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Methodist DeBakey Cardiovascular Journal 2021Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect.... (Review)
Review
Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect. Pulmonary arterial hypertension (PAH) has a multifactorial etiology depending on the size and nature of the cardiac defect as well as environmental factors. Although progress has been made in disease-targeting therapy using pulmonary vasodilators to treat Eisenmenger syndrome, important gaps still exist in the evaluation and management of adult patients with CHD-associated PAH (PAH-CHD) who have systemic-to-pulmonary shunts. The choice of interventional, medical, or both types of therapy is an ongoing dilemma that requires further data. This review focuses on the evaluation and management of PAH-CHD in the contemporary era.
Topics: Adult; Eisenmenger Complex; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Lung; Pulmonary Arterial Hypertension
PubMed: 34326934
DOI: 10.14797/UFEJ2329 -
Current Opinion in Pulmonary Medicine Sep 2023The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial... (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis.
RECENT FINDINGS
The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme.
SUMMARY
Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Prognosis; Familial Primary Pulmonary Hypertension; Echocardiography; Ventricular Dysfunction, Right
PubMed: 37410491
DOI: 10.1097/MCP.0000000000000980 -
Methodist DeBakey Cardiovascular Journal 2021A subgroup of patients diagnosed with pulmonary arterial hypertension (PAH) carry transmissible pathogenic gene mutations. For many of these patients, the heritable... (Review)
Review
A subgroup of patients diagnosed with pulmonary arterial hypertension (PAH) carry transmissible pathogenic gene mutations. For many of these patients, the heritable nature of their disease can only be uncovered by genetic testing. Because identification of PAH patients who carry pathogenic gene mutations has important implications for other family members, genetic counseling and testing should be offered to patients diagnosed with idiopathic or familial PAH. This review describes the current state of genetic counseling and testing for patients diagnosed with PAH.
Topics: Bone Morphogenetic Protein Receptors, Type II; Familial Primary Pulmonary Hypertension; Genetic Counseling; Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension
PubMed: 34326929
DOI: 10.14797/ZOQM5771 -
JAMA Oct 2012Pulmonary hypertension (PH), defined as elevated pulmonary artery pressure, is common in the general population and associated with increased mortality. Accordingly,...
Pulmonary hypertension (PH), defined as elevated pulmonary artery pressure, is common in the general population and associated with increased mortality. Accordingly, physicians commonly encounter patients with dyspnea, exercise intolerance, and/or right heart failure who have elevated pulmonary artery systolic pressure (PASP) on echocardiography. Although pulmonary arterial vasodilators may often be considered in this setting, these drugs have been predominantly tested in the subset of PH patients with pulmonary arterial hypertension (PAH). Elevated PASP alone is not sufficient for the diagnosis of PAH, and secondary causes of PASP elevation, most commonly left heart disease, are far more prevalent than isolated PAH. Treatment of this more common group of patients with PH due to left heart disease is challenging because there are few evidence-based treatment options, and pulmonary vasodilator therapy may lead to worsening symptoms. Therefore, improving symptoms and avoiding adverse outcomes in patients with PH requires the following: (1) understanding the optimal use of echocardiography for the diagnosis of PH; (2) recognizing the utility and proper interpretation of invasive hemodynamic testing prior to starting pulmonary vasodilator therapy; (3) differentiating PAH from pulmonary venous hypertension due to left heart disease; and (4) understanding the appropriate treatment strategies for PH and resultant right heart failure.
Topics: Aged; Algorithms; Echocardiography; Familial Primary Pulmonary Hypertension; Female; Humans; Hypertension, Pulmonary; Prognosis; Pulmonary Veins; Vasodilator Agents; Ventricular Dysfunction, Left; Ventricular Function, Right
PubMed: 23032553
DOI: 10.1001/jama.2012.12347 -
CMAJ : Canadian Medical Association... Aug 2016
Review
Topics: Disease Management; Humans; Hypertension, Pulmonary; Practice Guidelines as Topic; Randomized Controlled Trials as Topic; World Health Organization
PubMed: 27141034
DOI: 10.1503/cmaj.151075 -
Methodist DeBakey Cardiovascular Journal 2016Chronic thromboembolic pulmonary hypertension (CTEPH) occurs when thromboemboli travel to the pulmonary vasculature, fail to resolve, and cause elevated pulmonary... (Review)
Review
Chronic thromboembolic pulmonary hypertension (CTEPH) occurs when thromboemboli travel to the pulmonary vasculature, fail to resolve, and cause elevated pulmonary arterial pressure. Untreated, this disease leads to progressive right heart failure and death. It develops in approximately 1% to 5% of patients who suffer an acute pulmonary embolism (PE) and has an overall incidence of 3 to 30 per million in the general population. While it is not entirely evident why most but not all people are able to clear this clot burden, there are known risk factors for the development of CTEPH. These include signs of right heart strain at the time of incident PE, inherited coagulopathies, inflammatory conditions, hypothyroidism, and a history of splenectomy. Since CTEPH can be treated both surgically and medically, it is critical to understand the pathophysiology of the disease so affected patients can be identified and diagnosed appropriately.
Topics: Arterial Pressure; Chronic Disease; Humans; Hypertension, Pulmonary; Incidence; Prognosis; Pulmonary Artery; Pulmonary Embolism; Risk Assessment; Risk Factors
PubMed: 28289493
DOI: 10.14797/mdcj-12-4-195 -
American Journal of Respiratory and... Jun 2022
Topics: Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Pathology, Molecular; Proteome; Pulmonary Arterial Hypertension
PubMed: 35452380
DOI: 10.1164/rccm.202203-0537ED -
Journal of Investigative Medicine : the... Apr 2020Pulmonary hypertension (PH) is a chronic, complex and challenging disease. Advances in treatment are for the subset of patients with pulmonary arterial hypertension.... (Review)
Review
Pulmonary hypertension (PH) is a chronic, complex and challenging disease. Advances in treatment are for the subset of patients with pulmonary arterial hypertension. Selected review of the literature was conducted incorporating the European Society of Cardiology/European Respiratory Society 2015 guidelines and recommendations from the Sixth World Symposium on Pulmonary Hypertension. PH is classified into five groups based on WHO classification. Echocardiography remains the initial test of choice, and careful assessment of the right system aids in the diagnosis and prognosis of the disease. Right heart catheterization remains the gold standard of diagnosis and key guidance of treatment. Multidisciplinary approach is recommended for the care of patients with PH. Treatment selection is based on individual risk stratification of patients, and early referral to specialized PH centers improves outcomes of patients. Treating PH is complex and is best carried out in PH centers and with multidisciplinary approach. Early diagnosis and referral to those centers are key not to delay treatment.
Topics: Electrocardiography; Hemodynamics; Humans; Hypertension, Pulmonary; Prognosis; Risk Assessment; Severity of Illness Index
PubMed: 32241822
DOI: 10.1136/jim-2020-001291 -
Current Hypertension Reports Sep 2014Pulmonary hypertension (PH) is a progressive lung disease characterized by elevated pressure in the lung vasculature, resulting in right-sided heart failure and... (Review)
Review
Pulmonary hypertension (PH) is a progressive lung disease characterized by elevated pressure in the lung vasculature, resulting in right-sided heart failure and premature death. The pathogenesis of PH is complex and multifactorial, involving a dysregulated autonomic nervous system and immune response. Inflammatory mechanisms have been linked to the development and progression of PH; however, these are usually restricted to systemic and/or local lung tissue. Inflammation within the CNS, often referred to as neuroinflammation involves activation of the microglia, the innate immune cells that are found specifically in the brain and spinal cord. Microglial activation results in the release of several cytokines and chemokines that trigger neuroinflammation, and has been implicated in the pathogenesis of several disease conditions such as Alzheimer's, Parkinson's, hypertension, atherosclerosis, and metabolic disorders. In this review, we introduce the concept of neuroinflammation in the context of PH, and discuss possible strategies that could be developed for PH therapy based on this concept.
Topics: Disease Progression; Humans; Hypertension, Pulmonary; Inflammation; Microglia; Pulmonary Wedge Pressure
PubMed: 25090964
DOI: 10.1007/s11906-014-0469-1 -
Expert Review of Cardiovascular Therapy Aug 2016Inflammatory cells are present in the lungs from patients with many, if not all, forms of severe pulmonary hypertension. (Review)
Review
INTRODUCTION
Inflammatory cells are present in the lungs from patients with many, if not all, forms of severe pulmonary hypertension.
AREAS COVERED
Historically the first inflammatory cell identified in the pulmonary vascular lesions was the mast cell. T and B lymphocytes, as well as macrophages, are present in and around the pulmonary arterioles and many patients have elevated blood levels of interleukin 1 and 6; some patients show elevated levels of leukotriene B4. An overlap between collagen-vascular disease-associated pulmonary arterial hypertension (PAH) and idiopathic PAH exists, yet only a few studies have been designed that evaluate the effect of anti-inflammatory treatments. Here we review the pertinent data that connect PAH and inflammation/autoimmune dysregulation and evaluate experimental models of severe PAH with an emphasis on the Sugen/athymic rat model of severe PAH. Expert commentary: We postulate that there are several inflammatory phenotypes and predict that there will be several anti-inflammatory treatment strategies for severe PAH.
Topics: Animals; Anti-Inflammatory Agents; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Inflammation; Lung
PubMed: 27096622
DOI: 10.1080/14779072.2016.1180976