Prognostic value of longitudinal QTc interval analysis in pulmonary arterial hypertension.

Introduction: In pulmonary arterial hypertension, the QTc interval prolongation has been associated with worse right ventricular adaptation to afterload and worse prognosis. It is not known whether the QTc interval is a modifiable parameter or if its changes at follow-up could provide prognostic information.

Methods: We retrospectively studied 213 PAH patients with at least two electrocardiograms during follow-up from January 2010 to March 2022.

Comparison of Tricuspid Annular Plane Systolic Excursion to Pulmonary Artery Systolic Pressure Ratio Measured by Transthoracic Echocardiography and Right Heart Catheterization in Pulmonary Arterial Hypertension: Prognostic Implications.

: Ventricular-arterial (VA) coupling, assessed via the TAPSE/PASP ratio, is a well-established prognostic marker in pulmonary arterial hypertension (PAH). However, transthoracic echocardiography (TTE) often fails to estimate the pulmonary artery systolic pressure (PASP). This study evaluated the prognostic value of TAPSE/PASP when PSAP was obtained both via TEE and RHC and their correlation.

Haemodynamic spectrum in heritable pulmonary arterial hypertension: a continuum from pre-capillary to combined pulmonary hypertension-case series.

Background: Heritable pulmonary arterial hypertension (PAH) is a rare form of pre-capillary pulmonary hypertension that typically affects young patients. With increased survival and subsequent ageing of these patients, newly acquired cardiovascular conditions may influence the pulmonary haemodynamic profile and impact management.

Case Summary: We report a case series of four patients with mutations in genes associated with PAH to illustrate the spectrum of pulmonary haemodynamics under the influence of superimposed acquired conditions.

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.

Simplified risk stratification based on cardiopulmonary exercise test: A Spanish two-center experience.

A simplified 4-strata risk stratification approach based on three variables is widespread in pulmonary arterial hypertension (PAH) at follow-up. This study aimed to assess the impact of replacing the 6-min walk test (6MWT) with the peak 0 uptake evaluated by the cardiopulmonary exercise test (CPET) on risk stratification by this scale. We included 180 prevalent patients with PAH from two reference hospitals in Spain, followed up between 2006 and 2022.

Seven Additional Patients with Related Pulmonary Arterial Hypertension and Review of the Literature.

Pulmonary arterial hypertension (PAH) is an infrequent disorder characterized by high blood pressure in the pulmonary arteries. It may lead to premature death or the requirement for lung and/or heart transplantation. Genetics plays an important and increasing role in the diagnosis of PAH.

Value of exercise right heart catheterization in the differential diagnosis of chronic thromboembolic pulmonary disease.

Introduction And Objectives: Chronic thromboembolic disease refers to the presence of chronic thrombotic pulmonary vascular thrombosis without pulmonary hypertension (PH) at rest but with exercise limitation after pulmonary embolism (PE). Our aim was to evaluate the hemodynamic response to exercise in these patients and its correlation with the values reached in cardiopulmonary exercise testing.

Methods: We included symptomatic patients with persistent pulmonary thrombosis after PE.

Outcome of moderate-severe tricuspid regurgitation after pulmonary endarterectomy or balloon pulmonary angioplasty.

Introduction And Objectives: The management of persistent moderate-severe tricuspid regurgitation (TR) in patients with chronic thromboembolic pulmonary hypertension after treatment with pulmonary endarterectomy (PEA) or balloon pulmonary angioplasty (BPA) is not well defined. This study aimed to analyze the progression and predictors of significant persistent postintervention TR and its prognostic impact.

Methods: This single-center observational study included 72 patients undergoing PEA and 20 who completed a BPA program with a previous diagnosis of chronic thromboembolic pulmonary hypertension and moderate-to-severe TR.

Novel Loss-of-Function Variants in Pulmonary Arterial Hypertension.

Reduced expression and/or activity of Kv1.5 channels (encoded by ) is a common hallmark in human or experimental pulmonary arterial hypertension (PAH). Likewise, genetic variants in have been found in patients with PAH, but their functional consequences and potential impact on the disease are largely unknown.

Novel Molecular Mechanisms Involved in the Medical Treatment of Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension (PAH) is a severe condition with a high mortality rate despite advances in diagnostic and therapeutic strategies. In recent years, significant scientific progress has been made in the understanding of the underlying pathobiological mechanisms. Since current available treatments mainly target pulmonary vasodilation, but lack an effect on the pathological changes that develop in the pulmonary vasculature, there is need to develop novel therapeutic compounds aimed at antagonizing the pulmonary vascular remodeling.

Effectiveness and Safety of Balloon Pulmonary Angioplasty for the Treatment of Patients with Persistent Pulmonary Hypertension after Pulmonary Endarterectomy.

(1) Background: Pulmonary endarterectomy (PEA) is the "gold standard" treatment for operable patients with chronic thromboembolic pulmonary hypertension (CTEPH). Persistent pulmonary hypertension (PH) after PEA confers a worse prognosis. Balloon pulmonary angioplasty (BPA) could represent a useful therapy in this setting, but evidence about its effectiveness and safety in patients with previous PEA is limited.

Usefulness of genetics for clinical reclassification and refinement of prognostic stratification in pulmonary arterial hypertension.

Introduction And Objectives: Risk stratification in pulmonary arterial hypertension (PAH) is essential to provide more aggressive treatment for patients at higher risk. Nevertheless, recently introduced simplified prognostic tools neglect the genetic background. Additionally, pulmonary veno-oclusive disease (PVOD) has never been considered in risk assessment strategies.

The impact of COVID-19 pandemic on pulmonary hypertension: What have we learned?

The coronavirus 2019 disease (COVID-19) pandemic threatened the Spanish health-care system. Patients with demanding conditions such as precapillary pulmonary hypertension (PH) faced a potentially severe infection, while their usual access to medical care was restricted. This prospective, unicentric study assessed the impact of COVID-19 on PH patients' outcomes and the operational changes in the PH network.

Clinical Implications of the Genetic Background in Pediatric Pulmonary Arterial Hypertension: Data from the Spanish REHIPED Registry.

Background: Pulmonary arterial hypertension (PAH) is a severe and rare disease with an important genetic background. The influence of genetic testing in the clinical classification of pediatric PAH is not well known and genetics could influence management and prognosis.

Objectives: The aim of this work was to identify the molecular fingerprint of PH children in the (REHIPED), and to investigate if genetics could have an impact in clinical reclassification and prognosis.