Diagnosis and Management of Pulmonary Hypertension: New Insights.

Over the last decades, significant progress has been achieved in the pulmonary hypertension (PH) field. Pathophysiology of PH has been studied, leading to the classification of PH patients into five groups, while the hemodynamic definition has been recently revised. A diagnostic algorithm has been established and awareness has been raised in order to minimize diagnosis delay.

Multi-modality imaging of the systemic right ventricle in congenital heart disease.

A comprehensive and structured imaging approach in the evaluation of the systemic right ventricle (sRV) in patients with complete transposition of the great arteries (TGA) after atrial switch procedure and congenitally corrected transposition of the great arteries (ccTGA) is a key for their optimal lifelong surveillance. Despite the improvements in cardiovascular imaging of adults with congenital heart disease (ACHD), the imaging of sRV remains an ongoing challenge due to its complex morphology and the difficulty in applying the existing knowledge for the systemic left ventricle. While cardiac magnetic resonance (CMR) is considered the gold standard imaging method, echocardiographic evaluation is primarily preferred in everyday clinical setting.

Arrhythmias in Patients with Congenital Heart Disease: An Ongoing Morbidity.

With the aging of congenital heart disease (CHD) patients, the burden of arrhythmias is expanding. Atrial arrhythmias, especially intra-atrial reentrant tachycardia and atrial fibrillation, are the most prevalent forms of arrhythmia. Managing comorbidities, such as obesity, using pharmacotherapy, including antiarrhythmics and anticoagulants, and ablation therapy has become the cornerstone of arrhythmia management.

Focused ultrasound neuromodulation of the spleen activates an anti-inflammatory response in humans.

Focused ultrasound stimulation (FUS) activates mechanosensitive ion channels and is emerging as a method of noninvasive neuromodulation. In preclinical studies, FUS of the spleen (sFUS) activates an anti-inflammatory neural pathway which suppresses acute and chronic inflammation. However, the relevance of sFUS for regulating inflammatory responses in humans is unknown.

Association of Health Status Metrics with Clinical Outcomes in Patients with Adult Congenital Heart Disease and Atrial Arrhythmias.

The prognostic value of health status metrics in patients with adult congenital heart disease (ACHD) and atrial arrhythmias is unclear. In this retrospective cohort study of an ongoing national, multicenter registry (PROTECT-AR, NCT03854149), ACHD patients with atrial arrhythmias on apixaban are included. At baseline, health metrics were assessed using the physical component summary (PCS), the mental component summary (MCS) of the Short-Form-36 (SF-36) Health Survey, and the modified European Heart Rhythm Association (mEHRA) score.

The Adult Congenital Heart Disease Anatomic and Physiological Classification: Associations with Clinical Outcomes in Patients with Atrial Arrhythmias.

The implications of the adult congenital heart disease anatomic and physiological classification (AP-ACHD) for risk assessment have not been adequately studied. A retrospective cohort study was conducted using data from an ongoing national, multicentre registry of patients with ACHD and atrial arrhythmias (AA) receiving apixaban (PROTECT-AR study, NCT03854149). At enrollment, patients were stratified according to Anatomic class (AnatC, range I to III) and physiological stage (PhyS, range B to D).

Prediction Models and Scores in Adult Congenital Heart Disease.

Nowadays, most patients with congenital heart disease survive to adulthood due to advances in pediatric cardiac surgery but often present with various comorbidities and long-term complications, posing challenges in their management. The development and clinical use of risk scores for the prediction of morbidity and/or mortality in adults with congenital heart disease (ACHD) is fundamental in achieving optimal management for these patients, including appropriate follow-up frequency, treatment escalation, and timely referral for invasive procedures or heart transplantation. In comparison with other fields of cardiovascular medicine, there are relatively few studies that report prediction models developed in the ACHD population, given the small sample size, heterogeneity of the population, and relatively low event rate.

Rationale and design of a prospective, observational, multicentre study on the safety and efficacy of apixaban for the prevention of thromboembolism in adults with congenital heart disease and atrial arrhythmias: the PROTECT-AR study.

Introduction: The risk for stroke in adults with congenital heart disease (ACHD) is increased, especially in the setting of commonly ensuing atrial arrhythmias (AA), namely atrial fibrillation, atrial flutter or intra-atrial re-entrant tachycardia. Data are limited regarding treatment with non-vitamin K oral anticoagulants in long-term studies involving patients with ACHD and AA.

Methods And Analysis: PReventiOn of ThromboEmbolism in Adults with Congenital HearΤ disease and Atrial aRrhythmias is a prospective, multicenter, single-arm, non-interventional cohort study designed to investigate the safety and efficacy of apixaban for the prevention of thromboembolism in ACHD with AA in a 'real-world' setting.

Hospitalizations in adult patients with congenital heart disease: an emerging challenge.

The vast majority of patients with congenital heart disease (CHD) survive into adulthood, but many face lifelong complications, which often result in a hospital admission. The increasing number of hospitalizations in adults with CHD (ACHD) poses a significant challenge for healthcare systems globally, especially as heart failure (HF) is becoming increasingly common in this population and is the leading cause of morbidity and mortality. Besides HF, other major contributors to this increase in admission volume are hospitalizations related to mild lesions, comorbidities and pregnancies.

How to evaluate patients with congenital heart disease-related pulmonary arterial hypertension.

Patients with congenital heart disease (CHD), who develop pulmonary arterial hypertension (PAH), live longer, and have better quality of life compared to the past due to PAH-specific therapy and improved tertiary care. Areas covered: Clinical examination, objective assessment of functional capacity, natriuretic peptide levels, cardiac imaging, and hemodynamics all play a pivotal role in the evaluation, general care, and management of PAH-specific therapy. This review discusses the epidemiology and pathophysiology of PAH-CHD and provides hints for the optimal evaluation of these patients.

Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease.

Objective: Despite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.

Methods: Data were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.