scholarly journals Management of pulmonary arterial hypertension in patients aged over 65 years

2019 ◽  
Vol 21 (Supplement_K) ◽  
pp. K29-K36 ◽  
Author(s):  
Olivier Sitbon ◽  
Luke Howard
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Older Patients ◽  
Clinical Symptoms ◽  
Survival Rates ◽  
Specific Treatment ◽  
Younger Patients ◽  
Expert Opinions ◽  
Literature Searches ◽  
Pulmonary Arterial

Abstract Historically, pulmonary arterial hypertension (PAH) has been considered a disease of young adults, but over the last three decades, the average age at diagnosis has increased, presenting clinicians with some unique challenges. Clinical symptoms of PAH, including shortness of breath and reduced functional capacity, are not specific for the disease and may be present in older patients because of their age or as a result of comorbid conditions. Eliminating other causes for these symptoms can delay PAH diagnosis and initiation of PAH-specific treatment compared with younger patients. Currently, there are no specific guidelines relating to PAH in older patients and existing guidelines for identifying patients at potential risk of PAH may not be appropriate for patients aged over 65 years. Even though older patients tend to be diagnosed with more advanced symptoms, and evidence suggests that they are less responsive to PAH-specific therapies, treatment is often less aggressive than in younger patients. Even after adjusting for age, survival rates remain disproportionately lower in the older vs. younger PAH populations. Specific guidelines for diagnosis and treatment of older patients with PAH are needed to improve care and outcomes in this growing population. This review aims to assess the challenges associated with diagnosing and managing PAH in older patients, based on literature searches, authors’ experiences, and expert opinions.

scholarly journals The changing landscape of pulmonary arterial hypertension and implications for patient care

2014 ◽  
Vol 23 (134) ◽  
pp. 450-457 ◽  
Author(s):  
Marius M. Hoeper ◽  
J. Simon R. Gibbs
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Older Patients ◽  
Severe Disease ◽  
Western World ◽  
Diffusing Capacity ◽  
Younger Patients ◽  
Disease Characteristics ◽  
Pulmonary Arterial ◽  
Over Time

Registries have provided a wealth of information on the clinical and disease characteristics of patients living with pulmonary arterial hypertension (PAH) since the 1980s. Certain PAH demographics, such as the prevalence of various PAH subgroups and preponderance of female patients, appear to have remained stable over time. Contemporary registry data indicate that the average age of patients diagnosed with PAH has increased, at least in the Western world. Older patients with PAH are more likely to be diagnosed with a more advanced stage of the disease, have lower exercise capacity and present with multiple comorbidities. They also have worse survival compared with younger patients. Within the PAH population, there is also a subset of patients with a lower diffusing capacity of the lung for carbon monoxide who are generally older and display more severe disease characteristics. This review discusses the implications that the increased age of the PAH population at diagnosis has on the treatment and management of the disease, as well as the need for earlier and improved diagnosis in these patients.

Temporal trends in pulmonary arterial hypertension: Results from the COMPERA registry

2021 ◽  
pp. 2102024
Author(s):  
Marius M. Hoeper ◽  
Christine Pausch ◽  
Ekkehard Grünig ◽  
Gerd Staehler ◽  
Doerte Huscher ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Temporal Trends ◽  
Survival Rates ◽  
Term Survival ◽  
Pulmonary Arterial ◽  
Early Combination Therapy

BackgroundSince 2015, the European pulmonary hypertension guidelines recommend the use of combination therapy in most patients with pulmonary arterial hypertension (PAH). However, it is unclear to what extend this treatment strategy is adopted in clinical practice and if it is associated with improved long-term survival.MethodsWe analysed data from COMPERA, a large European pulmonary hypertension registry, to assess temporal trends in the use of combination therapy and survival of patients with newly diagnosed PAH between 2010 and 2019. For survival analyses, we look at annualized data and at cumulated data comparing the periods 2010–2014 and 2015–2019.ResultsA total of 2,531 patients were included. The use of early combination therapy (within 3 months after diagnosis) increased from 10.0% in patients diagnosed with PAH in 2010 to 25.0% in patients diagnosed with PAH in 2019. The proportion of patients receiving combination therapy 1 year after diagnosis increased from 27.7% to 46.3%. When comparing the 2010–2014 and 2015–2019 periods, 1-year survival estimates were similar (89.0% [95% CI, 87.2%, 90.9%] and 90.8% [95% CI, 89.3%, 92.4%]), respectively, whereas there was a slight but non-significant improvement in 3-year survival estimates (67.8% [95% CI, 65.0%, 70.8%] and 70.5% [95% CI, 67.8%, 73.4%]), respectively.ConclusionsThe use of combination therapy increased from 2010 to 2019, but most patients still received monotherapy. Survival rates at 1 year after diagnosis did not change over time. Future studies need to determine if the observed trend suggesting improved 3-year survival rates can be confirmed.

scholarly journals Echocardiographic Prognosis Relevance of Attenuated Right Heart Remodeling in Idiopathic Pulmonary Arterial Hypertension

2021 ◽  
Vol 8 ◽  
Author(s):  
Qin-Hua Zhao ◽  
Su-Gang Gong ◽  
Rong Jiang ◽  
Chao Li ◽  
Ge-Fei Chen ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Survival Rates ◽  
Left Ventricular ◽  
Functional Improvement ◽  
Right Heart ◽  
Long Term Follow Up ◽  
Pulmonary Arterial ◽  
Rv Function

Background: Right ventricular (RV) function is a great determination of the fate in patients with pulmonary arterial hypertension (PAH). Monitoring RV structure back to normal or improvement should be useful for evaluation of RV function. The aims of this study were to assess the prognostic relevance of changed right heart (RH) dimensions by echocardiography and attenuated RH remodeling (ARHR) in idiopathic PAH (IPAH).Methods: We retrospectively analyzed 232 consecutive adult IPAH patients at baseline assessment and included RH catheterization and echocardiography. ARHR at the mean 20 ± 12 months' follow-up was defined by a decreased right atrium area, RV mid-diameter, and left ventricular end-diastolic eccentricity index. The follow-up end point was all-cause mortality.Results: At mean 20 ± 12 months' follow-up, 33 of 232 patients (14.2%) presented with ARHR. The remaining 199 surviving patients were monitored for another 25 ± 20 months. At the end of follow-up, the survival rates at 1, 3, and 5 years were 89, 89, and 68% in patients with ARHR, respectively, and 84, 65 and 41% in patients without ARHR (log-rank p = 0.01). ARHR was an independent prognostic factor for mortality. Besides, ARHR was available to further stratify patients' risk assessment through the French PAH non-invasive-risk criteria.Conclusions: Echocardiographic ARHR is an independent determinant of prognosis in IPAH at long-term follow-up. ARHR might be a useful tool to indicate the RV morphologic and functional improvement associated with better prognostic likelihood.

scholarly journals Sex-specific differences and survival in patients with idiopathic pulmonary arterial hypertension 2008–2016

2019 ◽  
Vol 5 (3) ◽  
pp. 00075-2019 ◽  
Author(s):  
Barbro Kjellström ◽  
Magnus Nisell ◽  
David Kylhammar ◽  
Sven-Erik Bartfay ◽  
Bodil Ivarsson ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Survival Rates ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Hazard Ratio ◽  
Kaplan Meier ◽  
Comorbidity Burden ◽  
Pulmonary Arterial ◽  
Cox Proportional Hazard Regression ◽  
New Treatments

BackgroundWomen with idiopathic pulmonary arterial hypertension (IPAH) have been found to have a worse haemodynamic status at diagnosis, but better survival than men. Over the past decade, demographics have changed and new treatments have become available. The objective of this study was to investigate sex differences in an incident IPAH population diagnosed between 2008 and 2016.MethodsDifferences in clinical characteristics of patients included in the Swedish Pulmonary Arterial Hypertension Register (SPAHR) were analysed at the time of diagnosis. Survival by sex was investigated using Cox proportional hazard regression and Kaplan–Meier curves.ResultsThe study included 271 patients diagnosed with IPAH, median age was 68 (1st–3rd quartiles 54–74) years and 56% were women. At diagnosis, women were younger, had lower pulmonary vascular resistance and fewer comorbidities and more often received a combination of PAH-targeted therapies than men. Men had worse survival rates than women (hazard ratio 1.49; CI 1.02–2.18; p=0.038), but this difference did not remain after adjustment for age (hazard ratio 1.30; CI 0.89–1.90; p=0.178).ConclusionsMen with incident IPAH have worse crude survival than women. This is due to women being younger with a less pronounced comorbidity burden than men at the time of diagnosis.

P4688Additional role of unmodifiable risk factors in pulmonary arterial hypertension risk stratification according to current ESC/ERS guidelines

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
E Zuffa ◽  
F Dardi ◽  
M Palazzini ◽  
E Gotti ◽  
A Rinaldi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Arterial Hypertension ◽  
High Risk ◽  
Risk Stratification ◽  
Arterial Hypertension ◽  
Specific Treatment ◽  
Low Risk ◽  
P Value ◽  
Pulmonary Arterial

Abstract Background Current pulmonary hypertension (PH) guidelines stratify the risk of patients with pulmonary arterial hypertension (PAH) using a multiparametric approach. Anyway, the role of unmodifiable risk factors is not taken into account. Purpose The aim of this study was to evaluate the role of unmodifiable risk factors (age, gender, PAH aetiology) in PAH risk stratification using the recently proposed simplified risk table and to test if these factors influence the response to PAH-specific treatment. Methods All patients with PAH referred to a single centre were included from 2003 to 2017. We applied a simplified risk assessment strategy using the following criteria: WHO functional class, 6-min walking distance, right atrial pressure or brain natriuretic peptide plasma levels and cardiac index (CI) or mixed venous oxygen saturation (SvO2). The last 2 criteria were based on which parameter was available; if both were available the worst was chosen. Risk strata were defined as: Low risk= at least 3 low risk and no high-risk criteria; High risk= at least 2 high risk criteria including CI or SvO2; Intermediate risk= definitions of low or high risk not fulfilled. Then we performed multivariate Cox analysis to evaluate what are the independent predictors of survival (age, gender, PAH aetiology together with the recently proposed simplified PAH risk table) and we tested if these factors influence the response to PAH specific therapy comparing the % improvement of hemodynamic parameters from baseline to 3–4 months after starting treatment. Wilcoxon-Mann-Whitney test was used for comparisons. Results Six hundreds and twenty-one treatment-naïve patients were enrolled. Age [HR (95% CI) = 1.022 (1.014–1.030); p-value <0.001], male gender [HR (95% CI) = 1.881 (1.479–2.392); p-value <0.001] and connective tissue disease (CTD)-PAH aetiology [HR (95% CI)= 2.278 (1.733–2.995); p-value <0.001] were all independent predictors of prognosis in patients with PAH together with the recently validated simplified PAH risk table [HR (95% CI) = 2.161 (1.783–2.618); p-value <0.001] but they didn't significantly influence the response to PAH specific treatment as shown in the Figure. Figure 1 Conclusions Age, gender and CTD-PAH aetiology significantly influence prognosis together with the recently validated simplified PAH risk table but don't significantly influence the response to PAH-specific treatment. Acknowledgement/Funding None

scholarly journals Use of eHealth in the management of pulmonary arterial hypertension: review of the literature

2020 ◽  
Vol 27 (3) ◽  
pp. e100176
Author(s):  
Manuel C Gonzalez-Garcia ◽  
Farhad Fatehi ◽  
Marlien Varnfield ◽  
Hang Ding ◽  
Mohan Karunanithi ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Small Sample Size ◽  
Survival Rates ◽  
Poor Quality ◽  
Small Sample ◽  
Haemodynamic Monitoring ◽  
Clinical Benefits ◽  
Pulmonary Arterial ◽  
Diagnostic Technologies

BackgroundPulmonary arterial hypertension (PAH) is a severe chronic condition associated with poor quality of life and high risks of mortality and hospitalisation. The utilisation of novel diagnostic technologies has improved survival rates although the effectiveness of Electronic Health (eHealth) interventions in patients with a chronic cardiopulmonary disease remains controversial. As the effectiveness of eHealth can be established by specific evaluation for different chronic health conditions, the aim of this study was to explore and summarise the utilisation of eHealth in PAH.MethodWe searched PubMed, CINAHL and Embase for all studies reporting clinical trials on eHealth solutions for the management of PAH. No limitations in terms of study design or date of publication were imposed.Results18 studies (6 peer-reviewed journal papers and 12 conference papers) were identified. Seven studies addressed the accuracy, safety or reliability of eHealth technologies such as intra-arterial haemodynamic monitoring of the pulmonary artery pressure, self-administered 6-Minute walk test App, computerised step-pulse oximeter and ambulatory impedance cardiography. Two studies evaluated eHealth as part of the medical management and showed a reduction in hospitalisation rate.ConclusionsThe evidence of eHealth supporting the management of people with PAH is limited and only embraced through a few studies of small sample size and short-term duration. Given the proposed clinical benefits in heart failure, we postulate that the evaluation of eHealth for the clinical management of PAH is highly warranted.

scholarly journals Phenotype and outcome of pulmonary arterial hypertension patients carrying a TBX4 mutation

2020 ◽  
Vol 55 (5) ◽  
pp. 1902340 ◽  
Author(s):  
Pierre Thoré ◽  
Barbara Girerd ◽  
Xavier Jaïs ◽  
Laurent Savale ◽  
Maria-Rosa Ghigna ◽  
...  
Keyword(s):  
New York ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Survival Rates ◽  
Heart Association ◽  
Lung Parenchyma ◽  
Functional Class ◽  
Class Iii ◽  
High Resolution Computed Tomography ◽  
Pulmonary Arterial

IntroductionTBX4 mutation causes small patella syndrome (SPS) and/or pulmonary arterial hypertension (PAH). The characteristics and outcomes of PAH associated with TBX4 mutations are largely unknown.MethodsWe report the clinical, functional, radiologic, histologic and haemodynamic characteristics and outcomes of heritable PAH patients carrying a TBX4 mutation from the French pulmonary hypertension (PH) network.Results20 patients were identified in 17 families. They were characterised by a median age at diagnosis of 29 years (0–76 years) and a female to male ratio of three. Most of the patients (70%) were in New York Heart Association (NYHA) functional class III or IV with a severe haemodynamic impairment (median pulmonary vascular resistance (PVR) of 13.6 (6.2–41.8) Wood units). Skeletal signs of SPS were present in 80% of cases. Half of the patients had mild restrictive or obstructive limitation and diffusing capacity of the lung for carbon monoxide (DLCO) was decreased in all patients. High-resolution computed tomography (HRCT) showed bronchial abnormalities, peri-bronchial cysts, mosaic distribution and mediastinal lymphadenopathies. PAH therapy was associated with significant clinical improvement. At follow-up (median 76 months), two patients had died and two had undergone lung transplantation. One-year, three-year and five-year event-free survival rates were 100%, 94% and 83%, respectively. Histologic examination of explanted lungs revealed alveolar growth abnormalities, major pulmonary vascular remodelling similar to that observed in idiopathic pulmonary arterial hypertension (IPAH) and accumulation of cholesterol crystals within the lung parenchyma.ConclusionPAH due to TBX4 mutations may occur with or without skeletal abnormalities across a broad age range from birth to late adulthood. PAH is usually severe and associated with bronchial and parenchymal abnormalities.

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