Background and Aims: The aim of this study was to evaluate whether echocardiography-derived phenotypes describing different degrees of right ventricular (RV) remodelling and dysfunction add prognostic information to that of current risk stratification tools in patients with pulmonary arterial hypertension (PAH) at first follow-up. Methods: In 11 centres of the Italian Pulmonary Hypertension NETwork (IPHNET), data were prospectively collected from patients with PAH who underwent re-evaluation between 6 and 12 months after diagnosis. Echocardiographic variables were combined a priori to define four phenotypes representing different degrees of RV dilatation and right ventricular-pulmonary arterial (RV-PA) coupling: a mildly dilated right ventricle with preserved RV-PA coupling defined phenotype-1; a mildly dilated right ventricle with poor RV-PA coupling defined phenotype-2; a severely dilated right ventricle with preserved RV-PA coupling defined phenotype-3; a severely dilated right ventricle with poor RV-PA coupling, either with or without tricuspid regurgitation of moderate degree or more, defined phenotype-4. Patients were followed up for all-cause death for a median of 3.7 years. Results: These echocardiographic phenotypes were present in all European Society of Cardiology/European Respiratory Society or REVEAL 2.0 risk groups except for the high-risk groups, which included only phenotype-3 and phenotype-4. In each risk group, RV phenotype-4 identified patients with a poorer prognosis; RV phenotype-1 identified patients with better survival in intermediate risk groups. Conclusions: Echocardiography-derived phenotypes describing different degrees of RV remodelling and dysfunction provide prognostic information which is independent of and additional to the clinically defined risk in PAH patients at first follow-up.

Chronic heart failure (HF) is a prevalent condition associated with significant morbidity, mortality, and economic burden worldwide. The pharmacological management of HF has evolved over time with various drug classes demonstrating efficacy in improving patient outcomes. This review examines the pharmacoeconomic aspects of these therapies, including common and newer HF therapies as angiotensin receptor-neprilysin inhibitors, sodium–glucose co-transporter-2 inhibitors, iron supplementation, and vericiguat, a novel soluble guanylate cyclase stimulator. By analysing cost-effectiveness studies and their implications on healthcare resource utilization, this paper aims to inform clinicians and policymakers on HF management optimization from both clinical and economic perspectives.

Background: Acute pericarditis as a sign of mediastinal mass is rare and aetiological diagnosis can be challenging without adequate imaging. Case summary: An 18-year-old woman came to our attention describing acute sharp chest pain radiated to the left arm, exacerbated with supine positioning and attenuated while sitting or leaning forward. The electrocardiogram showed diffuse ST elevation and PR depression, with sinus tachycardia. Cardiac biomarkers and D-dimer were negative; echocardiography showed no abnormalities and the absence of pericardial effusion. Her blood work revealed no sign of inflammation or bacterial infection (PCR and procalcitonin were normal); thyroid-stimulating hormone plasma levels were suppressed, showing decompensated thyrotoxicosis. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Epstein–Barr virus, human immunodeficiency virus, hepatitis C virus, Enterovirus, Parvovirus B19, and Adenovirus tests were normal. Her past medical history was silent, apart from Grave’s disease on treatment with methimazole. Chest computed tomography (CT) was performed and showed the presence of slightly increased density pericardial effusion, with a maximum thickness of 15 mm in the upper mediastinum. Finally, cardiac magnetic resonance (MR) identified a mass of 73 × 51 mm located in the upper mediastinum. The mass was subsequently biopsed with video-assisted thoracoscopic surgery and the histological analysis showed thymic hyperplasia. Discussion: This case shows the importance of an adequate clinical suspicion of thymic hyperplasia in the context of acute pericarditis symptoms and known Graves’ disease. In this case, a negative chest CT finding may not be sufficient to rule out the diagnosis and cardiac MR imaging is necessary.