Health-related quality of life and symptom concordance between patients and physicians in pulmonary arterial hypertension in the United States, Europe, and Japan.

This study involved analyses of a real world, cross-sectional survey of physicians and their patients with pulmonary arterial hypertension (PAH). Physician and patient symptom concordance was assessed, and patients self-reported Health-Related Quality of Life (HRQoL). Data were drawn from the Adelphi PAH Disease Specific Programme (DSP)™ in the United States, France, Germany, Italy, Spain, United Kingdom, and Japan, between March and August 2022.

OPTImal PHARMacological therapy for patients with heart failure: Rationale and design of the OPTIPHARM-HF registry.

Aims: Patients with heart failure (HF) remain often undertreated for multiple reasons, including treatment inertia, contraindications, and intolerance. The OPTIimal PHARMacological therapy for patients with Heart Failure (OPTIPHARM-HF) registry is designed to evaluate the prevalence of evidence-based medical treatment prescription and titration, as well as the causes of its underuse, in a broad real-world population of consecutive patients with HF across the whole ejection fraction spectrum and among different clinical phenotypes.

Methods: The OPTIPHARM-HF registry (NCT06192524) is a prospective, multicenter, observational, national study of adult patients with symptomatic HF, as defined by current international guidelines, regardless of ejection fraction.

Prognostic value of deep echocardiographic phenotyping in pulmonary arterial hypertension.

Background: A novel approach to derive prognostic information from echocardiography in pulmonary arterial hypertension (PAH) is to define a phenotype of right heart function combining standard echocardiographic parameters which describe right ventricular pump function and systemic venous congestion. We tested the hypothesis that the combination of advanced strain imaging parameters could yield high prognostic accuracy.

Methods: This was a prospective observational study with a single centre derivation cohort and a second centre validation cohort.

Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: a COMPERA analysis.

Objectives: Pulmonary arterial hypertension (PAH) occurs in various connective tissue diseases (CTDs). We sought to assess contemporary treatment patterns and survival of patients with various forms of CTD-PAH.

Methods: We analysed data from COMPERA, a European pulmonary hypertension registry, to describe treatment strategies and survival in patients with newly diagnosed PAH associated with SSc, SLE, MCTD, UCTD and other types of CTD.

[Oral combination therapy for the treatment of patients with pulmonary arterial hypertension: what evidence from long-term studies?].

The introduction of specific drugs for the treatment of pulmonary arterial hypertension over the last 20 years has led to an improvement of clinical and hemodynamic conditions and prognosis of affected patients. The use of combination therapy has made it possible to act simultaneously on several biological pathways involved in the etiopathogenesis of the disease. However, although the diagnosis and management have radically changed in recent years, the disease remains progressive and often fatal.

An official European Respiratory Society statement: pulmonary haemodynamics during exercise.

There is growing recognition of the clinical importance of pulmonary haemodynamics during exercise, but several questions remain to be elucidated. The goal of this statement is to assess the scientific evidence in this field in order to provide a basis for future recommendations.Right heart catheterisation is the gold standard method to assess pulmonary haemodynamics at rest and during exercise.

Haemodynamic effects of an acute vasodilator challenge in heart failure patients with reduced ejection fraction and different forms of post-capillary pulmonary hypertension.

Aims: The most recent European guidelines have proposed new definitions of pulmonary hypertension (PH) in left heart disease, to better approach the characteristics required to reflect the presence of pulmonary vascular disease. The purpose of this study was to assess whether different haemodynamic definitions of post-capillary PH imply a different reversibility of PH in response to acute vasodilator administration in heart failure patients with reduced ejection fraction and PH (HFrEF-PH).

Methods And Results: Right heart catheterization and reversibility testing was performed in 156 HFrEF-PH patients.

Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias.

Background: Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.

Long-term safety and efficacy of imatinib in pulmonary arterial hypertension.

Background: Imatinib is an oral inhibitor of several protein kinases implicated in the pathophysiology of pulmonary hypertension. Treatment with imatinib resulted in improved hemodynamics and exercise capacity in a controlled trial (Imatinib [QTI571] in Pulmonary Arterial Hypertension, a Randomized Efficacy Study [IMPRES]), among pulmonary arterial hypertension (PAH) patients inadequately responsive to 2 to 3 PAH-specific therapies.

Methods: The long-term (up to 204 weeks) safety and efficacy of imatinib in this open-label extension study were reviewed until early study termination on April 16, 2014.

Right ventricular remodeling in idiopathic pulmonary arterial hypertension: adaptive versus maladaptive morphology.

Background: Although increased pulmonary pressure is caused by changes in the pulmonary vasculature, prognosis in idiopathic pulmonary arterial hypertension (IPAH) is strongly associated with right ventricular (RV) function. The aim of this study was to describe the best RV adaptive remodeling pattern to increased afterload in IPAH.

Methods: In 60 consecutive patients with IPAH, RV morphologic and functional features were evaluated by echocardiography and cardiac magnetic resonance imaging.

Right heart and pulmonary vessels structure and function.

The right ventricle (RV) can be described in terms of 3 components: the inlet, the apex, and the infundibulum. In the normal adult, the RV shows an arrangement suited for pumping blood against low resistance, with a mass about one sixth that of left ventricle (LV) mass, and a larger volume than the LV. The RV is able to manage a progressive increase in the afterload by increasing contractility and remodeling.

Myocardial and microvascular inflammation/infection in patients with HIV-associated pulmonary artery hypertension.

Background: Right ventricle compromise affects survival of patients with HIV-associated pulmonary artery hypertension (PAH).

Design: Myocardial histology with viral assessment may clarify the mechanism of right ventricular deterioration and provide clues on PAH origin.

Methods: Fifteen patients with HIV infection, PAH and right ventricular dysfunction underwent cardiac magnetic resonance, catheterization, coronary with ventricular angiography and biventricular endomyocardial biopsy.

Right ventricular dyssynchrony in idiopathic pulmonary arterial hypertension: determinants and impact on pump function.

Background: Right ventricular (RV) dyssynchrony has been described in pulmonary arterial hypertension (PAH), but no evidence is available on its morphologic determinants and its effect on systolic function. The aim of this study was to evaluate the morphologic determinants of RV dyssynchrony by echocardiographic and cardiac magnetic resonance imaging and its effect on systolic function.

Methods: In 60 consecutive idiopathic PAH (IPAH) patients with narrow QRS, RV dyssynchrony was evaluated by 2D speckle-tracking echocardiography, calculating the standard deviation of the times to peak systolic strain for the four mid-basal RV segments (RV-SD4).

Heart failure in patients with human immunodeficiency virus: a review of the literature.

Coronary artery disease represents the leading cause of death for HIV patients treated with highly active antiretroviral treatment. Besides this, an extensive amount of data related to the risk of overt heart failure and consequently of atrial fibrillation and sudden cardiac death (SCD) in this population has been reported. It seems that persistent deregulation of immunity in HIV-infected patients is a common pathway related to both of these adverse clinical outcomes.

Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA).

Background: For almost 30 years, anticoagulation has been recommended for patients with idiopathic pulmonary arterial hypertension (IPAH). Supporting evidence, however, is limited, and it is unclear whether this recommendation is still justified in the modern management era and whether it should be extended to patients with other forms of pulmonary arterial hypertension (PAH).

Methods And Results: We analyzed data from Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), an ongoing European pulmonary hypertension registry.

Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry.

Background: Originally reported to occur predominantly in younger women, idiopathic pulmonary arterial hypertension (IPAH) is increasingly diagnosed in elderly patients. We aimed to describe the characteristics of such patients and their survival under clinical practice conditions.

Methods: Prospective registry in 28 centers in 6 European countries.

Pregnancy outcomes in pulmonary arterial hypertension in the modern management era.

Previous studies have reported mortality rates of up to 56% associated with pregnancy in pulmonary arterial hypertension (PAH) but the management of this disease has changed considerably in recent years. We compiled a multinational, prospective registry to examine the contemporary outcome of pregnancies in patients with PAH. During a 3-yr period, the 13 participating centres reported 26 pregnancies.

Current perspectives modern hemodynamic evaluation of the pulmonary circulation. Application to pulmonary arterial hypertension and embolic pulmonary hypertension.

The hemodynamic evaluation of the pulmonary circulation normally includes the measurements of mean pulmonary artery pressure and a calculation of pulmonary vascular resistance (PVR). The definition of PVR can be improved by the measurements of pulmonary vascular pressures at several levels of flow to derive a pressure-flow line, and the site of PVR can be identified by the analysis of pulmonary artery pressure decay curves after balloon occlusion. An analysis of the morphology of pulmonary artery pressure and flow waves informs about right ventricular (RV) hydraulic load.

[Pulmonary hypertension and HIV: implementation of a Regional Registry].

In the era of new, potent antiretroviral therapy, much more attention is being given to non-infectious complications of HIV diseases, such as cardiomyopathy, pericardial effusion and pulmonary hypertension (PH). PH diagnosis is based on a mean pulmonary artery pressure of more than 25 mmHg at rest, or more than 30 mmHg with exercise. The incidence of PH is about 0.

Locoregional versus general anesthesia in carotid surgery: is there an impact on perioperative myocardial ischemia? Results of a prospective monocentric randomized trial.

Purpose: The incidence of cardiac morbidity and mortality in patients who undergo carotid surgery ranges from 0.7% to 7.1%, but it still represents almost 50% of all perioperative complications.