Pulmonary Hypertension, Cor Pulmonale, and other Pulmonary Vascular Conditions

Arterial Hypertension ◽

Cor Pulmonale ◽

World Health ◽

Pulmonary Vasculature ◽

This review covers the diseases that affect the pulmonary vasculature directly. These conditions include pulmonary hypertension; pulmonary arterial hypertension; chronic thromboembolic pulmonary hypertension; pulmonary hypertension attributed to left heart disease, lung disease and/or hypoxemia, and other disorders; cor pulmonale; pulmonary atriovenous malformations; and pulmonary aneurysms. Figures show changes in the pulmonary vasculature in pulmonary hypertension, pathways involved in the development of pulmonary hypertension, general guidelines for the evaluation of suspected pulmonary hypertension, enlarged proximal pulmonary arteries with pruning of distal pulmonary vasculature (typical of advanced pulmonary arterial hypertension), the remodeling of the heart and continuous-wave Doppler study results observed with chronic pulmonary hypertension, ventilation and perfusion scans of the lungs with results typical of chronic thromboembolic pulmonary hypertension, and a general approach to the treatment of patients with pulmonary arterial hypertension. Tables list the revised nomenclature and classification of pulmonary hypertension, the World Health Organization classification of functional capacity in patients with pulmonary hypertension, advanced vascular medications for pulmonary artery hypertension, and perioperative management of pulmonary arterial hypertension. This review contains 8 highly rendered figures, 4 tables, and 118 references.

Respiratory rate modulation improves symptoms in patients with pulmonary hypertension

SAGE Open Medicine ◽

10.1177/20503121211053930 ◽

2021 ◽

Vol 9 ◽

pp. 205031212110539

Author(s):

Barbro Kjellström ◽

Bodil Ivarsson ◽

Lise-Lotte Landenfelt Gestré ◽

Henrik Ryftenius ◽

Magnus Nisell

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Respiratory Rate ◽

World Health ◽

Pulmonary Arterial ◽

Respiratory Modulation ◽

Health Organization

Background: Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are chronic diseases with a severe symptom burden. Common symptoms are dyspnoea at light activity and general fatigue that limits daily activities. Respiratory modulation by device-guided breathing decreased symptoms in patients with heart failure. The aim of this pilot study was to investigate if respiratory modulation could improve symptoms of dyspnoea in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Method: Adult patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension with symptoms of dyspnoea at rest or light activity performed home-based respiratory modulation by device-guided breathing 20 min a day for 3 months. Patients were on stable disease-specific treatment ⩾3 months and willing to undergo all study procedures. Dyspnoea score, World Health Organization class, physical status, N-terminal pro b-type natriuretic peptide, quality of life, respiratory rate and 6-min walk distance were assessed before and after 3 months with respiratory modulation. Results: Nine patients with pulmonary arterial hypertension and five with chronic thromboembolic pulmonary hypertension completed the study protocol. Mean age was 71 ± 14 years, and 11 were women. After 3 months of respiratory modulation, dyspnoea score (−0.6, p = 0.014), respiratory rate at rest (−3 breaths/min, p = 0.013), World Health Organization class (−0.3, p = 0.040), quality of life (EuroQol Visual Analogue Scale +5 points, not significant) and decreased N-terminal pro b-type natriuretic peptide (−163 ng/L, p = 0.043) had improved. The fatigue and respiratory rate after the 6-min walk decreased while the 6-min walk distance remained unchanged. Conclusion: Patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension that used device-guided breathing for 3 months improved symptoms of dyspnoea and lowered the respiratory rate at rest and after exercise.

Chronic thromboembolic pulmonary hypertension: a distinct disease entity

European Respiratory Review ◽

10.1183/16000617.00001115 ◽

2015 ◽

Vol 24 (136) ◽

pp. 246-252 ◽

Cited By ~ 53

Author(s):

Irene Lang

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Right Heart Failure ◽

Pulmonary Vasculature ◽

Distinct Disease ◽

Perfusion Scan ◽

Chronic thromboembolic pulmonary hypertension (CTEPH) is a distinct subtype of pulmonary hypertension (PH). One disease hypothesis is that CTEPH results from the non-resolution of venous thromboembolism. CTEPH is characterised by the presence of obstructive fibrotic thromboembolic material in the major pulmonary vessels, with concomitant microvascular arteriopathy, resulting in progressive PH. The clinical presentation of CTEPH is similar to pulmonary arterial hypertension with nonspecific symptoms, but it is distinguished from pulmonary arterial hypertension by the presence of mismatched segmental defects on the ventilation/perfusion scan. The exact prevalence and incidence of CTEPH are unknown, but are thought to have been underestimated in the past. CTEPH is unique among the subgroups of PH in that it is potentially curable with pulmonary endarterectomy, a surgical intervention intended to remove the occlusive material from the pulmonary vasculature. However, in some patients the obstructions are technically inaccessible or the risk/benefit ratios are unfavourable, making the condition inoperable. It is thought that the involvement of the smaller, more distal vessels is a target for medical treatment. Untreated, CTEPH may result in right heart failure and death. The pathophysiological mechanisms which cause CTEPH are complex and have not yet been fully elucidated.

Identification of the Novel Variants in Patients With Chronic Thromboembolic Pulmonary Hypertension

Journal of the American Heart Association ◽

10.1161/jaha.120.015902 ◽

2020 ◽

Vol 9 (21) ◽

Author(s):

Nobuhiro Yaoita ◽

Kimio Satoh ◽

Taijyu Satoh ◽

Toru Shimizu ◽

Sakae Saito ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Pulmonary Vasculature ◽

Nonsynonymous Variant ◽

Whole Exome ◽

History Of ◽

Background Although chronic thromboembolic pulmonary hypertension (CTEPH) and acute pulmonary embolism (APE) share some clinical manifestations, a limited proportion of patients with CTEPH have a history of APE. Moreover, in histopathologic studies, it has been revealed that pulmonary vasculature lesions similar to pulmonary arterial hypertension existed in patients with CTEPH. Thus, it remains unknown whether these 3 disorders also share genetic backgrounds. Methods and Results Whole exome screening was performed with DNA isolated from 51 unrelated patients with CTEPH of Japanese ancestry. The frequency of genetic variants associated with pulmonary arterial hypertension or APE in patients with CTEPH was compared with those in the integrative Japanese Genome Variation Database 3.5KJPN. Whole exome screening analysis showed 17 049 nonsynonymous variants in patients with CTEPH. Although we found 6 nonsynonymous variants that are associated with APE in patients with CTEPH, there was no nonsynonymous variant associated with pulmonary arterial hypertension. Patients with CTEPH with a history of APE had nonsynonymous variants of F5 , which encodes factor V. In contrast, patients with CTEPH without a history of APE had a nonsynonymous variant of THBD , which encodes thrombomodulin. Moreover, thrombin‐activatable fibrinolysis inhibitor, which is one of the pathogenic proteins in CTEPH, was significantly more activated in those who had the variants of THBD compared with those without it. Conclusions These results provide the first evidence that patients with CTEPH have some variants associated with APE, regardless of the presence or absence of a history of APE. Furthermore, the variants might be different between patients with CTEPH with and without a history of APE.

Orphan designation: Treprostinil diethanolamine (oral use), Treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Case Medical Research ◽

10.31525/cmr-d973c6 ◽

2019 ◽

Author(s):

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Orphan Designation ◽

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

Journal of Patient-Reported Outcomes ◽

10.1186/s41687-021-00327-9 ◽

2021 ◽

Vol 5 (1) ◽

Author(s):

Brooke Currie ◽

Evan Davies ◽

Amélie Beaudet ◽

Larissa Stassek ◽

Leah Kleinman

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Shortness Of Breath ◽

Response Options ◽

Qualitative Interview Study ◽

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.

Support Experienced by Patients Living with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension

Heart Lung and Circulation ◽

10.1016/j.hlc.2015.03.026 ◽

2016 ◽

Vol 25 (1) ◽

pp. 35-40 ◽

Cited By ~ 9

Author(s):

Bodil Ivarsson ◽

Björn Ekmehag ◽

Trygve Sjöberg

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Riociguat is a novel medication for the treatment of pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Medical Council ◽

10.21518/2079-701x-2016-4-20-27 ◽

2016 ◽

pp. 20-27

Author(s):

N. A. Tsareva ◽

S. N. Avdeev

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Bosentan therapy for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: A systemic review and meta-analysis

The Clinical Respiratory Journal ◽

10.1111/crj.12774 ◽

2018 ◽

Vol 12 (6) ◽

pp. 2065-2074 ◽

Cited By ~ 4

Author(s):

Xinwang Chen ◽

Zhenguo Zhai ◽

Ke Huang ◽

Wanmu Xie ◽

Jun Wan ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Meta Analysis ◽

Systemic Review ◽

Pathophysiology, incidence, management, and consequences of cardiac arrhythmia in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Pulmonary Circulation ◽

10.1177/2045894019834890 ◽

2019 ◽

Vol 9 (1) ◽

pp. 204589401983489 ◽

Cited By ~ 3

Author(s):

Meghan M. Cirulis ◽

John J. Ryan ◽

Stephen L. Archer

Keyword(s):

Pulmonary Hypertension ◽

Arterial Hypertension ◽

Molecular Mechanisms ◽

Outcome Data ◽

Current Evidence ◽

Clinical Aspects ◽

Arrhythmias are increasingly recognized as serious, end-stage complications of pre-capillary pulmonary hypertension, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Although arrhythmias contribute to symptoms, morbidity, in-hospital mortality, and possibly sudden death in PAH/CTEPH, there remains a paucity of epidemiologic, pathophysiologic, and outcome data to guide management of these patients. This review summarizes the most current evidence on the topic: from the molecular mechanisms driving arrhythmia in the hypertrophied or failing right heart, to the clinical aspects of epidemiology, diagnosis, and management.