Embracing the challenges of neonatal and paediatric pulmonary hypertension.

Paediatric pulmonary arterial hypertension (PAH) shares common features with adult disease, but is associated with several additional disorders and challenges that require unique approaches. This article discusses recent advances, ongoing challenges and distinct approaches for caring for infants and children with PAH, as presented by the paediatric task force of the 7th World Symposium on Pulmonary Hypertension. We provide updates on diagnosing, classifying, risk-stratifying and treating paediatric pulmonary hypertension (PH) and identify critical knowledge gaps.

Comparison of different treatment strategies in patients with chronic thromboembolic pulmonary hypertension: a single centre real-world experience.

Background: Pulmonary endarterectomy (PEA) has been the most effective therapy for chronic thromboembolic pulmonary hypertension (CTEPH). However, there is a substantial proportion of patients deemed not operable in whom other treatment strategies are available: medical therapy and balloon pulmonary angioplasty (BPA). We aimed to compare different CTEPH treatment strategies effect in a real-world setting.

Pulmonary Arterial Hypertension Associated with Portal Hypertension and HIV Infection: Comparative Characteristics and Prognostic Predictors.

Background: Pulmonary arterial hypertension (PAH) may complicate both portal hypertension (Po-PAH) and HIV infection (HIV-PAH). These two conditions, however, frequently coexist in the same patient (HIV/Po-PAH). We evaluated clinical, functional, hemodynamic characteristics and prognostic parameters of these three groups of patients.

Progress in the treatment of acute pulmonary embolism and chronic thrombo-embolic pulmonary hypertension/disease.

The combination of an initial clinical approach aimed at evaluating the early risk of mortality with subsequent diagnostic and therapeutic approaches articulated on the overall patient's profile is recommended in acute pulmonary embolism (PE). The presence of pulmonary hypertension associated with the persistence of chronic vascular obstructions in the pulmonary arteries after one or more acute thrombo-embolic events identifies a condition defined as chronic thrombo-embolic pulmonary hypertension (CTEPH). The evolution of technology and knowledge in the field of imaging has allowed us to qualify the computed tomography angiography of the pulmonary arteries as the gold standard for the diagnostic confirmation of both acute PE and CTEPH.

Therapeutic alternatives in chronic thromboembolic pulmonary hypertension: from pulmonary endarterectomy to balloon pulmonary angioplasty to medical therapy. State of the art from a multidisciplinary team.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease with a very complex pathophysiology differing from other causes of pulmonary hypertension (PH). It is an infrequent consequence of acute pulmonary embolism that is frequently misdiagnosed. Pathogenesis has been related to coagulation abnormalities, infection or inflammation, although these disturbances can be absent in many cases.

A pragmatic approach to risk assessment in pulmonary arterial hypertension using the 2015 European Society of Cardiology/European Respiratory Society guidelines.

Objective: To optimise treatment of patients with pulmonary arterial hypertension (PAH), the 2015 European Society of Cardiology/European Respiratory Society guidelines recommend using risk stratification, with the aim of patients achieving low-risk status. Previous analyses of registries made progress in using risk stratification approaches, however, the focus is often on patients with a low-risk prognosis, whereas most PAH patients are in intermediate-risk or high-risk categories. Using only six parameters with high prognostic relevance, we aimed to demonstrate a pragmatic approach to individual patient risk assessment to discriminate between patients at low risk, intermediate risk and high risk of death.

[Progress in the treatment of pulmonary vascular diseases].

Multiple progresses have been achieved in pulmonary vascular diseases in the last decades, including the areas of pulmonary hypertension and pulmonary thromboembolic disease. The increase in knowledge has been accomplished in pathophysiological, clinical and treatment domains and has included as examples the discovery of gene mutations related to the hereditary forms of pulmonary arterial hypertension and the proposals of personalized treatment algorithms in patients with acute pulmonary embolism, chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension, validated in this specific area by more than 45 randomized controlled trials. The diagnostic processes have been refined, increasing the awareness that appropriate and precise diagnosis is essential for the optimal treatment strategy.

[Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension:a paradigmatic case report].

Chronic thromboembolic pulmonary hypertension (CTEPH) is a complex disease where organized pulmonary thrombi and progressive vascular remodeling of the pulmonary arterial tree act synergistically to increase pulmonary vascular resistance and cause pulmonary hypertension. Balloon pulmonary angioplasty (BPA) has gained a renewed interest for the treatment of patients with CTEPH who are not undergoing surgery with pulmonary endarterectomy (PEA) or with persistent/recurrent pulmonary hypertension after PEA and has shown promising results in several observational studies conducted to date. We describe the case of a 42-year-old man with inoperable CTEPH in NYHA functional class III who normalized functional capacity, hemodynamic profile and main hemodynamic parameters after three BPA sessions.

[Balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension: 5 years of experience in Italy].

Background: Balloon pulmonary angioplasty (BPA) represents a therapeutic option for the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in patients who are not eligible for surgical pulmonary endarterectomy (PEA) or with persistent/recurrent symptomatic pulmonary arterial hypertension after PEA. This study evaluated the safety of BPA during 5 years of experience of the only Italian center systematically performing this procedure.

Methods: The BPA program was activated at the S.

Pulmonary arterial hypertension populations of special interest: portopulmonary hypertension and pulmonary arterial hypertension associated with congenital heart disease.

Guidelines exist for management of pulmonary arterial hypertension (PAH), but information is limited for certain patient subgroups, including adults with portopulmonary hypertension (PoPH) or with PAH associated with congenital heart disease (PAH-CHD). This article discusses screening, clinical management, and prognosis in PoPH and PAH-CHD and, as such, considers the most recent clinical data and expert advice. A multidisciplinary consultation and follow-up by specialists are crucial for management of both PoPH and PAH-CHD, but each condition presents with unique challenges.

A Combined Targeted and Whole Exome Sequencing Approach Identified Novel Candidate Genes Involved in Heritable Pulmonary Arterial Hypertension.

The pathogenesis of idiopathic and heritable forms of pulmonary arterial hypertension is still not completely understood, even though several causative genes have been proposed, so that a third of patients remains genetically unresolved. Here we applied a multistep approach to extend identification of the genetic bases of such a disease by searching for novel candidate genes/pathways. Twenty-eight patients belonging to 18 families were screened for BMPR2 mutations and BMPR2-negative samples were tested for 12 additional candidate genes by means of a specific massive parallel sequencing-based assay.

Diagnosis of pulmonary hypertension.

A revised diagnostic algorithm provides guidelines for the diagnosis of patients with suspected pulmonary hypertension, both prior to and following referral to expert centres, and includes recommendations for expedited referral of high-risk or complicated patients and patients with confounding comorbidities. New recommendations for screening high-risk groups are given, and current diagnostic tools and emerging diagnostic technologies are reviewed.

Update of screening and diagnostic modalities for connective tissue disease-associated pulmonary arterial hypertension.

Objective: Pulmonary arterial hypertension (PAH) has high morbidity and mortality in connective tissue diseases (CTDs), especially systemic sclerosis (SSc). In this systematic review, we provide an update on screening measures for early detection of PAH in CTD.

Methods: Manuscripts published between July 2012 and October 2017, which incorporated screening measures to identify patients with PAH by right heart catheterization, were identified.

Anticoagulant treatment in patients with pulmonary arterial hypertension associated with systemic sclerosis: More shadows than lights.

Pulmonary arterial hypertension is a chronic and progressive disease characterized by elevated pulmonary artery pressure and pulmonary vascular resistance leading to heart failure and premature death. Pulmonary arterial hypertension is characterized by proliferative and obstructive lesions in the distal pulmonary arteries and some descriptions include also thrombotic lesions. Despite this, in an era when multiple effective pulmonary arterial hypertension therapies are available, the role of anticoagulation in the treatment of pulmonary arterial hypertension remains uncertain.