Risk assessment models and survival in pulmonary arterial hypertension: a SPAHR analysis.

Background: Multicomponent improvement (MCI) is a novel endpoint for predicting survival in patients with pulmonary arterial hypertension (PAH), included in the sotatercept clinical program. For the first time, we investigated the prognostic value of MCI, ESC/ERS 4-strata risk (4SR) assessment, and the non-invasive French risk stratification score (FRS), for predicting survival in PAH patients in Sweden. All risk prediction models are based on three parameters: WHO-FC (World Health Organization Functional Class), NT-proBNP, and 6MWD (6-minute walk distance).

Right ventricular dyssynchrony predicts outcome in pulmonary arterial hypertension when assessed in multiple cardiac magnetic resonance views.

Background: Right ventricular (RV) dyssynchrony or post systolic contraction (PSC) causes inefficient pumping and has not been investigated as a prognostic marker in pulmonary arterial hypertension (PAH). The objective was to investigate if RV dyssynchrony and PSC are prognostic markers of transplantation-free survival in PAH and if multiple RV views improve prognostication.

Methods: Patients with PAH undergoing cardiovascular magnetic resonance between 2003 and 2021 were included.

Does Periodontitis Increase the Risk for Future Cardiovascular Events? Long-Term Follow-Up of the PAROKRANK Study.

Background And Aim: The study 'Periodontitis and Its Relation to Coronary Artery Disease' (PAROKRANK) reported an association between periodontitis (PD) and the first myocardial infarction (MI). This follow-up study aims to test the hypothesis that those with PD-compared to periodontally healthy individuals-are at increased risk for cardiovascular (CV) events and death.

Methods: A total of 1587 participants (age <75 years; females 19%) had a dental examination including panoramic radiographs between 2010 and 2014.

Left ventricular dysfunction in pulmonary arterial hypertension is attributed to underfilling rather than intrinsic myocardial disease: a CMR 2D phase contrast study.

The pathophysiology underlying impaired LV function in PAH remains unclear, with some studies implicating intrinsic myocardial dysfunction and others pointing to LV underfilling. Evaluation of pulmonary vein area (PVA) and flow may offer novel, mechanistic insight by distinguishing elevated LV filling pressure common in myocardial dysfunction from LV underfilling. This study aimed to elucidate LV filling physiology in PAH by assessing PVA and flow using cardiac magnetic resonance (CMR) and compare pulmonary vein flow in PAH with HFrEF as a model representing elevated filling pressures, in addition to healthy controls.

Change in health-related quality of life at early follow-up in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.

Symptoms associated with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) impact patient's health-related quality of life (HRQoL). Studies on change and if a minimal clinically important difference (MCID) in HRQoL is reached within a year after diagnosis are lacking. The aim was to investigate the change in HRQoL as well as the proportion of patients that reached MCID at an early postdiagnosis visit.

Atrioventricular plane displacement and regional function to predict outcome in pulmonary arterial hypertension.

To investigate if left and right atrioventricular plane displacement (AVPD) or regional contributions to SV are prognostic for outcome in patients with pulmonary arterial hypertension (PAH). Seventy-one patients with PAH and 20 sex- and age-matched healthy controls underwent CMR. Myocardial borders and RV insertion points were defined at end diastole and end systole in cine short-axis stacks to compute biventricular volumes, lateral (SV) and septal (SV) contribution to stroke volume.

Societal costs associated to chronic thromboembolic pulmonary hypertension: A study utilizing linked national registries.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare but serious complication after a pulmonary embolism. Healthcare resource utilization (HCRU; hospitalization, outpatient visits, and drug utilization) as well as productivity loss (sick leave and disability pension) before and after the CTEPH diagnosis is sparsely studied. By linking several Swedish national databases, this study estimated the societal costs in a national CTEPH cohort ( = 369, diagnosed with CTEPH in 2008-2019) 5 years before and 5 years after diagnosis (index date) and compared to an age, sex, and geographically matched control group ( = 1845, 1:5 match).

Societal costs associated with pulmonary arterial hypertension: A study utilizing linked national registries.

Pulmonary arterial hypertension (PAH) is a progressive disease with no cure. Healthcare resource utilization (HCRU; hospitalization, outpatient visits, and drug utilization) before diagnosis and productivity loss (sick leave and disability pension) before and after PAH diagnosis are not well known. By linking several Swedish national databases, this study have estimated the societal costs in a national PAH cohort ( = 749, diagnosed with PAH in 2008-2019) 5 years before and 5 years after diagnosis and compared to an age, sex, and geographically matched control group ( = 3745, 1:5 match).

Prevalence of potential drug-drug interactions with disease-specific treatments in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension: A registry study.

Polypharmacy increases the risk of drug-drug interactions that may disturb treatment effects. The aim of this study was to investigate the frequency of codispensing of potentially interacting or contraindicated drugs related to PH-specific treatment in the Swedish pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) population. All prescribed drugs, on an individual level, dispensed 2016-2017 at pharmacies to patients with PAH or CTEPH were obtained from The National Board of Health and Welfare's pharmaceutical registry.

Long-term prognosis after a first myocardial infarction: eight years follow up of the case-control study PAROKRANK.

To explore long-term cardiovascular outcomes and mortality in patients after a first myocardial infarction (MI) compared with matched controls in a contemporary setting. . During 2010-2014 the Swedish study PAROKRANK recruited 805 patients <75 years with a first MI and 805 age-, gender-, and area-matched controls.

Five year risk assessment and treatment patterns in patients with chronic thromboembolic pulmonary hypertension.

Aims: Repeated risk assessments and treatment patterns over long time are sparsely studied in chronic thromboembolic pulmonary hypertension (CTEPH); thus, we aimed to investigate changes in risk status and treatment patterns in incident patients with CTEPH over a 5 year period.

Methods And Results: Descriptive and explorative study including 311 patients diagnosed with CTEPH 2008-2019 from the Swedish pulmonary hypertension registry, stratified by pulmonary endarterectomy surgery (PEA). Risk and PH-specific treatment were assessed in surgically treated (PEA) and medically treated (non-PEA) patients at diagnosis and up to 5 years follow-up.

Respiratory rate modulation improves symptoms in patients with pulmonary hypertension.

Background: Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are chronic diseases with a severe symptom burden. Common symptoms are dyspnoea at light activity and general fatigue that limits daily activities. Respiratory modulation by device-guided breathing decreased symptoms in patients with heart failure.

Early risk prediction in idiopathic connective tissue disease-associated pulmonary arterial hypertension: call for a refined assessment.

Despite systematic screening and improved treatment strategies, the prognosis remains worse in patients with connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) compared to patients with idiopathic/hereditary pulmonary arterial hypertension (IPAH). We aimed to investigate differences in clinical characteristics, outcome and performance of the European Society of Cardiology (ESC)/ European Respiratory Society (ERS) risk stratification tool in these patient groups.  This retrospective analysis included incident patients with CTD-PAH (n=197, of which 64 had interstitial lung disease, ILD) or IPAH (n=305) enrolled in the Swedish PAH Register (SPAHR) 2008-2019.

The Odyssey from Symptom to Diagnosis of Pulmonary Hypertension from the Patients and Spouses Perspective.

Introduction/objective: Diagnostic delays in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are related to increased morbidity and mortality. The risk of a delayed, or even a missed, diagnosis is high as the conditions are rare. The aim was to describe patients' and spouses' experiences of the journey from the first symptom to an established diagnosis.

Accuracy of echocardiographic estimates of pulmonary artery pressures in pulmonary hypertension: insights from the KARUM hemodynamic database.

Accurate assessment of pulmonary artery (PA) pressures is integral to diagnosis, follow-up and therapy selection in pulmonary hypertension (PH). Despite wide utilization, the accuracy of echocardiography to estimate PA pressures has been debated. We aimed to evaluate echocardiographic accuracy to estimate right heart catheterization (RHC) based PA pressures in a large, dual-centre hemodynamic database.

Predicting mortality during long-term follow-up in pulmonary arterial hypertension.

The European Society of Cardiology (ESC) and European Respiratory Society (ERS) guideline recommendation of comprehensive risk assessments, which classify patients with pulmonary arterial hypertension (PAH) as having low, intermediate or high mortality risk, has not been evaluated during long-term follow-up in a "real-life" clinical setting. We therefore aimed to investigate the utility of risk assessment in a clinical setting for up to 5 years post diagnosis. 386 patients with PAH from the Swedish PAH Registry were included.

Periodontitis, assessed using periodontal treatment as a surrogate marker, has no association with a first myocardial infarction in a Swedish population.

Background: Periodontitis is suggested to be associated with a risk of cardiovascular events. Using periodontal treatment recorded in Swedish national registries as a surrogate marker, we aimed to investigate whether periodontitis was associated with a first myocardial infarction.

Methods: This nationwide case-control study, with data from national registries, involved 51,880 individuals with a first myocardial infarction in 2011 to 2013 (index date) and 246,978 controls matched 5:1 for age, gender, and geographic area.

Underfilling decreases left ventricular function in pulmonary arterial hypertension.

To evaluate the association between impaired left ventricular (LV) longitudinal function and LV underfilling in patients with pulmonary arterial hypertension (PAH). Thirty-nine patients with PAH and 18 age and sex-matched healthy controls were included. LV volume and left atrial volume (LAV) were delineated in short-axis cardiac magnetic resonance (CMR) cine images.

Outpatient specialist clinics for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension in the Nordic countries.

Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are rare conditions that require complex interventions by multidisciplinary teams. The European Society of Cardiology (ESC)/the European Respiratory Society (ERS) 2015 guidelines included recommendations for pulmonary hypertension (PH) referral centers including minimum number of patients, staff, facilities, and network. The aim of the present study was to investigate how the PH-specialist centers in the Nordic countries are presently organized.

Adherence to disease-specific drug treatment among patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.

Background: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) require lifelong treatment. The aim of the present study was to investigate adherence to disease-specific treatment in patients with PAH or CTEPH.

Methods: The study comprised an adult population diagnosed with PAH (n=384) or CTEPH (n=187) alive in 2016-2017.

Longitudinal changes in risk status in pulmonary arterial hypertension.

Aims: Low-risk status in pulmonary arterial hypertension (PAH) predicts better survival. The present study aimed to describe changes in risk status and treatment approaches over multiple clinical assessments in PAH, taking age and comorbidity burden into consideration.

Methods And Results: The study included incident patients from the Swedish PAH registry, diagnosed with PAH in 2008-2019.

Poor outcome of patients with pulmonary arterial hypertension with insufficient response to phosphodiesterase-5 inhibitors alone or in combination with other specific therapy: a registry-based study.

Phosphodiesterase-5 inhibitors are commonly used in pulmonary arterial hypertension but, as suggested by the RESPITE study, phosphodiesterase-5 inhibitor therapy (mono-/combination) does not always have a satisfactory treatment effect. This study aimed to investigate the clinical course of pulmonary arterial hypertension patients not at treatment goal after at least 90 days of treatment with phosphodiesterase-5 inhibitors, alone or in combination with other pulmonary arterial hypertension therapies. The study included 106 incident patients from the Swedish Pulmonary Arterial Hypertension Registry, treated with phosphodiesterase-5 inhibitors for ≥90 days, who were not at a pre-specified treatment goal, i.

Correction to: Cardiac Magnetic Resonance Imaging in Pulmonary Arterial Hypertension: Ready for Clinical Practice and Guidelines?

The original version of this article contains error in the text.

Cardiac Magnetic Resonance Imaging in Pulmonary Arterial Hypertension: Ready for Clinical Practice and Guidelines?

Purpose Of Review: Pulmonary arterial hypertension (PAH) is a progressive disease with high mortality. A greater understanding of the physiology and function of the cardiovascular system in PAH will help improve survival. This review covers the latest advances within cardiovascular magnetic resonance imaging (CMR) regarding diagnosis, evaluation of treatment, and prognostication of patients with PAH.