331.3 ± 110.3 vs. 361.8 ± 135.7 m (p = 0.0006). Echo data showed a comparable sPAP between groups; CTEPH population had a more pronounced increase in the area of the right atrium (SRA) (24 [20; 32] cm2 and 19 [15; 26] cm2, respectively), and a significant decrease in FAC (24.7 [22, 4; 29.0] and 29.0 [23.0; 31.0] %, respectively) as compared to the PAH group. RHC showed a comparable increase of sPAP and mPAP in PAH and CTEPH groups. 15.2% of patients with IPAH and HPAH demonstrated positive results in the acute vasoreactivity testing. CTEPH patients were more often obese and suffered from arterial hypertension and right heart failure. Deep venous thrombosis was significantly more often observed in patients with CTEPH (53%). The most common concomitant pathology was erosive-ulcerative lesion of the stomach/duodenum, less often of the esophagus (23.5% and 44.5%, respectively).ConclusionAccording to the Russian registry in patients with PAH and IPAH, the diagnosis is established at a younger age in comparison with the European registries. CTEPH patients are characterized by more severe functional status, pronounced signs of right heart failure taking into account the older age and the spectrum of comorbid pathology, which limits the possibility of surgical treatment. An increase in the number of expert centers participating in the registry is the key to improving early diagnosis of PH and optimal follow-up according to common standards in order to timely optimize therapy and reduce mortality of patients.

Dataset Information

Disease characteristics and clinical outcome over two decades from the Swiss pulmonary hypertension registry.

Publications

Disease characteristics and clinical outcome over two decades from the Swiss pulmonary hypertension registry.

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