Advances in pulmonary hypertension最新文献_第10页

PH Professional Network: Comprehensive Evaluation and Ongoing Approach to Children With Down Syndrome Who Have Pulmonary Hypertension or Are at Risk of Developing Pulmonary Hypertension PH专业网络：对患有肺动脉高压或有发展为肺动脉高压风险的唐氏综合症儿童的综合评估和持续治疗方法

Advances in pulmonary hypertension Pub Date : 2019-03-01 DOI: 10.21693/1933-088X-18.1.37

Traci Housten, E. Jackson, A. Davis

{"title":"PH Professional Network: Comprehensive Evaluation and Ongoing Approach to Children With Down Syndrome Who Have Pulmonary Hypertension or Are at Risk of Developing Pulmonary Hypertension","authors":"Traci Housten, E. Jackson, A. Davis","doi":"10.21693/1933-088X-18.1.37","DOIUrl":"https://doi.org/10.21693/1933-088X-18.1.37","url":null,"abstract":"Children with Down syndrome and pulmonary hypertension (PH) are a unique and challenging group of patients. Down syndrome, or Trisomy 21, affects approximately one in every 600 to 800 live births. PH, currently defined as a resting mean pulmonary artery pressure of ≥25 mm Hg, is known to increase morbidity and mortality significantly in this group of patients and has been identified in as many as 28% of all patients with Down syndrome. Furthermore, specific risk factors and comorbidities have been shown to increase the chance of developing PH in this population. Careful screening and proper treatment is imperative in children with Down syndrome to prevent the development, recurrence, or progression of PH in this population. Recent findings from Bush et al demonstrate clear clinical characteristics and risk factors for development of PH in children with Down syndrome. Prior to this study, data regarding the incidence of PH throughout the Down syndrome lifespan, associations with comorbidities, exacerbating factors, and overall impact of PH in the Down syndrome population were lacking. Most notably, perhaps, was their finding that a vast majority (87%) of patients who suffered from recurrent PH after a previous resolution were classified as World Health Organization (WHO) Group 3 or associated with lung disease. The study also demonstrated that obstructive sleep apnea (OSA), recurrent hypoxia, and aspiration are clear risk factors for development or recurrence of PH. Given these findings, we as providers must take an organized approach in screening for potentially preventable lung insults that contribute to the development and further progression of PH.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48040568","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 3

Pulmonary Hypertension Roundtable: The Crossover From Child to Adult With PH and Congenital Heart Disease 肺动脉高压圆桌会议：从儿童到成人PH和先天性心脏病的转变

Advances in pulmonary hypertension Pub Date : 2019-03-01 DOI: 10.21693/1933-088x-18.1.25

引用次数: 0

Pressure vs Flow-Induced Pulmonary Hypertension 压力与流量诱发的肺动脉高压

Advances in pulmonary hypertension Pub Date : 2019-03-01 DOI: 10.21693/1933-088X-18.1.19

J. Fineman, S. Black

{"title":"Pressure vs Flow-Induced Pulmonary Hypertension","authors":"J. Fineman, S. Black","doi":"10.21693/1933-088X-18.1.19","DOIUrl":"https://doi.org/10.21693/1933-088X-18.1.19","url":null,"abstract":"The pathophysiology of pulmonary hypertension (PH) is multifactorial, complex, and incompletely understood. However, it is known that abnormal mechanical forces within the pulmonary vasculature participate in the disease process. The pulmonary vasculature is continually exposed to hemodynamic forces that include: (1) shear stress, the tangential friction force acting on the vessel wall due to blood flow; (2) hydrostatic pressure, the perpendicular force acting on the vascular wall; and (3) cyclic strain, the circumferential stretch of the vessel wall. Mechanosensors on pulmonary vascular endothelial cells detect these forces and transduce them into biochemical signals that trigger vascular responses (Figure 1). Among the various force-induced signaling molecules, nitric oxide (NO), reactive oxygen species (ROS), and endothelin-1 (ET-1) have been implicated in vascular health and disease. For example, increases in physiologic shear stress associated with increased cardiac output (ie, during exercise) result in induction of NO production with decreased ROS and ET-1, facilitating pulmonary vasodilation and increased flow. However, the pathologic pulmonary vasculature may induce supraphysiologic levels of shear stress, pressure, and cyclic strain resulting in decreased NO with increased ROS and ET-1. Thus, abnormal hemodynamic forces develop in and participate in the disease progression of most forms of pulmonary vascular disease (PVD). However, the influence of hemodynamic forces in the pathobiology of PVD is most clearly demonstrated in patients with PH secondary to congenital heart disease (CHD).","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2019-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43406948","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4

Pulmonary Vascular Disease in the Single-Ventricle Patient: Is it Really Pulmonary Hypertension and if So, How and When Should We Treat it? 单心室患者的肺血管疾病:真的是肺动脉高压吗?如果是，我们应该如何以及何时治疗?

Advances in pulmonary hypertension Pub Date : 2019-03-01 DOI: 10.21693/1933-088X-18.1.14

Stephanie S. Handler, J. Feinstein

引用次数: 2

PH Grand Rounds: Interferon-Associated Pulmonary Arterial Hypertension PH大循环：干扰素相关性肺动脉高压

Advances in pulmonary hypertension Pub Date : 2018-11-01 DOI: 10.21693/1933-088X-17.3.123

F. Shah, A. Duarte

引用次数: 0

Ask the Expert: What Is the Association Between Thyroid Disease and Pulmonary Hypertension: Do Pulmonary Arterial Hypertension Patients Need a Closer Look? 问专家:甲状腺疾病和肺动脉高压之间的关系是什么?肺动脉高压患者需要仔细观察吗?

Advances in pulmonary hypertension Pub Date : 2018-11-01 DOI: 10.21693/1933-088X-17.3.121

S. Studer, C. King, O. Shlobin

引用次数: 0

Metabolomic Insights in Pulmonary Arterial Hypertension 肺动脉高压的代谢组学研究

Advances in pulmonary hypertension Pub Date : 2018-11-01 DOI: 10.21693/1933-088X-17.3.103

C. Rhodes, J. Wharton, M. Wilkins

引用次数: 2

PH Professional Network: The Burden of Prior Authorization for Pulmonary Hypertension Medications: A Practical Guide for Managing the Process PH专业网络：肺动脉高压药物事先授权的负担：管理过程的实用指南

Advances in pulmonary hypertension Pub Date : 2018-11-01 DOI: 10.21693/1933-088X-17.3.126

Traci Housten, Anna M. Brown

{"title":"PH Professional Network: The Burden of Prior Authorization for Pulmonary Hypertension Medications: A Practical Guide for Managing the Process","authors":"Traci Housten, Anna M. Brown","doi":"10.21693/1933-088X-17.3.126","DOIUrl":"https://doi.org/10.21693/1933-088X-17.3.126","url":null,"abstract":"Medications for pulmonary hypertension (PH) are expensive and often require prior authorization from insurance payers. The task of submitting prior authorization requests and appealing denials can burden PH practices with a heavy workload and delay or interrupt medical treatment. However, it is possible to reduce this burden, improve success rates, and reduce waiting times by implementing a standard office workflow for managing the prior authorization process. Such a system involves several key components: assessment of existing staff and level of expertise; dedicated office staff to oversee the process from start to finish; streamlined gathering, storage, and transmittal of patient documents; direct communication with pharmacies and Risk Evaluation Mitigation Strategy programs; and careful documentation of PH diagnosis and treatment plans for a given patient, aimed at reducing the necessity for appeals. This article reviews prior authorization strategies and systems used at PH clinics, and case studies in other therapeutic areas that demonstrate how such systems can reduce staff time and waiting time for initiation of medications while improving the rate of success. The article also describes the special challenges of requesting prior authorization for PH medications prescribed to pediatric patients.","PeriodicalId":92747,"journal":{"name":"Advances in pulmonary hypertension","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2018-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42289955","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 4

Clinical Trials Targeting Metabolism in Pulmonary Arterial Hypertension 针对肺动脉高压代谢的临床试验

Advances in pulmonary hypertension Pub Date : 2018-11-01 DOI: 10.21693/1933-088X-17.3.110

E. Brittain

引用次数: 1

Pulmonary Hypertension Roundtable: Metabolic Disease and PH 肺动脉高压圆桌会议:代谢性疾病和PH值

Advances in pulmonary hypertension Pub Date : 2018-11-01 DOI: 10.21693/1933-088x-17.3.115

A. Hemnes, R. Zamanian

引用次数: 0