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The European Respiratory Journal Jan 2019The function of the right ventricle determines the fate of patients with pulmonary hypertension. Since right heart failure is the consequence of increased afterload, a... (Review)
Review
The function of the right ventricle determines the fate of patients with pulmonary hypertension. Since right heart failure is the consequence of increased afterload, a full physiological description of the cardiopulmonary unit consisting of both the right ventricle and pulmonary vascular system is required to interpret clinical data correctly. Here, we provide such a description of the unit and its components, including the functional interactions between the right ventricle and its load. This physiological description is used to provide a framework for the interpretation of right heart catheterisation data as well as imaging data of the right ventricle obtained by echocardiography or magnetic resonance imaging. Finally, an update is provided on the latest insights in the pathobiology of right ventricular failure, including key pathways of molecular adaptation of the pressure overloaded right ventricle. Based on these outcomes, future directions for research are proposed.
Topics: Adaptation, Physiological; Animals; Cardiac Catheterization; Echocardiography; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Pulmonary Circulation; Ventricular Dysfunction, Right; Ventricular Function, Right
PubMed: 30545976
DOI: 10.1183/13993003.01900-2018 -
Pediatric Critical Care Medicine : a... Aug 2016To review the clinical classification, diagnosis, and pathophysiology of pulmonary hypertension in children, emphasizing the role of right ventricular function,... (Review)
Review
OBJECTIVES
To review the clinical classification, diagnosis, and pathophysiology of pulmonary hypertension in children, emphasizing the role of right ventricular function, ventricular interaction, and congenital heart disease in the evolution and progression of disease, as well as management strategies and therapeutic options.
DATA SOURCE
MEDLINE, PubMed.
CONCLUSIONS
Critically ill children with pulmonary hypertension associated with congenital heart disease are a high-risk population. Congenital cardiac defects resulting in either increased pulmonary blood flow or impaired pulmonary venous drainage predispose patients to developing structural and functional aberrations of the pulmonary vasculature. Mortality from pulmonary hypertension is most directly related to right ventricular failure.
Topics: Child; Heart Defects, Congenital; Heart Ventricles; Humans; Hypertension, Pulmonary; Ventricular Dysfunction, Right
PubMed: 27490592
DOI: 10.1097/PCC.0000000000000754 -
European Respiratory Review : An... Dec 2017http://ow.ly/7y3l30gN0hq
http://ow.ly/7y3l30gN0hq
Topics: Animals; Congresses as Topic; Humans; Hypertension, Pulmonary
PubMed: 29263177
DOI: 10.1183/16000617.0120-2017 -
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 -
Methodist DeBakey Cardiovascular Journal 2024Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance.... (Review)
Review
Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance. Females have a higher incidence of PAH, which is reflected globally across registries in the United States, Europe, and Asia. However, despite female predominance, women had better outcomes compared with male patients, a finding that has been labeled the "estrogen paradox." Special considerations should be given to women with PAH regarding sexual health, contraception, family planning, and treatment before, during, and after pregnancy. Pregnant women with PAH should be referred to a pulmonary hypertension care center; a multidisciplinary team approach is recommended, and Cesarean section is the preferred mode of delivery. While pregnancy outcomes have improved over the years with PAH-specific therapy, pregnancy portends a high-risk for those with PAH. Continued research is needed to tailor PAH treatment for women.
Topics: Female; Pregnancy; Humans; Male; Hypertension, Pulmonary; Cesarean Section; Pregnancy Outcome; Familial Primary Pulmonary Hypertension; Hypertension
PubMed: 38495664
DOI: 10.14797/mdcvj.1308 -
Revista Medica de Chile Jul 2016
Topics: Humans; Hypertension, Pulmonary
PubMed: 27661543
DOI: 10.4067/S0034-98872016000700001 -
International Journal of Molecular... Jun 2016Pulmonary hypertension (PH) is defined by a mean pulmonary arterial pressure over 25 mmHg at rest and is diagnosed by right heart catheterization. Among the different... (Review)
Review
Pulmonary hypertension (PH) is defined by a mean pulmonary arterial pressure over 25 mmHg at rest and is diagnosed by right heart catheterization. Among the different groups of PH, pulmonary arterial hypertension (PAH) is characterized by a progressive obstruction of distal pulmonary arteries, related to endothelial cell dysfunction and vascular cell proliferation, which leads to an increased pulmonary vascular resistance, right ventricular hypertrophy, and right heart failure. Although the primary trigger of PAH remains unknown, oxidative stress and inflammation have been shown to play a key role in the development and progression of vascular remodeling. These factors are known to increase DNA damage that might favor the emergence of the proliferative and apoptosis-resistant phenotype observed in PAH vascular cells. High levels of DNA damage were reported to occur in PAH lungs and remodeled arteries as well as in animal models of PH. Moreover, recent studies have demonstrated that impaired DNA-response mechanisms may lead to an increased mutagen sensitivity in PAH patients. Finally, PAH was linked with decreased breast cancer 1 protein (BRCA1) and DNA topoisomerase 2-binding protein 1 (TopBP1) expression, both involved in maintaining genome integrity. This review aims to provide an overview of recent evidence of DNA damage and DNA repair deficiency and their implication in PAH pathogenesis.
Topics: Animals; DNA Damage; DNA Repair; Genes, Mitochondrial; Humans; Hypertension, Pulmonary; Oxidative Stress
PubMed: 27338373
DOI: 10.3390/ijms17060990 -
Journal of the American College of... Dec 2013Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of... (Review)
Review
Pulmonary hypertension (PH) is a rare disease in newborns, infants, and children that is associated with significant morbidity and mortality. In the majority of pediatric patients, PH is idiopathic or associated with congenital heart disease and rarely is associated with other conditions such as connective tissue or thromboembolic disease. Incidence data from the Netherlands has revealed an annual incidence and point prevalence of 0.7 and 4.4 for idiopathic pulmonary arterial hypertension and 2.2 and 15.6 for pulmonary arterial hypertension, respectively, associated with congenital heart disease (CHD) cases per million children. The updated Nice classification for PH has been enhanced to include a greater depth of CHD and emphasizes persistent PH of the newborn and developmental lung diseases, such as bronchopulmonary dysplasia and congenital diaphragmatic hernia. The management of pediatric PH remains challenging because treatment decisions continue to depend largely on results from evidence-based adult studies and the clinical experience of pediatric experts.
Topics: Age Factors; Animals; Child; Humans; Hypertension, Pulmonary; Incidence; Infant; Infant, Newborn; Netherlands
PubMed: 24355636
DOI: 10.1016/j.jacc.2013.10.028 -
International Journal of Molecular... Jan 2024Pulmonary hypertension (PH) is recognized as a pathophysiological disorder encompassing a wide spectrum of clinical conditions related to various cardiovascular and...
Pulmonary hypertension (PH) is recognized as a pathophysiological disorder encompassing a wide spectrum of clinical conditions related to various cardiovascular and respiratory diseases [...].
Topics: Humans; Pulmonary Arterial Hypertension; Familial Primary Pulmonary Hypertension; Hypertension, Pulmonary
PubMed: 38256236
DOI: 10.3390/ijms25021166 -
Tuberkuloz Ve Toraks Sep 2017Schistosomiasis is one of the most prevelant parazitic diseases in the world. It is endemic in more than 70 countries, and more than 200 million people worldwide are... (Review)
Review
Schistosomiasis is one of the most prevelant parazitic diseases in the world. It is endemic in more than 70 countries, and more than 200 million people worldwide are infected with Schistosoma. Pulmonary hypertension (PHT) is one of the chronic complications of schistosomiasis. The exact pathogenesis of schistosomiasis-associated pulmonary hypertension (S-PHT) remains unclear, although several mechanisms such as parazitic arterial embolisation, pulmonary arteriopathy, and portopulmonary hypertension have been suggested. Pathological pulmonary vascular changes in S-PHT were found similar to those in idiopathic pulmonary arterial hypertension (IPAH). The fact that schistosomiasis is one of the most common causes of pulmonary arterial hypertension (PAH), particularly in the developing countries, underlines the importance of enhancing our knowledge on this disease. Developments in the treatment of PAH have resulted in improved prognosis and significant increase in life expectancy and quality of life in the last two decades, which has enhanced the importance of S-PHT. Schistosomiasis is treated with praziquantel. Nevertheless, there is limited evidence that this treatment is effective for PHT. Although antihelmintic medications do not lead to significant improvement, they have beneficial effects and may slow down disease progression. Using PAH-specific treatments in the patients with schistosomiasis-associated PAH (S-PAH) can improve prognosis. However, inadequate clinical studies and limited sources in the endemic regions restrict extensive usage of these expensive medications. Further studies are required to determine the efficacy of these treatment modalities.
Topics: Animals; Developing Countries; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; Lung; Prognosis; Schistosoma mansoni; Schistosomiasis mansoni
PubMed: 29135402
DOI: 10.5578/tt.53798