Prognostic Value of Cardiac MRI Late Gadolinium Enhancement Granularity in Participants with Ischemic Cardiomyopathy.

Background Ischemic late gadolinium enhancement (LGE) assessed with cardiac MRI is a well-established prognosticator in ischemic cardiomyopathy. However, the prognostic value of additional LGE parameters, such as extent, transmurality, location, and associated midwall LGE, remains unclear. Purpose To assess the prognostic value of ischemic LGE features to predict all-cause mortality in ischemic cardiomyopathy.

Right ventricular reserve in cardiopulmonary disease: A simultaneous hemodynamic and three-dimensional echocardiographic study.

Background: Right ventricular (RV) reserve has been linked to exercise capacity and prognosis in cardiopulmonary diseases. However, evidence in this setting is limited, due to the complex shape and load dependency of the RV. We sought to study RV adaptation to exercise by simultaneous three-dimensional echocardiography (3DE) and right heart catheterization (RHC).

Integration of genetic testing into diagnostic pathways for cardiomyopathies: a clinical consensus statement by the ESC Council on Cardiovascular Genomics.

In the modern era, cardiologists managing patients and families with cardiomyopathies need to be familiar with every stage of the diagnostic pathway from clinical phenotyping to the prescription and interpretation of genetic tests. This clinical consensus statement from the ESC Council for Cardiovascular Genomics aims to promote the integration of genetic testing into routine cardiac care of patients with cardiomyopathies, as recommended in the 2023 ESC guidelines for cardiomyopathies. The document describes the types of genetic tests currently available and provides advice on their prescription and for counselling after the return of genetic findings, including the approach in patients and families with variants of unknown significance.

Left ventricular hypertrophy: do not forget Fabry disease. Diagnostic work-up and differential diagnosis.

Background: Left ventricular (LV) hypertrophy is a common clinical finding. Differential diagnosis includes Fabry disease, a rare and progressive, but treatable storage disease caused by deficiency of α-galactosidase A. However, diagnosis of Fabry is often hampered by its clinical heterogeneity, LV hypertrophy phenocopies and unawareness of the clinician.

Case report: Serious unexpected vascular events in two patients with lymphocytic variant hypereosinophilic syndrome.

Background: Lymphocytic-variant hypereosinophilic syndrome (L-HES) is a form of reactive hypereosinophilia, most commonly associated with interleukin-5 over-production by clonal, most commonly CD3CD4CD2CD5CD45RO T-cells. Patients often present with predominant cutaneous and soft-tissue manifestations, while cardiovascular involvement is uncommon.

Methods: We reviewed the medical files of two L-HES patients followed in our center who developed serious vascular complications and performed a literature review for similar cases.

Pediatric pulmonary arterial hypertension due to a novel homozygous GDF2 missense variant affecting BMP9 processing and activity.

Pulmonary arterial hypertension (PAH) is a rare and severe disorder characterized by progressive pulmonary vasculopathy. Growth differentiation factor (GDF)2 encodes the pro-protein bone morphogenetic protein (BMP) 9, activated after cleavage by endoproteases into an active mature form. BMP9, together with BMP10, are high-affinity ligands of activin receptor-like kinase 1 (ALK1) and BMP receptor type II (BMPR2).

Right Heart Adaptation to Exercise in Pulmonary Hypertension: An Invasive Hemodynamic Study.

Background: Right heart failure (RHF) is associated with a dismal prognosis in patients with pulmonary hypertension (PH). Exercise right heart catheterization may unmask right heart maladaptation as a sign of RHF. We sought to (1) define the normal limits of right atrial pressure (RAP) increase during exercise; (2) describe the right heart adaptation to exercise in PH owing to heart failure with preserved ejection fraction (PH-HFpEF) and in pulmonary arterial hypertension (PAH); and (3) identify the factors associated with right heart maladaptation during exercise.

The cholinergic anti-inflammatory pathway inhibits inflammation without lymphocyte relay.

The magnitude of innate inflammatory immune responses is dependent on interactions between peripheral neural and immune cells. In particular, a cholinergic anti-inflammatory pathway (CAP) has been identified in the spleen whereby noradrenaline (NA) released by splenic nerves binds to ß2-adrenergic receptors (β2-AR) on CD4 T cells which, in turn, release acetylcholine (ACh). The binding of ACh to α7 acetylcholine receptors (α7-AChR) expressed by splenic macrophages inhibits the production of inflammatory cytokines, including tumor necrosis factor (TNF).

An updated meta-analysis of hemodynamics markers of prognosis in patients with pulmonary hypertension due to left heart disease.

Pulmonary hypertension (PH) is associated with a poor prognosis in left heart disease (LHD). We sought to provide an updated analysis on the association of hemodynamic variables, such as pulmonary vascular resistance (PVR), pulmonary artery compliance (PAC), and diastolic pressure gradient (DPG), with prognosis in PH-LHD, through a systematic literature review. Sixteen articles were identified, including 9600 patients with LHD, heterogeneous in terms of age, sex, and etiology of cardiac disease.

Impact of COVID-19 lockdown on exercise capacity in PAH patients.

The outbreak of novel coronavirus-19 disease (COVID-19) was classified as a global pandemic thanks to the rapid viral spread, and restrictive policy measures of infection containment, including "lockdown" periods and self-isolation, were first instituted in Belgium from March to June 2020. The consequent reduction in physical activity could have a negative impact on exercise capacity, especially in frail patients with pre-existing chronic diseases, such as pulmonary arterial hypertension (PAH). With the aim to define the impact of COVID-19 lockdown on functional status, we included in our observational analysis clinically stable PAH patients, who had performed at least four consecutive 6-min walking tests (6MWT) during 2019-2020, to compare their exercise performance before and after the lockdown.

Exercise haemodynamics in heart failure with preserved ejection fraction: a systematic review and meta-analysis.

Aims: Exercise right heart catheterization (RHC) is considered the gold-standard test to diagnose heart failure with preserved ejection fraction (HFpEF). However, exercise RHC is an insufficiently standardized technique, and current haemodynamic thresholds to define HFpEF are not universally accepted. We sought to describe the exercise haemodynamics profile of HFpEF cohorts reported in literature, as compared with control subjects.

Early-onset and severe pulmonary arterial hypertension due to a novel compound heterozygous association of rare mutations: A case report and review of existing data.

Very rare cases of pulmonary arterial hypertension (PAH) have been linked to homozygous or compound heterozygous von Hippel-Lindau () tumor suppressor gene mutations, while heterozygous mutations lead to VHL tumor syndrome. Although those entities are defined, the genotype-phenotype correlation is incompletely understood, and patient management recommendations are lacking. Here, we describe a case of severe early-onset PAH due to a so-far unreported compound heterozygous association of mutations and review the existing data.

Summary of 2020 ESC guidelines on non-STE ACS, adult congenital heart disease, sports cardiology and atrial fibrillation.

During the ESC congress in September 2020, the new ESC guidelines were presented and are available on the ESC website. The new guidelines describe management recommendations on following cardiovascular diseases: non-STE ACS, adult congenital heart disease, sports cardiology and atrial fibrillation. The present document gives a summary of these guidelines and highlights the most important recommendations and changes in the management of these diseases.

Hypereosinophilic syndrome: considerations for the cardiologist.

Eosinophil-mediated endomyocardial damage is a well-known complication in patients with hypereosinophilic syndromes (HES). Although management and survival have improved significantly, some patients continue to develop severe cardiomyopathy as a direct consequence of uncontrolled hypereosinophilia. Cardiologists play a key role in early detection and treatment.

Current Limitations of Invasive Exercise Hemodynamics for the Diagnosis of Heart Failure With Preserved Ejection Fraction.

Background: Exercise hemodynamics can differentiate heart failure with preserved ejection fraction (HFpEF) from noncardiac dyspnea. However, respiratory pressure swings may impact hemodynamic measurements, potentially leading to misdiagnosis of HFpEF. Moreover, threshold values for abnormal hemodynamic response indicative of HFpEF are not universally accepted.

Pregnancy outcome in thoracic aortic disease data from the Registry Of Pregnancy And Cardiac disease.

Background: Cardiovascular disease is the leading cause of death during pregnancy with thoracic aortic dissection being one of the main causes. Thoracic aortic disease is commonly related to hereditary disorders and congenital heart malformations such as bicuspid aortic valve (BAV). Pregnancy is considered a high risk period in women with underlying aortopathy.

Feasibility and cost of FH cascade screening in Belgium (BEL-CASCADE) including a novel rapid rule-out strategy.

Background: Familial hypercholesterolaemia (FH) is underdiagnosed in most countries. We report our first experience from a national pilot project of cascade screening in relatives of FH patients.

Methodology: Participating specialists recruited consecutive index patients (IP) with Dutch Lipid Clinic Network (DLCN) score ≥6.

Genetic counselling and testing in adults with congenital heart disease: A consensus document of the ESC Working Group of Grown-Up Congenital Heart Disease, the ESC Working Group on Aorta and Peripheral Vascular Disease and the European Society of Human Genetics.

Thanks to a better knowledge of the genetic causes of many diseases and an improvement in genetic testing techniques, genetics has gained an important role in the multidisciplinary approach to diagnosis and management of congenital heart disease and aortic pathology. With the introduction of strategies for precision medicine, it is expected that this will only increase further in the future. Because basic knowledge of the indications, the opportunities as well as the limitations of genetic testing is essential for correct application in clinical practice, this consensus document aims to give guidance to care-providers involved in the follow-up of adults with congenital heart defects and/or with hereditary aortic disease.

[Left ventricular hypertrophy : how to identity the cause ?].

Left ventricular hypertrophy (LVH) is defined by an increase in left ventricular mass. LVH can be adaptive and related to an increase in left ventricular pressure or volume load, or can be related to a primary myocardial disease including sarcomeric, inflammatory or infiltrative disorders. The prevalence of left ventricular hypertrophy increases with age, and its presence is a risk factor for cardiovascular events and death.