Advances in pulmonary hypertension最新文献

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Genetic Counseling and Testing for Pulmonary Arterial Hypertension in the United States 美国肺动脉高压的遗传咨询和检测

Advances in pulmonary hypertension

Pub Date : 2021-12-01 DOI: 10.21693/1933-088x-20.5.157

Sumathi I. Rachamadugu, M. Emmerson, B. Girerd, D. H. Best

is identified, coordinate segregation patient Consider DNA of uninformative genetic test result. DNA be pretest some circumstances.

经鉴定，配合分离患者考虑无信息基因检测结果的DNA。DNA在某些情况下是预先测试过的。

引用次数: 0

PH Professional Network: Genetic Counseling and Pulmonary Arterial Hypertension PH专业网络：遗传咨询与肺动脉高压

Advances in pulmonary hypertension

Pub Date : 2021-12-01 DOI: 10.21693/1933-088x-20.5.164

Athena Angelopoulos, R. Farrell

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Genotypes and Phenotypes: A Review of Pulmonary Hypertension in Genetic Syndromes 基因型和表型:肺动脉高压遗传综合征的综述

Advances in pulmonary hypertension

Pub Date : 2021-12-01 DOI: 10.21693/1933-088x-20.5.150

Rachel T. Sullivan, E. Austin

There has been significant advancement in the understanding of the genetics of pulmonary hypertension (PH), particularly in those with heritable or idiopathic pulmonary arterial hypertension. In addition to genetic variants with a primarily pulmonary vascular disease phenotype, the prevalence of PH in other genetic syndromes is increasingly recognized. We will review the current knowledge of PH associated with multisystem genetic syndromes. There is high prevalence of coexisting cardiac and pulmonary disease, making it challenging to discern whether PH is secondary to these processes or underlying genetic makeup. There is a paucity of data on response to PH-targeted therapy and implications on overall prognosis.

在理解肺动脉高压（PH）的遗传学方面取得了重大进展，特别是在那些具有遗传性或特发性肺动脉高压的患者中。除了主要具有肺血管疾病表型的遗传变异外，PH在其他遗传综合征中的患病率也越来越得到认可。我们将回顾目前有关PH与多系统遗传综合征的知识。心脏病和肺病并存的患病率很高，因此很难判断PH是继发于这些过程还是潜在的基因构成。关于PH靶向治疗的反应和对整体预后的影响，缺乏数据。

引用次数: 1

PH Professional Network: 360-degree Care for the Bronchopulmonary Dysplasia Infant with Pulmonary Hypertension: A Comprehensive Review PH专业网络：360度护理伴肺动脉高压的支气管肺发育不良婴儿：综述

Advances in pulmonary hypertension

Pub Date : 2021-10-01 DOI: 10.21693/1933-088x-20.4.123

N. Villafranco, E. Whalen, N. Varghese

Premature infants are at risk of developing bronchopulmonary dysplasia and associated pulmonary hypertension. These infants make up a complex group of patients with unique considerations regarding development of lung and vascular disease, comorbidities, and care plans. They are high risk for many complications and poor outcomes due to the severity and complexity of disease. Because of this, a comprehensive approach to care with consideration for multiple organ systems and with an interdisciplinary team of experts is the preferred approach. Here we describe in detail the major considerations in care for these infants.

早产儿有发生支气管肺发育不良和相关肺动脉高压的危险。这些婴儿构成了一个复杂的患者群体，在肺部和血管疾病的发展、合并症和护理计划方面具有独特的考虑。由于疾病的严重性和复杂性，他们有许多并发症和不良预后的高风险。因此，考虑到多器官系统和跨学科专家团队的综合护理方法是首选的方法。在这里，我们详细描述了这些婴儿护理的主要考虑因素。

引用次数: 0

Connective Tissue Disease, Interstitial Lung Disease, and Pulmonary Hypertension (CTD PH-ILD): A Distinct Entity and Potential Opportunity 结缔组织疾病、间质性肺病和肺动脉高压（CTD PH-ILD）：一个独特的实体和潜在的机会

Advances in pulmonary hypertension

Pub Date : 2021-10-01 DOI: 10.21693/1933-088x-20.4.109

D. Sese, K. Highland

Connective tissue diseases are a multisystem disorder that can cause impairments in quality of life, shorten life expectancy, and increase the risk of mortality at a younger age. These patients have an increased risk for the development of pulmonary hypertension through several mechanisms including pulmonary arterial hypertension and pulmonary hypertension associated with interstitial lung disease. This review aims to discuss the various presentations of connective tissue disease associated with pulmonary hypertension associated with interstitial lung disease, demographics, and survival. It gives an overview of accepted mechanisms of disease pathogenesis, discusses advances in diagnostics, and treatment options. Despite a deeper understanding of disease pathogenesis, treatment for this remains limited to prevention of disease progression. The identification of the primary disease driver requires careful evaluation of the disease phenotype and is a potential target for treatment and prevention of death.

结缔组织疾病是一种多系统疾病，可导致生活质量下降，缩短预期寿命，并增加年轻时的死亡率。通过多种机制，包括肺动脉高压和与间质性肺病相关的肺动脉高压，这些患者发展为肺动脉高压的风险增加。这篇综述旨在讨论结缔组织疾病与肺动脉高压相关的各种表现与间质性肺病、人口统计学和生存率。它概述了公认的疾病发病机制，讨论了诊断和治疗选择的进展。尽管对疾病的发病机制有了更深入的了解，但治疗仍然局限于预防疾病进展。确定主要疾病驱动因素需要仔细评估疾病表型，是治疗和预防死亡的潜在目标。

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Management of PH-ILD: Past, Present, and Future 博士学位的管理:过去，现在和未来

Advances in pulmonary hypertension

Pub Date : 2021-10-01 DOI: 10.21693/1933-088x-20.4.119

E. Harder, A. Waxman

Pulmonary hypertension associated with interstitial lung disease signifies worse outcomes. Given previous negative clinical trials, the use of pulmonary vasodilators in pulmonary hypertension associated with interstitial lung disease has traditionally been on a case-by-case basis; however, the recent INCREASE study has led to the first and milestone approval of inhaled treprostinil for this population. This review discusses the management of pulmonary hypertension associated with interstitial lung disease from the pulmonary vascular perspective, with an emphasis on clinical trials in this population.

与间质性肺病相关的肺动脉高压意味着更糟糕的结果。鉴于之前的阴性临床试验，肺血管舒张剂在与间质性肺病相关的肺动脉高压中的使用传统上是根据具体情况进行的；然而，最近的INCREASE研究首次批准吸入曲前列素用于该人群，这也是一个里程碑。这篇综述从肺血管的角度讨论了与间质性肺病相关的肺动脉高压的治疗，重点是在这一人群中的临床试验。

引用次数: 1

PH-ILD: Identification, Evaluation, and Monitoring: A Diagnostic View From Both Sides 博士:鉴定，评估和监测:从双方的诊断观点

Advances in pulmonary hypertension

Pub Date : 2021-10-01 DOI: 10.21693/1933-088x-20.4.103

F. Rahaghi, F. Rahaghi

INTRODUCTION Pulmonary hypertension (PH) has long been recognized as a complication of interstitial lung disease (ILD). It contributes significantly to morbidity and mortality and thus is of key importance in prognostication and deciding the timing of referral for lung transplant. There is increasing evidence of the complexity of its pathogenesis beyond simple fibrosis and hypoxemic vasoconstriction. The pathophysiologic overlap with pulmonary arterial hypertension (PAH) has led to trials of pulmonary vasodilatory therapy in PH-ILD. While prior trials of pulmonary vasodilatory therapy in ILD have presented mixed results, a recent trial of inhaled pulmonary vasodilator therapy in this group has shown positive effect. As a result, the early recognition of the development of PH in ILD may have a greater implication for patients than just prognostication and assessment during considerations for transplant, and may contribute to better outcomes. In this paper we review the current understanding of the pathogenesis of PH in patients with ILD and what is known about the clinical impact of PH in the context of ILD. We then review the importance of hemodynamic assessment to the diagnosis of PH in ILD. Lastly, we review different symptoms, physical exam findings and studies that raise the index of suspicion for the presence of PH in ILD and considerations for incorporating these into initial and subsequent evaluations for patients with ILD. DEFINING PH-ILD While PH can occur in many different contexts in a patient who also has ILD, the implications of labeling an individual as having PH-ILD suggests that ILD is the primary driver of the presence of PH. This can be a subtle distinction: many patients with group 1 PH (PAH), and in particular those with connective tissue disease (CTD), may have a mild form of ILD while also having PAH. Similarly, patients with sarcoidosis may have both ILD and PH while still not being considered as group 3 PH. The understanding of these distinctions is crucial for interpretation of results of clinical studies, which often use such definitions for inclusion or exclusion. Significant history exists in classification of patients with ILD into group 3 PH (PH associated with chronic lung disease) using a combination of hemodynamics and the degree of lung disease. The hemodynamic definition of PH, in the context of chronic lung disease (group 3 PH) was updated in the 6 World Symposium on Pulmonary Hypertension to include a resting mean pulmonary artery pressure of >20 mm Hg, a pulmonary artery occlusion pressure ≤15 mm Hg, and a pulmonary vascular resistance of >3 Wood units. It is important, however, to note hemodynamic definitions do not create a distinction between group 3 and group 1 PH, rather the distinction relies on defining chronic lung disease as the primary driver of precapillary PH. This is done through a combination of pulmonary function testing and imaging—evidence of significant decrement in lung volumes or evidence

引言肺动脉高压（PH）长期以来一直被认为是间质性肺病（ILD）的并发症。它对发病率和死亡率有显著影响，因此在预测和决定肺移植转诊时间方面具有关键重要性。越来越多的证据表明，其发病机制的复杂性超出了单纯的纤维化和低氧性血管收缩。与肺动脉高压（PAH）的病理生理学重叠导致了肺血管舒张治疗PH-ILD的试验。虽然先前在ILD中进行的肺血管舒张治疗试验结果喜忧参半，但最近在该组中进行的吸入性肺血管舒张剂治疗试验显示出了积极的效果。因此，早期认识到ILD中PH的发展可能对患者有更大的影响，而不仅仅是在考虑移植时的预测和评估，并可能有助于更好的结果。在这篇文章中，我们回顾了目前对ILD患者PH发病机制的理解，以及对PH在ILD背景下的临床影响的了解。然后，我们回顾了血液动力学评估对ILD中PH诊断的重要性。最后，我们回顾了不同的症状、体检结果和提高ILD中PH存在的怀疑指数的研究，以及将这些纳入ILD患者的初始和后续评估的考虑因素。定义PH-ILD虽然在患有ILD的患者中，PH可能发生在许多不同的情况下，但将个体标记为患有PH-ILD的含义表明，ILD是PH存在的主要驱动因素。这可能是一个微妙的区别：许多患有第1组PH（PAH）的患者，尤其是那些患有结缔组织病（CTD）的患者，可以具有温和形式的ILD，同时也具有PAH。同样，结节病患者可能同时患有ILD和PH，但仍不被视为第3组PH。了解这些区别对于解释临床研究结果至关重要，临床研究通常使用这些定义进行纳入或排除。根据血流动力学和肺部疾病的程度，将ILD患者分为3组PH（与慢性肺部疾病相关的PH）存在显著的病史。在第6届世界肺动脉高压研讨会上，对慢性肺病（第3组PH）中PH的血液动力学定义进行了更新，包括静息平均肺动脉压>20 mm Hg、肺动脉闭塞压≤15 mm Hg和肺血管阻力>3 Wood单位。然而，重要的是要注意，血液动力学定义并没有在第3组和第1组PH之间产生区别，相反，这种区别依赖于将慢性肺病定义为毛细血管前PH的主要驱动因素。这是通过肺功能测试和成像相结合来完成的——肺容量显著减少的证据或成像上ILD负担显著的证据将患者从第1组转移到第3组。然后，挑战变成了定义“重大ILD负担”。这在CTD等条件下尤其困难，其中PH可以在存在和不存在ILD的情况下存在。如果我们观察大多数PAH试验，接近70%的强迫肺活量（FVC）下限或60%的总肺活量的下限被用作硬截止值，这表明ILD负担较高的患者应被归类为第3组。必须特别注意结节病，结节病通过多种机制导致ILD和PH的发展。目前，结节病引起的PH仍被归类为第5组疾病，并被排除在PH-ILD的许多研究和讨论之外。

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PH Roundtable: Conundrums and Controversies in PH-ILD: To Treat or Not to Treat—Identifying Optimal Treatment Candidates PH圆桌会议:PH- ild的难题和争议:治疗或不治疗-确定最佳治疗候选者

Advances in pulmonary hypertension

Pub Date : 2021-10-01 DOI: 10.21693/1933-088x-20.4.132

J. Edelman, J. Elwing, A. Vaidya, S. Mathai

This fall, Jeffrey Edelman, MD, Associate Professor of Medicine at the University of Washington and at the VA Medical Center in Seattle, Washington, gathered with Jean Elwing, MD, Professor of Medicine and the Director of the Pulmonary Hypertension Program at the University of Cincinnati; Anjali Vaidya, MD, Associate Professor of Medicine and Co-Director of the Pulmonary Hypertension, Right Heart Failure & CTEPH Program at Temple University in Philadelphia; and Steve Mathai, MD, Associate Professor of Medicine at Johns Hopkins School of Medicine and Co-Director of the Ann Dana Kusch Multidisciplinary Research Program for Pulmonary Hypertension and Interstitial Lung Disease, to discuss pulmonary hypertension (PH) in World Health Organization (WHO) Group 3 patients with interstitial lung disease (ILD).

今年秋天，华盛顿大学和华盛顿西雅图退伍军人医疗中心的医学副教授Jeffrey Edelman医学博士与辛辛那提大学医学教授兼肺动脉高压项目主任Jean Elwing医学博士齐聚一堂;Anjali Vaidya医学博士，费城坦普尔大学肺动脉高压、右心衰和CTEPH项目的医学副教授和联合主任;约翰霍普金斯医学院医学副教授、Ann Dana Kusch肺高压和间质性肺病多学科研究项目联合主任Steve Mathai医学博士，讨论世界卫生组织(WHO)第3组间质性肺病(ILD)患者的肺动脉高压(PH)。

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Pulmonary Hypertension Clinical Trials and COVID-19: A Discussion With John Ryan and Roham Zamanian 肺动脉高压临床试验与新冠肺炎：与John Ryan和Roham Zamanian讨论

Advances in pulmonary hypertension

Pub Date : 2021-01-01 DOI: 10.21693/1933-088x-20.1.26

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PH Roundtable: Pulmonary Hypertension and Telehealth in the Time of Coronavirus Disease 2019 PH圆桌会议:2019冠状病毒疾病时期的肺动脉高压和远程医疗

Advances in pulmonary hypertension

Pub Date : 2021-01-01 DOI: 10.21693/1933-088x-20.1.16

Anonymous

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