Joan Albert Barbera

• Hospital Clínic de Barcelona

Pulmonary arterial hypertension (PAH) is the most characteristic group of pulmonary hypertensive states. Among other conditions, PAH includes the idiopathic type and forms associated with connective tissue diseases (CTDs), particularly systemic sclerosis, CREST syndrome, and mixed connective tissue disease. Symptoms and signs of PAH include shortne...

Background Exercise pulmonary hypertension (exercise PH) was defined by a mean pulmonary arterial pressure (mPAP)/cardiac output (CO) slope >3 mmHg·L ⁻¹ ·min ⁻¹ between rest and exercise in the 2022 ESC/ERS PH guidelines. However, large, multi-center studies on the prognostic relevance of exercise hemodynamics and its added value to resting hemodyn...

Purpose of review To provide timely and relevant insights into the complex relationship between pulmonary vascular disease (PVD) and chronic lung disease (CLD), focusing on the causative and consequential dynamics between these conditions. Recent findings There are shared pathogenic mechanisms between pulmonary arterial hypertension (PAH) and grou...

Background Pulmonary Arterial Hypertension (PAH) is a rare disease where the thickening of the precapillary pulmonary arteries ends up inducing right heart failure. Nowadays, obtaining an early diagnosis is challenging and typically delayed until undergoing right-heart catheterization. Methods We performed small RNA sequencing (microRNA-seq) in the...

Rationale: Chronic Thromboembolic Pulmonary Hypertension involves formation and non-resolution of thrombus, dysregulated inflammation, angiogenesis and the development of a small vessel vasculopathy. Objectives: We aimed to establish the genetic basis of chronic thromboembolic pulmonary hypertension to gain insight into its pathophysiological co...

Pulmonary hypertension (PH) can affect both pulmonary arterial tree and cardiac function, often leading to right heart failure and death. Despite the urgency, the lack of understanding has limited the development of effective cardiac therapeutic strategies. Our research reveals that MCJ modulates mitochondrial response to chronic hypoxia. MCJ level...

Physical capacity tests are used for the diagnosis, prognosis and monitoring of chronic respiratory diseases (CRD), such as Chronic Obstructive Pulmonary Disease (COPD) or Pulmonary Hypertension (PH). Among these tests, the most widely used is the 6-minute walking test (6MWT). Additionally , the guidelines highlight the need to continuously record...

Introduction: In stable patients with pulmonary arterial hypertension (PAH), pulmonary rehabilitation (PR) is an effective, safe and cost-effective non-pharmacological treatment. However, the effects of PR on vascular function have been poorly explored. This study aimed to compare the amounts of circulating progenitor cells (PCs) and endothelial m...

Background Chronic Thromboembolic Pulmonary Hypertension (CTEPH) involves formation and non-resolution of thrombus, dysregulated inflammation, angiogenesis and the development of a small vessel vasculopathy. We aimed to establish the genetic basis of CTEPH to gain insight into these pathophysiological contributors. Methods We conducted a genome-wid...

Background: Imbalance between cell proliferation and apoptosis underlies the development of pulmonary arterial hypertension (PAH). Current vasodilator treatment of PAH does not target the uncontrolled proliferative process in pulmonary arteries. Proteins involved in the apoptosis pathway may play a role in PAH and their inhibition might represent a...

Skeletal muscle dysfunction in chronic obstructive pulmonary disease (COPD) is characterized by a significant reduction in muscle strength and endurance. Preclinical studies show that stimulation of the soluble guanlylate cyclase (sGC)-cGMP pathway attenuates muscle mass loss and prevents cigarette smoke-induced oxidative stress, indicating that ph...

There is a wide range of sequelae affecting COVID-19 survivors, including impaired physical capacity. These sequelae can affect the quality of life and return to work of the active population. Therefore, one of the pillars of following-up is the evaluation of physical capacity, which can be assessed with field tests (such as the six-minute walk tes...

Background The six-minute walking distance (6MWD) is often used to assess prognosis in pulmonary arterial hypertension (PAH) patients. Whether or not changes in arterial oxygen saturation (SpO2) during exercise add prognostic value to the 6MWD in these patients is unclear. The objective of this study was to investigate if SpO2 changes during exerci...

Background and aims: Pulmonary hypertension (PH) associated with left heart disease is an increasingly prevalent problem, orphan of targeted therapies, and related to a poor prognosis, particularly when pre and postcapillary PH combine. The current study aimed to determine whether treatment with the selective β3 adrenoreceptor agonist mirabegron i...

https://doi.org/10.1093/eurheartj/ehac237

Coronavirus disease 2019 (COVID-19) is a pandemic respiratory disease associated with high morbidity and mortality. Although many patients recover, long-term sequelae after infection have become increasingly recognized and concerning. Among other sequelae, the available data indicate that many patients who recover from COVID-19 could develop fibrot...

Purinergic and nitric oxide (NO) signaling pathways appear to be involved in the development of emphysema and vascular remodeling of chronic obstructive pulmonary disease (COPD). In an animal model, we evaluate the gene and protein expression of ENTPD1 /CD39 and NT5E /CD73, and the effect on this expression of a guanylate cyclase (GC) stimulator. F...

To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH...

Background Some COVID-19 survivors present lung function abnormalities during follow-up, particularly reduced carbon monoxide lung diffusing capacity (DLCO). To investigate risk factors and underlying pathophysiology, we compared the clinical characteristics and levels of circulating pulmonary epithelial and endothelial markers in COVID-19 survivor...

Pulmonary endarterectomy (PEA) resected material offers a unique opportunity to develop an in vitro endothelial cell model of chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to comprehensively analyze the endothelial function, molecular signature, and mitochondrial profile of CTEPH-derived endothelial cells to better understand the...

Background: Endothelial dysfunction is central to PAH. In this study, we simultaneously analysed circulating levels of endothelial microvesicles (EMVs) and progenitor cells (PCs) in PAH and in controls, as biomarkers of pulmonary endothelial integrity and evaluated differences among PAH subtypes and as a response to treatment. Methods: Forty-sev...

Background Pulmonary hypertension (PH) is a frequent complication in patients with COPD. Objective To determine if, in patients with COPD, the presence of PH decreases exercise tolerance. Methods We included studies that analysed exercise tolerance using a cardiopulmonary exercise test (CPET) in patients with COPD with PH (COPD-PH) and without PH...

Rationale: Pulmonary endarterectomy (PEA) resected material offers a unique opportunity to develop an in vitro endothelial cell model of chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to comprehensively analyze the endothelial function, molecular signature and mitochondrial profile of CTEPH-derived endothelial cells to better under...

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary hypertension characterized by the presence of fibrotic intraluminal thrombi and causing obliteration of the pulmonary arteries. Although both endothelial cell (EC) dysfunction and inflammation are linked to CTEPH pathogenesis, regulation of the basal inflammatory response...

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) can be cured by pulmonary endarterectomy (PEA). It is considered the best and only curable treatment option for patients with accessible lesions evaluated as optimal candidates. We describe the experience of the two reference centers in Spain, in order to reinforce the need for refe...

SARS-CoV-2, a new strain of a Coronaviridae virus which presents 79% genetic similarity to the severe acute respiratory syndrome coronavirus (SARS-CoV) has been recently recognized as the cause of a global pandemic by the World Health Organization (WHO) implying a major threat to the world public health. SARS-CoV-2 infects host human cells by bindi...

Resumen Introducción y objetivos El tratamiento de la hipertensión pulmonar tromboembólica crónica (HTPTEC) ha evolucionado en la última década. Sin embargo, apenas se dispone de información sobre el impacto de estos logros en la población general a escala nacional. Este estudio se diseñó para describir las características de los pacientes con HTP...

Introduction In chronic obstructive pulmonary disease (COPD), endothelial dysfunction and stiffness of systemic arteries may contribute to increased cardiovascular risk. Pulmonary vascular disease (PVD) is frequent in COPD. The association between PVD and systemic vascular dysfunction has not been thoroughly evaluated in COPD. Methods A total of 1...

Introduction The impact of pulmonary hypertension (PH) on exercise tolerance in chronic obstructive pulmonary disease (COPD) has not been fully elucidated. It is necessary to characterize pulmonary hemodynamics in patients with moderate to severe COPD in order to improve their management. The aim of the study was to determine whether in COPD the pr...

Introduction and objectives Chronic thromboembolic pulmonary hypertension (CTEPH) treatment has evolved in the last decade. However, there is scarce information on the long-term impact of this progress in a real-life population at a national level. This study was designed to analyze the characteristics of CTEPH patients in Spain over the last decad...

Pulmonary hypertension (PH) is a frequent and important complication of chronic obstructive pulmonary disease (COPD). It is associated with worse clinical courses with more frequent exacerbation episodes, shorter survival, and greater need of health resources. PH is usually of moderate severity and progresses slowly, without altering right ventricu...

Background: Macitentan is a dual endothelin receptor antagonist indicated for the long-term treatment of pulmonary arterial hypertension (PAH). We evaluated the change over time in REVEAL risk score in incident and prevalent patients receiving macitentan for the first time. Methods: Retrospective, observational study including adult patients wit...

Persistent thrombotic lesions are common in patients with pulmonary embolism. These lesions occur on a clinical spectrum, ranging from an asymptomatic course with complete functional recovery to chronic thromboembolic pulmonary hypertension. The concept of chronic thromboembolic disease has emerged in recent years to describe a subgroup of patients...

Even mild pulmonary hypertension (PH) is associated with increased mortality and morbidity in patients with chronic obstructive pulmonary disease (COPD). However, the underlying mechanisms remain elusive; therefore, specific and efficient treatment options are not available. Therapeutic approaches tested in the clinical setting, including long‐term...

Combined pre-and post-capillary hypertension (CpcPH) is a relatively common complication of heart failure (HF) associated with a poor prognosis. Currently, there is no specific therapy approved for this entity. Recently, treatment with beta-3 adrenergic receptor (β3AR) agonists was able to improve pulmonary hemodynamics and right ventricular (RV) p...

Imbalance between cell proliferation and apoptosis may underlie the development of PAH (Pulmonary Arterial Hypertension). Survivin is a member of the apoptosis inhibitor gene family involved in cell proliferation. This study aimed to explore if the expression of survivin is increased in a mice model of PAH. We also investigated the effects of the s...

Imbalance between cell proliferation and apoptosis may underlie the development of PAH (Pulmonary Arterial Hypertension). Survivin is a member of the apoptosis inhibitor gene family involved in cell proliferation. This study aimed to explore if the expression of survivin is increased in a mice model of PAH. We also investigated the effects of the s...

Imbalance between cell proliferation and apoptosis may underlie the development of PAH (Pulmonary Arterial Hypertension). Survivin is a member of the apoptosis inhibitor gene family involved in cell proliferation. This study aimed to explore if the expression of survivin is increased in a mice model of PAH. We also investigated the effects of the s...

Persistent thrombotic lesions are common in patients with pulmonary embolism. These lesions occur on a clinical spectrum, ranging from an asymptomatic course with complete functional recovery to chronic thromboembolic pulmonary hypertension. The concept of chronic thromboembolic disease has emerged in recent years to describe a subgroup of patients...

Introduction: The impact of pulmonary hypertension (PH) on exercise tolerance in chronic obstructive pulmonary disease (COPD) has not been fully elucidated. It is necessary to characterize pulmonary hemodynamics in patients with moderate to severe COPD in order to improve their management. The aim of the study was to determine whether in COPD the...

Background: Initial combination therapy with ambrisentan and tadalafil reduced the risk of clinical failure events for treatment-naïve participants with pulmonary arterial hypertension (PAH) as compared to monotherapy. Previous studies in PAH have demonstrated greater treatment benefits in more symptomatic participants. Methods: AMBITION was an...

Background & Significance Exposure to cigarette smoke has been shown to lead to vascular remodelling. CT-imaging measures of vascular pruning have been associated with pulmonary vascular disease, an important morbidity associated with smoking. In this study we compare CT-based measures of distal vessel loss to histologic vascular and parenchymal ch...

We have analysed the effect of the soluble guanylate cyclase (sGC) stimulator BAY41-2272 in a therapeutic intervention in guinea pigs chronically exposed to cigarette smoke (CS). The effects of sGC-stimulation on respiratory function, pulmonary hemodynamics, airspace size, vessel remodelling and inflammatory cell recruitment to the lungs were evalu...

Introduction and Objectives Determinants of chronic obstructive pulmonary disease (COPD) in the early stages of its natural history are not well known. Improving our knowledge of these factors will help to design interventions that can modify prognosis. Study objectives are: (a) to characterize a COPD population of young adults aged 35–50 years fro...

Background Pulmonary vascular abnormalities are a characteristic feature of chronic obstructive pulmonary disease (COPD). Cigarette smoking is the most important risk factor for COPD. It is believed that its constant exposure triggers endothelial cell damage and vascular remodelling. Under pathological conditions, progenitor cells (PCs) are mobiliz...

Background Mapping the genetic component of molecular mechanisms responsible for the reduced penetrance (RP) of rare disorders constitutes one of the most challenging problems in human genetics. Heritable pulmonary arterial hypertension (PAH) is one such disorder characterised by rare mutations mostly occurring in the bone morphogenetic protein rec...

Cardiovascular diseases (CVD) are the leading cause of death worldwide. CVD comprise a range of diseases affecting the functionality of the heart and blood vessels, including acute myocardial infarction (AMI) and pulmonary hypertension (PH). Despite their different causative mechanisms, both AMI and PH involve narrowed or blocked blood vessels, hyp...

There is scarce evidence for pulmonary artery denervation (PADN) as a potential treatment for chronic postcapillary pulmonary hypertension (PH). We aimed to perform a proof-of-concept of PADN in a translational model of chronic PH. Nineteen pigs with chronic postcapillary PH (secondary to pulmonary vein banding) were randomized to surgical-PADN (us...

Introducción: Imbalance between cell proliferation and apoptosis may underlie the development of PAH (Pulmonary Arterial Hypertension). PAH is currently considered as an uncontrolled proliferative process in pulmonary arteries. Proteins involved in the apoptosis pathway may play a role in PAH and their inhibition may represent a potential therapeut...