Correction of Tricuspid Regurgitation in Pulmonary Arterial Hypertension.

Background: Pulmonary hypertension is a contraindication to correction of tricuspid regurgitation.

Case Summary: A 75-year-old Italian woman with previous episodes of right heart failure was diagnosed with World Health Organization (WHO) functional class IV pulmonary arterial hypertension (PAH) complicated by torrential tricuspid regurgitation. After 6 months of treatment with diuretic agents, macitentan, and tadalafil, she improved to WHO functional class III, with a pulmonary vascular resistance (PVR) decreasing from 5.

Evaluation of non-invasive low-risk criteria in patients with connective tissue disease-associated pulmonary arterial hypertension: A 12-months analysis from the INSPECTIO study.

Background: Connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH) is a progressive, high-risk subtype of PAH characterized by immune-mediated vascular remodeling, poor treatment response, and reduced survival. Real-world data on therapeutic response and risk evolution in this population remain limited.

Methods: This post hoc analysis of the multicenter, prospective INSPECTIO study evaluated the CTD-PAH subpopulation treated with macitentan and/or selexipag.

The fluid challenge for identification of pulmonary hypertension associated with left heart disease.

Purpose Of Review: Left heart disease (LHD) is the commonest cause of pulmonary hypertension (PH). The differential diagnosis between PH associated with LHD (PH-LHD) and pulmonary arterial hypertension (PAH) may be difficult. PH associated with LHD is causally related to chronically increased pulmonary artery wedge pressure (PAWP).

Exercise Doppler Echocardiography of the Right Heart and Pulmonary Circulation in Patients With Cardiovascular Risk Factors: Observations From the RIGHT Heart International NETwork (RIGHT-NET).

Background: The precise impact of cardiovascular risk factors (CVRFs) on the right ventricle-pulmonary circulation unit remains unclear.

Research Question: Are there any differences regarding resting and exercise right ventricular function in patients with CVRFs but without overt cardiovascular disease when compared with healthy control patients?

Study Design And Methods: We extracted from the Right Heart International Network international registry the data of 362 patients with at least 1 CVRF but no overt cardiovascular disease, and 375 healthy control patients. All patients and control patients had undergone resting and exercise echocardiographic evaluations on a semirecumbent cycle ergometer.

Exercise Echocardiography of Left Ventricular Diastolic Function in Healthy Subjects: Insights From the RIGHT-NET.

Background: Exercise transthoracic Doppler echocardiography (TTE) is considered suggestive of left ventricular (LV) diastolic dysfunction when the ratio of mitral Doppler E to tissue Doppler e' waves is >15 with or without a peak tricuspid regurgitation velocity (TRV) >3.4 m/s. However, these measurements may be affected by exercise intensity.

Guidelines for the Echocardiographic Assessment of the Right Heart in Adults and Special Considerations in Pulmonary Hypertension: Recommendations from the American Society of Echocardiography.

Right heart adaptation to pulmonary hypertension (PH) is a critical determinant of clinical outcomes, morbidity, and mortality in patients with or at risk for cardiopulmonary disease. The World Symposium on Pulmonary Hypertension recently redefined PH as a mean pulmonary arterial pressure >20 mm Hg, based on a wealth of epidemiologic evidence underscoring the significant impact of even mildly elevated mean pulmonary artery pressures on major adverse clinical events. The lowered diagnostic threshold for PH has renewed interest in echocardiography and its critical role in early detection and screening, refined hemodynamic evaluation, and longitudinal monitoring.

Echocardiography of the right heart in pulmonary arterial hypertension: insights from the ULTRA RIGHT VALUE study.

Aims: Outcome in pulmonary arterial hypertension (PAH) is determined by right ventricular (RV) function adaptation to increased afterload. Echocardiography is easily available to assist bedside evaluation of the RV. However, no agreement exists about the feasibility and most relevant measurements.

High-resolution DNA methylation changes reveal biomarkers of heart failure with preserved ejection fraction versus reduced ejection fraction.

Novel biomarkers are needed to better identify-and distinguish-heart failure with preserved ejection fraction (HFpEF) from other clinical phenotypes. The goal of our study was to identify epigenetic-sensitive biomarkers useful to a more accurate diagnosis of HFpEF. We performed a network-oriented genome-wide DNA methylation study of circulating CD4 T lymphocytes isolated from peripheral blood using reduced representation bisulfite sequencing (RRBS) in two cohorts (i.

Pulmonary arterial hypertension with left to right shunts: When to treat and/or close?

Pulmonary arterial hypertension (PAH) is defined as increase in mean pulmonary arterial pressure and pulmonary vascular resistance (PVR). It can be associated with congenital heart disease (CHD) with the following subtypes: 1) uncorrected left-to-right (L-R) intracardiac shunt leading to overload of the pulmonary circulation and a progressive increase of PVR; 2) Eisenmenger syndrome, appearing when a large post-tricuspid shunt is left uncorrected and pulmonary vascular disease (PVD) is severe, so the shunt becomes bidirectional or right-to-left, causing cyanosis; 3) PAH after shunt closure, when PVR arises after a defect correction; and 4) PAH associated with small or coincidental defects. While the treatment of patients with Eisenmenger syndrome is well established, the treatment of patients with PAH in whom there is a L-R shunt (with no cyanosis) remains unclear and requires expertise.

A pulmonary hypertension targeted algorithm to improve referral to right heart catheterization: A machine learning approach.

Background: Pulmonary hypertension (PH) is a pathophysiological problem that may involve several clinical symptoms and be linked to various respiratory and cardiovascular illnesses. Its diagnosis is made invasively by Right Cardiac Catheterization (RHC), which is difficult to perform routinely. Aim of the current study was to develop a Machine Learning (ML) algorithm based on the analysis of anamnestic data to predict the presence of an invasively measured PH.

Prognostic relevance of exercise pulmonary hypertension: results of the multicentre PEX-NET Clinical Research Collaboration.

Background: Exercise pulmonary hypertension (PH) was defined by a mean pulmonary arterial pressure (mPAP)/cardiac output (CO) slope >3 mmHg·min·L between rest and exercise in the 2022 European Society of Cardiology/European Respiratory Society PH guidelines. However, large, multicentre studies on the prognostic relevance of exercise haemodynamics and its added value to resting haemodynamics are missing.

Patients And Methods: The PEX-NET (Pulmonary Haemodynamics during Exercise Network) registry enrolled patients who underwent clinically indicated right heart catheterisations both at rest and ergometer exercise from 23 PH centres worldwide.

Gaps in evidence in the treatment of prevalent patients with pulmonary arterial hypertension at intermediate risk: An expert consensus.

Despite the innovations introduced in the 2022 European Society of Cardiology/European Respiratory Society Guidelines on Pulmonary Hypertension, risk discrimination and management of pulmonary arterial hypertension (PAH) patients at intermediate risk still represents a grey zone. Additionally, clinical evidence derived from currently available studies is limited. This expert panel survey intends to aid physicians in choosing the best therapeutic strategy for patients at intermediate risk despite ongoing oral therapy.

Pathophysiology of the right ventricle and its pulmonary vascular interaction.

The right ventricle and its stress response is perhaps the most important arbiter of survival in patients with pulmonary hypertension of many causes. The physiology of the cardiopulmonary unit and definition of right heart failure proposed in the 2018 World Symposium on Pulmonary Hypertension have proven useful constructs in subsequent years. Here, we review updated knowledge of basic mechanisms that drive right ventricular function in health and disease, and which may be useful for therapeutic intervention in the future.

Right ventricular phenotyping in incident patients with idiopathic pulmonary arterial hypertension.

Background: Right ventricular (RV) imaging has not a definite role in risk stratification of pulmonary arterial hypertension (PAH) patients. We tested the hypothesis that echocardiography-derived phenotypes, depicting different degrees of RV remodeling and dysfunction, may provide additional prognostic information to current risk stratification tools.

Methods: Consecutive incident PAH patients aged ≥18 years, diagnosed between January 2005 and December 2021, underwent clinical assessment, right heart catheterization, standard echocardiography.

Endothelial Function in Pulmonary Arterial Hypertension: From Bench to Bedside.

Pulmonary arterial hypertension is a complex pathology whose etiology is still not completely well clarified. The pathogenesis of pulmonary arterial hypertension involves different molecular mechanisms, with endothelial dysfunction playing a central role in disease progression. Both individual genetic predispositions and environmental factors seem to contribute to its onset.

Gaps in evidence in the management of patients with intermediate-risk pulmonary arterial hypertension: Considerations following the ESC/ERS 2022 guidelines.

A comprehensive evaluation of risk, using multiple indices, is necessary to provide reliable prognostic information and guide therapy in pulmonary arterial hypertension (PAH). The current ESC/ERS guidelines suggest using a three-strata model for incident (newly diagnosed) patients and a four-strata model for prevalent patients with PAH. The four-strata model serves as a fundamental risk-stratification tool and relies on a minimal dataset of indicators that must be considered during follow-up.

[ANMCO/SIC Consensus statement on pulmonary arterial hypertension].

Pulmonary hypertension (PH) is a frequent pathological condition worldwide, mainly secondary to cardiovascular and respiratory diseases, with a poor prognosis. Pulmonary arterial hypertension (PAH) is a rare form that affects the arterial pulmonary vasculature. PH and PAH are characterized by non-specific symptoms and a progressive increase of pulmonary vascular resistance that results in progressive, sometimes irreversible, right ventricular dysfunction.

The Unveiled Triad: Clinical, Radiological and Pathological Insights into Hypersensitivity Pneumonitis.

Hypersensitivity pneumonitis (HP) is a diffuse parenchymal lung disease (DLPD) characterized by complex interstitial lung damage with polymorphic and protean inflammatory aspects affecting lung tissue targets including small airways, the interstitium, alveolar compartments and vascular structures. HP shares clinical and often radiological features with other lung diseases in acute or chronic forms. In its natural temporal evolution, if specific therapy is not initiated promptly, HP leads to progressive fibrotic damage with reduced lung volumes and impaired gas exchange.

Contribution of pressure and flow changes to resistance reduction after pulmonary arterial hypertension treatment: a meta-analysis of 3898 patients.

Background: Pulmonary arterial hypertension (PAH)-targeted therapies exert significant haemodynamic changes; however, systematic synthesis is currently lacking.

Methods: We searched PubMed, CENTRAL and Web of Science for studies evaluating mean pulmonary artery pressure (mPAP), cardiac index/cardiac output (CI/CO) and pulmonary vascular resistance (PVR) of PAH-targeted therapies either in monotherapy or combinations as assessed by right heart catheterisation in treatment-naïve PAH patients. We performed a random-effects meta-analysis with meta-regression.