Treatment algorithm for pulmonary arterial hypertension.

Pulmonary arterial hypertension leads to significant impairment in haemodynamics, right heart function, exercise capacity, quality of life and survival. Current therapies have mechanisms of action involving signalling one of four pathways: endothelin-1, nitric oxide, prostacyclin and bone morphogenetic protein/activin signalling. Efficacy has generally been greater with therapeutic combinations and with parenteral therapy compared with monotherapy or nonparenteral therapies, and maximal medical therapy is now four-drug therapy.

Diagnostic pitfalls in patients referred for arrhythmogenic right ventricular cardiomyopathy.

Background: The diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) is challenging because of nonspecific clinical findings and lack of conclusive answers from genetic testing (ie, an ARVC-related variant is neither necessary nor sufficient for diagnosis). Despite the revised 2010 Task Force Criteria, patients are still misdiagnosed with ARVC.

Objective: In patients referred for ARVC, we sought to identify the clinical characteristics and diagnostic confounders for those patients in whom ARVC was ultimately ruled out.

Antiarrhythmic Drug Therapy in Arrhythmogenic Right Ventricular Cardiomyopathy.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable progressive myocardial disorder that predisposes patients to ventricular arrhythmias and sudden cardiac death. Antiarrhythmic medications have an important role in reducing the frequency of ventricular arrhythmias and the morbidity associated with recurrent implantable cardioverter-defibrillator (ICD) shocks. Although several studies have examined the use of antiarrhythmic drugs in ARVC, these have been mostly retrospective in nature and inconsistent in their methodology, patient population and endpoints.

Eicosapentaenoic acid vs. docosahexaenoic acid for the prevention of cardiovascular disease.

Purpose Of Review: Populations with greater fatty fish intake have lower risk of coronary heart disease. However, trials testing omega-3 fatty acids (FA) on cardiovascular outcomes have yielded inconsistent results. In this review, we summarize the major cardiovascular trials examining omega-3 FA supplementation, and compare differences with eicosapentaenoic acid (EPA) alone vs.

Multimodality Imaging in the Detection of Ischemic Heart Disease in Women.

Women with coronary artery disease tend to have a worse short and long-term prognosis relative to men and the incidence of atherosclerotic cardiovascular disease is increasing. Women are less likely to present with classic anginal symptoms when compared with men and more likely to be misdiagnosed. Several non-invasive imaging modalities are available for diagnosing ischemic heart disease in women and many of these modalities can also assist with prognostication and help to guide management.

New Strategies for Lowering Low Density Lipoprotein Cholesterol for Cardiovascular Disease Prevention.

Purpose Of Review: The primary and secondary prevention of atherosclerotic cardiovascular disease (ASCVD) relies on optimizing cardiovascular health and appropriate pharmacotherapy, a mainstay of which is low-density lipoprotein-cholesterol (LDL-C) lowering. Typically, statin therapy remains the first line approach. Advances in technology and understanding of lipid metabolism have facilitated the development of several novel therapeutic targets and medications within the last decade.

Nondominant Right Coronary Artery Occlusion: Small Vessel, Dramatic Sequelae.

Nondominant right coronary artery (NDRCA) occlusion is rare and generally affects a small volume of myocardium. Despite this, NDRCA occlusion can result in dramatic clinical sequelae. These cases demonstrate the characteristic electrocardiographic findings and consequences of NDRCA occlusion, highlighting the importance of recognition of this pathologic condition to institute appropriate management.

Characteristics of chronic thromboembolic pulmonary hypertension in Ireland.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and under-recognised complication of acute pulmonary embolism. Information regarding the characteristics of CTEPH in Ireland is limited, and the aim of this retrospective cohort study was to address this knowledge gap. Seventy-two cases of CTEPH were diagnosed in the National Pulmonary Hypertension Unit (NPHU) in Ireland between 2010 and 2020.

An interesting case of progressive dyspnoea and diffuse mediastinal adenopathy in a 25-year-old man.

https://bit.ly/3cfe9pX.

Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertension.

Pulmonary arterial hypertension is a rare disease of the pulmonary vasculature, characterised pathologically by proliferation, remodelling and thrombosis . Unfortunately, existing therapeutic interventions do not reverse these findings and the disease continues to result in significant morbidity and premature mortality. A number of haematological derangements have been described in pulmonary arterial hypertension which may provide insights into the pathobiology of the disease and opportunities to explore new therapeutic pathways.

Pulmonary hypertension in chronic lung disease and hypoxia.

Pulmonary hypertension (PH) frequently complicates the course of patients with various forms of chronic lung disease (CLD). CLD-associated PH (CLD-PH) is invariably associated with reduced functional ability, impaired quality of life, greater oxygen requirements and an increased risk of mortality. The aetiology of CLD-PH is complex and multifactorial, with differences in the pathogenic sequelae between the diverse forms of CLD.

Recent advances in targeting the prostacyclin pathway in pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a severe disease characterised by increased pulmonary vascular resistance, which leads to restricted pulmonary arterial blood flow and elevated pulmonary arterial pressure. In patients with PAH, pulmonary concentrations of prostacyclin, a prostanoid that targets several receptors including the IP prostacyclin receptor, are reduced. To redress this balance, epoprostenol, a synthetic prostacyclin, or analogues of prostacyclin have been given therapeutically.

[Treatment goals of pulmonary hypertension].

With significant therapeutic advances in the field of pulmonary arterial hypertension, the need to identify clinically relevant treatment goals that correlate with long-term outcome has emerged as 1 of the most critical tasks. Current goals include achieving modified New York Heart Association functional class I or II, 6-min walk distance >380 m, normalization of right ventricular size and function on echocardiograph, a decreasing or normalization of B-type natriuretic peptide (BNP), and hemodynamics with right atrial pressure <8 mm Hg and cardiac index >2.5 L/dk/m2.

Treatment goals of pulmonary hypertension.

With significant therapeutic advances in the field of pulmonary arterial hypertension, the need to identify clinically relevant treatment goals that correlate with long-term outcome has emerged as 1 of the most critical tasks. Current goals include achieving modified New York Heart Association functional class I or II, 6-min walk distance >380 m, normalization of right ventricular size and function on echocardiograph, a decreasing or normalization of B-type natriuretic peptide (BNP), and hemodynamics with right atrial pressure <8 mm Hg and cardiac index >2.5 mg/kg/min(2).

Detection of high-sensitivity troponin in outpatients with stable pulmonary hypertension identifies a subgroup at higher risk of adverse outcomes.

Background: The detection of elevations in cardiorenal biomarkers, such as troponins, B-type natriuretic peptides (BNPs), and neutrophil gelatinase-associated lipocalins, are associated with poor outcomes in patients hospitalized with acute heart failure. Less is known about the association of these markers with adverse events in chronic right ventricular dysfunction due to pulmonary hypertension, or whether their measurement may improve risk assessment in the outpatient setting.

Methods And Results: We performed a cohort study of 108 patients attending the National Pulmonary Hypertension Unit in Dublin, Ireland, from 2007 to 2009.

Percutaneous atrial septostomy with modified butterfly stent and intracardiac echocardiographic guidance in a patient with syncope and refractory pulmonary arterial hypertension.

Purpose: Syncope is associated with poor prognosis in patients with pulmonary hypertension. Atrial septostomy improves cardiac index and functional class in appropriately selected patients with pulmonary hypertension, and has been shown to improve syncope. One of the major challenges to its effectiveness is maintaining septostomy patency.

Management of pulmonary arterial hypertension.

Purpose Of Review: Pulmonary arterial hypertension (PAH) is a complex disease with a high mortality. Management of this disease is underpinned by supportive and general therapies delivered by multidisciplinary teams in specialist centres. In recent years, a number of PAH-specific therapies have improved patient outcomes.

Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry.

Background: Originally reported to occur predominantly in younger women, idiopathic pulmonary arterial hypertension (IPAH) is increasingly diagnosed in elderly patients. We aimed to describe the characteristics of such patients and their survival under clinical practice conditions.

Methods: Prospective registry in 28 centers in 6 European countries.

Medical treatment of pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a sinister condition that is often diagnosed late and is associated with a progressive clinical deterioration and premature death. However, over the past 10 years the field has seen a dramatic increase in the number of effective therapies underpinned by advances in our understanding of the pathobiology of this illness. Identification of dysfunctional signaling pathways in the pulmonary circulation provided novel therapeutic targets with the goal of reducing pulmonary vascular resistance and prevention of death from right ventricular failure.

End points and clinical trial design in pulmonary arterial hypertension.

New and emerging therapies might provide benefit in patients with pulmonary arterial hypertension. Their efficacy and safety will be compared with existing combination therapies in randomized clinical trials. Appropriate end points for these trials need to be identified: these will include exercise testing, the composite end point of time to clinical worsening, and hemodynamic markers, including advanced imaging modalities and biomarkers.

Updated clinical classification of pulmonary hypertension.

The aim of a clinical classification of pulmonary hypertension (PH) is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH initially adopted in 1998 during the 2nd World Symposium was slightly modified. During the 4th World Symposium held in 2008, it was decided to maintain the general architecture and philosophy of the previous clinical classifications.

Poor outcomes associated with drainage of pericardial effusions in patients with pulmonary arterial hypertension.

Objectives: Pulmonary arterial hypertension (PAH) in its advanced stages is complicated by right heart failure and often pericardial effusion. The optimal treatment of large or hemodynamically significant pericardial effusions in this group has not been defined.

Methods: All patients followed at the Johns Hopkins Hospital for PAH during a 1-year period that underwent pericardiocentesis or pericardial window placement were identified.