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In addition, bempedoic acid, lomitapide, and evinacumab are available options that may be instituted in select patients. In development is inclisiran, a small interfering RNA molecule, which antagonizes PCSK9 production. With good adherence and the use of a greater assortment of medications, patients may experience atherogenic lipoprotein lowering, leading to a decrease in cardiovascular disease.Hyperlipidemia is a prevalent condition in the United States and a significant contributor to atherosclerotic cardiovascular disease (ASCVD). ASCVD is a primary cause of morbidity and mortality in the United States. Low-density lipoprotein cholesterol (LDL-C) is a causal factor for the development of ASCVD. Reductions in LDL-C produce a corresponding decrease in ASCVD risk for cardiovascular events. HMG-CoA reductase inhibitors, commonly referred to as statins, remain the gold standard of hyperlipidemia treatment. However, statin monotherapy is often ineffective in reducing LDL-C to treatment guideline-recommended levels, especially in high-risk patients with established ASCVD or familial hypercholesterolemia (FH). Statin therapy causes myalgias in 5% to 10% of patients, which may lead to inadequate dose optimization, nonadherence, or inability to take a statin. Clinical guidelines recommend add-on therapy with ezetimibe and proprotein convertase subtilisin/kexin type 9 (PCSK9) inhibitors when maximally tolerated statin therapy results in suboptimal LDL-C reduction. Hyperlipidemia, especially FH, is associated with substantial clinical and financial burden and is often undertreated. Although undertreatment is partially attributable to failure to optimize statin therapy, a significant portion of patients will require a PCSK9 inhibitor for adequate LDL-C reduction. Despite this, PCSK9 inhibitor utilization rates remain low. Barriers to treatment may include clinical inertia, high out-of-pocket costs, and pharmacy benefit access issues. Managed care pharmacists can help appropriate patients overcome these barriers to PCSK9 inhibitor use and improve the attainment of LDL-C goals and outcomes, especially in high-risk patients with FH or clinical ASCVD.Pulmonary arterial hypertension (PAH) is a rare, progressive disorder associated with a poor prognosis if not treated appropriately. Fortunately, new treatment options have significantly improved survival rates and prognosis. Despite these advances, many patients do not receive the diagnosis until years into their disease or are inappropriately diagnosed. Early referral to specialized treatment centers that allows for early diagnosis and initiation of treatment significantly improves patient outcomes including survival as well as reduction in hospital admissions, which are a main driver of economic burden of disease. It is important that evidence-based guidelines are followed and treatment is individualized based on patient-specific factors. Pharmacologic therapies carry a very high cost for PAH; however, extensive utilization of management strategies may hinder access to medication and may lead to disease progression. Cost containment strategies may help to facilitate care coordination for earlier diagnosis and initiation of treatment, adherence to PAH medications, and patient education to ensure they are using medications appropriately to optimize therapy. Managed care pharmacists can play a crucial role in the multidisciplinary team in terms of medication safety, adherence, patient education, and follow-up to improve patient engagement that leads to improved outcomes.Pulmonary arterial hypertension (PAH) is a severe disease with poor prognosis and shortened life expectancy. Treatment has traditionally involved the sequential use of endothelin receptor agonists, prostacyclin therapies, and nitric oxide pathway modulators, which each have distinct mechanisms of action leading to pulmonary vasodilation, and improvement in exercise capacity, hemodynamic measures, and clinical outcomes for patients with PAH. This article provides a review of goals of therapy in PAH, determinants of prognosis and levels of patient risk, and additional factors that guide treatment decision making. Recent research in combination therapies has created a paradigm shift in the treatment of PAH and will be reviewed. Additionally, recent updates to the American College of Chest Physicians guidelines will be reviewed along with the updated evidence-based treatment algorithm. Finally, trial data will be evaluated for the recently developed agent selexipag and improved treprostinil delivery formulations that may provide enhanced convenience.Group 1 pulmonary hypertension (or pulmonary arterial hypertension) is a rare, highly complex, and progressive disorder that is incurable and ultimately can lead to premature death. PAH causes significant physical, social, work, and emotional burdens among affected patients and their caregivers. Early diagnosis and initiation of treatment is required for best outcomes; however, the clinical presentation of PAH is nonspecific and frequently overlaps with several other conditions, often leading to a delay in diagnosis or misdiagnosis. In the past decades, increased understanding of the pathobiology of PAH has led to changes in its definition. Additionally, contemporary PAH registries have shown greater survival rates among patients with PAH and have allowed for the development of risk calculator tools that are now used to drive therapeutic goals. https://www.selleckchem.com/products/epz-5676.html To date, multiple PAH-specific therapies have been developed, and all currently target one of 3 pathways that contribute to the endothelial dysfunction pathogenesis of PAH (prostacyclin, endothelin, and nitric oxide pathways). Because PAH is classified into 7 subgroups, it is essential that individuals are grouped appropriately for the efficacy of treatment and avoidance of harm. As health-related quality of life for PAH is multifactorial, it is important that patients are involved in the clinical decision-making process and have access to multidisciplinary care. The purpose of this review is to update healthcare professionals on the management of PAH with the most current information on epidemiology, pathophysiology, clinical presentation, and diagnostic considerations.
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