scholarly journals EXPRESS: What’s New in Pulmonary Hypertension Clinical Research: Lessons from the Best Abstracts at the 2020 American Thoracic Society International Conference

2021 ◽  
pp. 204589402110407
Author(s):  
Andrew J Sweatt ◽  
Raju Reddy ◽  
Farbod Rahaghi ◽  
Nadine Al-Naamani
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Research ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
American Thoracic Society ◽  
Future Directions ◽  
International Conference ◽  
Metabolic Dysregulation ◽  
Current State ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

In this conference paper, we review the 2020 American Thoracic Society (ATS) International Conference session titled, “What’s New in Pulmonary Hypertension Clinical Research: Lessons from the Best Abstracts”. This virtual mini-symposium took place on October 21, 2020, in lieu of the annual in-person ATS International Conference which was cancelled due to the COVID-19 pandemic. Seven clinical research abstracts were selected for presentation in the session, which encompassed five major themes: (1) standardizing diagnosis and management of pulmonary hypertension, (2) improving risk assessment in pulmonary arterial hypertension, (3) evaluating biomarkers of disease activity, (4) understanding metabolic dysregulation across the spectrum of pulmonary hypertension, and (5) advancing knowledge in chronic thromboembolic pulmonary hypertension. Focusing on these five thematic contexts we review the current state of knowledge, summarize presented research abstracts, appraise their significance and limitations, and then discuss relevant future directions in pulmonary hypertension clinical research.

scholarly journals Decreased time constant of the pulmonary circulation in chronic thromboembolic pulmonary hypertension

2013 ◽  
Vol 305 (2) ◽  
pp. H259-H264 ◽  
Author(s):  
Robert V. MacKenzie Ross ◽  
Mark R. Toshner ◽  
Elaine Soon ◽  
Robert Naeije ◽  
Joanna Pepke-Zaba
Keyword(s):  
Pulmonary Hypertension ◽  
Time Constant ◽  
Pulmonary Circulation ◽  
Wave Reflection ◽  
Arterial Compliance ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Left Heart Failure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Rc Time Constant

This study analyzed the relationship between pulmonary vascular resistance (PVR) and pulmonary arterial compliance ( Ca) in patients with idiopathic pulmonary arterial hypertension (IPAH) and proximal chronic thromboembolic pulmonary hypertension (CTEPH). It has recently been shown that the time constant of the pulmonary circulation (RC time constant), or PVR × Ca, remains unaltered in various forms and severities of pulmonary hypertension, with the exception of left heart failure. We reasoned that increased wave reflection in proximal CTEPH would be another cause of the decreased RC time constant. We conducted a retrospective analysis of invasive pulmonary hemodynamic measurements in IPAH ( n = 78), proximal CTEPH ( n = 91) before (pre) and after (post) pulmonary endarterectomy (PEA), and distal CTEPH ( n = 53). Proximal CTEPH was defined by a postoperative mean pulmonary artery pressure (PAP) of ≤25 mmHg. Outcome measures were the RC time constant, PVR, Ca, and relationship between systolic and mean PAPs. The RC time constant for pre-PEA CTEPH was 0.49 ± 0.11 s compared with post-PEA-CTEPH (0.37 ± 0.11 s, P < 0.0001), IPAH (0.63 ± 0.14 s, P < 0.001), and distal CTEPH (0.55 ± 0.12 s, P < 0.05). A shorter RC time constant was associated with a disproportionate decrease in systolic PAP with respect to mean PAP. We concluded that the pulmonary RC time constant is decreased in proximal CTEPH compared with IPAH, pre- and post-PEA, which may be explained by increased wave reflection but also, importantly, by persistent structural changes after the removal of proximal obstructions. A reduced RC time constant in CTEPH is in accord with a wider pulse pressure and hence greater right ventricular work for a given mean PAP.

scholarly journals Symptoms, impacts, and suitability of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire in patients with chronic thromboembolic pulmonary hypertension (CTEPH): a qualitative interview study

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Brooke Currie ◽  
Evan Davies ◽  
Amélie Beaudet ◽  
Larissa Stassek ◽  
Leah Kleinman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Shortness Of Breath ◽  
Response Options ◽  
Qualitative Interview Study ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
The Impact

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension caused by blood clots and scar tissue in the blood vessels of the lungs. Health-related quality of life is often significantly impaired in patients with CTEPH. However, a better understanding of how CTEPH symptoms affect patients’ lives is needed to optimally assess the impact of the disease and treatment. Objectives This qualitative study aimed to better understand the symptoms of CTEPH and how they affect patients’ lives, as well as to determine the appropriateness of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire for use in this patient population. Methods Adults diagnosed with CTEPH, recruited from two clinical sites in the US, participated in one-to-one qualitative telephone interviews. They described their experience of CTEPH symptoms and the impact these symptoms have on their lives. They also provided feedback on the comprehensibility and relevance of the PAH-SYMPACT™‘s instructions, items, and response options. Results Participants (N = 12) had a mean age of 62.5 years. Two thirds were female and most (83%) had undergone pulmonary endarterectomy and/or balloon pulmonary angioplasty. The most frequently endorsed symptoms were shortness of breath (endorsed by all 12 participants), fatigue (11 participants), and lightheadedness (10 participants). All participants identified shortness of breath as an “extremely important” symptom, and seven participants rated fatigue as “extremely important.” The most frequent impacts of CTEPH were on ability to walk quickly (endorsed by all 12 participants), ability to walk up inclines or stairs (11 participants), and ability to carry things (11 participants). The PAH-SYMPACT™ items were relevant to most participants and reflected their experience of CTEPH. All participants indicated that no important CTEPH symptoms were missing from the PAH-SYMPACT™. Overall, the instructions, items, and response options of the PAH-SYMPACT™ were clear and easy to understand. Conclusions The symptoms and impacts experienced by patients with CTEPH align with items included in the PAH-SYMPACT™. The PAH-SYMPACT™ appears to be fit for purpose for assessing disease status in patients with CTEPH.

scholarly journals Effect of Coronavirus Disease 2019 in Pulmonary Circulation. The Particular Scenario of Precapillary Pulmonary Hypertension

Diagnostics ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. 548 ◽  
Author(s):  
Jorge Nuche ◽  
Teresa Segura de la Cal ◽  
Carmen Jiménez López Guarch ◽  
Francisco López-Medrano ◽  
Carmen Pérez-Olivares Delgado ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Respiratory Infections ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
The Elderly ◽  
Angiotensin Converting Enzyme 2 ◽  
Common Features ◽  
Pulmonary Vasodilators ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Low Incidence

The Coronavirus Disease of 2019 (COVID-19) has supposed a global health emergency affecting millions of people, with particular severity in the elderly and patients with previous comorbidities, especially those with cardiovascular disease. Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) could represent an especially vulnerable population because of the high mortality rates reported for respiratory infections. However, the number of COVID-19 cases reported among PAH and CTEPH patients is surprisingly low. Furthermore, the clinical picture that has been described in these patients is far from the severity that experts would expect. Endothelial dysfunction is a common feature between patients with PAH/CTEPH and COVID-19, leading to ventilation/perfusion mismatch, vasoconstriction, thrombosis and inflammation. In this picture, the angiotensin-converting enzyme 2 plays an essential role, being directly involved in the pathophysiology of both clinical entities. Some of these common characteristics could explain the good adaptation of PAH and CTEPH patients to COVID-19, who could also have obtained a benefit from the disease’s specific treatments (anticoagulant and pulmonary vasodilators), probably due to its protective effect on the endothelium. Additionally, these common features could also lead to PAH/CTEPH as a potential sequelae of COVID-19. Throughout this comprehensive review, we describe the similarities and differences between both conditions and the possible pathophysiological and therapeutic-based mechanisms leading to the low incidence and severity of COVID-19 reported in PAH/CTEPH patients to date. Nevertheless, international registries should look carefully into this population for better understanding and management.

scholarly journals Prognostic Relevance of Nonsustained Ventricular Tachycardia in Patients with Pulmonary Hypertension

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Dirk Bandorski ◽  
Harilaos Bogossian ◽  
Johanna Stempfl ◽  
Werner Seeger ◽  
Matthias Hecker ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Tachycardia ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Holter Ecg ◽  
Nonsustained Ventricular Tachycardia ◽  
Prognostic Relevance ◽  
Systolic Pulmonary Arterial Pressure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Background.Increased pulmonary vascular resistance in patients with pulmonary hypertension (PH) leads to an increased afterload of right heart and cardiac remodeling which could provide the substrate or trigger for arrhythmias. Supraventricular arrhythmias were associated with clinical deterioration but were not associated with sudden cardiac death (SCD). SCD has been reported to account for approximately 30% of deaths in patients with pulmonary arterial hypertension (PAH).Objective.The role of nonsustained ventricular tachycardia (nsVT) and its prognostic relevance in patients with PH remains unclear. This study evaluated the prognostic relevance of nsVT in patients with PAH and chronic thromboembolic pulmonary hypertension (CTEPH).Methods.Retrospectively, patients with PAH and CTEPH who underwent Holter ECG monitoring and available data of survival were investigated.Results.Seventy-eight (PAH: 55, CTEPH: 23) patients were evaluated. Holter ECG revealed nsVT in 12 patients. Twenty-one patients died during follow-up. In patients with nsVT, tricuspid annular plane systolic excursion was lower(p=0.001), and systolic pulmonary arterial pressure was higher(p=0.163). Mean survival of patients without/with nsVT was 155.2 ± 8.5/146.4 ± 21.4 months(p=0.690). The association between arrhythmias and survival was not confounded by age(p=0.681), gender(p=0.752), 6-MW distance(p=0.196), or arterial hypertension(p=0.238).Conclusions.In patients with PH, nsVT occurs more often than previously reported, and patients with PH group 1 seem to be more at risk.

Pulmonary hypertension: definition

ESC CardioMed ◽  
2018 ◽  
pp. 2482-2484
Author(s):  
Stephan Rosenkranz
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Left Heart ◽  
Chronic Lung Diseases ◽  
Common Causes ◽  
Thromboembolic Pulmonary Hypertension ◽  
Underlying Causes ◽  
Pulmonary Arterial ◽  
Clinical Phenotyping ◽  
Global Population

Pulmonary hypertension (PH) in general is characterized by an elevation of pulmonary artery pressure which may result from various underlying causes. PH is a common disorder affecting approximately 1% of the global population, and up to 10% of patients older than 65 years of age. The most common causes are diseases of the left heart and chronic lung diseases, whereas disorders primarily originating from the pulmonary vessels including pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension remain relatively rare conditions. PH is primarily defined by haemodynamic variables, and subdivided into pre- and post-capillary PH. Further information obtained from imaging, pathology, genetics, and clinical phenotyping may be required for accurate subclassification of PH.

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