scholarly journals Peripheral microangiopathy in precapillary pulmonary hypertension: a nailfold video capillaroscopy prospective study

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Alexandra Arvanitaki ◽  
George Giannakoulas ◽  
Eva Triantafyllidou ◽  
Christos Feloukidis ◽  
Afroditi K. Boutou ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Structural Changes ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Multiple Comparisons ◽  
Capillary Density ◽  
Pulmonary Vascular Disease ◽  
Healthy Controls ◽  
Continuous Variables ◽  
Thromboembolic Pulmonary Hypertension ◽  
Bonferroni Test

Abstract Background Although pulmonary vascular bed has been the main subject of research for many years in pulmonary hypertension (PH), interest has recently started to divert towards the possibility of a co-existing peripheral microangiopathy. The aim of the current study was to investigate the presence of nailfold video-capillaroscopic (NVC) structural changes in patients with precapillary PH and to identify possible associations of NVC measurements with markers of disease severity. Methods Α prospective case–control study was performed in 28 consecutive patients with precapillary PH [14 with idiopathic pulmonary arterial hypertension (IPAH) and 14 with chronic thromboembolic pulmonary hypertension (CTEPH)] and 30 healthy controls. NVC quantitative and qualitative parameters were evaluated using Optilia Digital Capillaroscope. To ensure inter-observer repeatability capillaroscopic images were reviewed by two independent investigators. For multiple comparisons among continuous variables, one-way ANOVA or the Kruskal–Wallis test were used. Differences between the groups were tested with post-hoc analysis with adjustment for multiple comparisons (Bonferroni test). Results Both IPAH (71.4% were women, mean age 53.1 ± 13.4 years) and CTEPH (64.3% women, mean age 60.9 ± 14.4 years) groups presented reduced capillary density compared to healthy controls (8.4 ± 1.2 loops/mm and 8.0 ± 1.2 loops/mm vs. 9.7 ± 0.81 loops/mm, p < 0.001) and increased loop width (15.7 ± 3.9 μm and 15.8 ± 1.9 μm vs. 11.5 ± 2.3 μm, p < 0.001). More than half of patients with IPAH presented microhaemorrhages on capillary nailfold, while increased shape abnormalities in capillary morphology and more capillary thrombi per linear mm were detected in patients with CTEPH compared to patients with IPAH and healthy controls. All PH patients presented a non-specific NVC pattern compared to controls (p < 0.001). Conclusion The findings of the study reveal a degree of significant peripheral microvascular alterations in patients with IPAH and CTEPH, suggesting a generalized impairment of peripheral microvasculature in pulmonary vascular disease.

scholarly journals Peripheral Microangiopathy in Pre-capillary Pulmonary Hypertension: A Nailfold Video Capillaroscopy Prospective Study

2020 ◽  
Author(s):  
Alexandra Arvanitaki ◽  
George Giannakoulas ◽  
Eva Triantafyllidou ◽  
Christos Feloukidis ◽  
Alexandros Garyfallos ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Structural Changes ◽  
Ventricular Remodeling ◽  
Connective Tissue Diseases ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Capillary Density ◽  
Cross Sectional Study ◽  
Healthy Controls ◽  
Cross Sectional ◽  
Microvascular Changes

Abstract Background: Although pulmonary vascular bed has been the main subject of research for many years in pulmonary hypertension (PH), interest has recently started to divert towards the possibility of a co-existing peripheral microangiopathy. The aim of this study was to investigate the presence of peripheral microangiopathy in patients with precapillary pulmonary hypertension by exploring nailfold video-capillaroscopic (NVC) structural changes among various subgroups with precapillary PH and to identify possible associations of NVC characteristics with markers of disease severity. Methods: Α cross-sectional study was performed in 46 consecutive patients with precapillary PH [14 with idiopathic pulmonary arterial hypertension (IPAH), 18 with PAH associated with connective tissue diseases (CTD-PAH) and 14 with chronic thromboembolic pulmonary hypertension (CTEPH)] and 30 healthy controls. NVC quantitative and qualitative parameters were evaluated. Results: Patients with precapillary PH (71.7% were women, mean age 60.8 ± 13.4 years) presented reduced capillary density compared to healthy controls (7.5 ± 1.6 loops/mm vs. 9.7 ± 0.81 loops/mm, p<0.001) and increased capillary loop width (18.8 ± 6.7 μm vs. 11.5 ± 2.3 μm, p<0.001) detected mainly in PAH-CTD. Half of patients presented microhaemorrhages on capillary nailfold, while morphological capillary abnormalities where also detected compared to healthy controls. Log10 (NT-proBNP) was independently associated with capillary density in patients with precapillary PH [r= -0.68, B= -1.9, 95% CI (-3,3, -0.4) p=0.014].Conclusion: Significant NVC microvascular changes were detected in patients with various types of precapillary PH, suggesting an impaired peripheral microcirculation associated with right ventricular remodeling parallel to pulmonary vasculopathy.

P4670Long-term changes of exercise haemodynamics and physical capacity in chronic thromboembolic pulmonary hypertension after pulmonary thromboendarterectomy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
F Waziri ◽  
S Mellemkjaer ◽  
T S Clemmensen ◽  
V E Hjortdal ◽  
L B Ilkjaer ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Cardiac Output ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Peak Oxygen Uptake ◽  
Physical Capacity ◽  
Peak Exercise ◽  
Healthy Controls ◽  
Pulmonary Thromboendarterectomy ◽  
Right Heart Catheterisation ◽  
Thromboembolic Pulmonary Hypertension

Abstract Background A substantial number of chronic thromboembolic pulmonary hypertension (CTEPH) patients experience dyspnoea on exertion and limited exercise capacity despite surgically successful pulmonary endarterectomy (PEA). Purpose We sought to prospectively evaluate resting and peak exercise haemodynamics before, 3 and 12 months after PEA in consecutive CTEPH patients and correlate it to physical functional capacity. Methods Twenty CTEPH patients who underwent PEA treatment were examined between December 2014 and January 2017. The patients were examined pre-operatively and 3 and 12 months post-operatively with right heart catheterisation at rest and during a maximal symptom-limited, semi-supine cardiopulmonary exercise test. Finally, the data were compared with 15 healthy controls who had previously undergone the same protocol. Results Twelve months after PEA, 75% of patients with severely increased pre-PEA mean pulmonary arterial pressure (mPAP) at rest had normal or mildly increased mPAP. However, mPAP reduction was less pronounced during exercise where only 45% had normal or mildly increased mPAP at 12 months. Cardiac output (CO) reserve (CO increase from rest to peak exercise) was increased (5.7±2.9 L/min) 12 months after PEA compared with pre-PEA (2.5±1.8 L/min), p<0.0001. However, 12 months after PEA, the CO reserve was only 49% of that of healthy controls, p<0.0001. Changes in cardiac output (ΔCO), calculated as the difference between CO before PEA and 12 months later, were significantly correlated with six-minute-walk-test and peak oxygen uptake (VO2), both at rest and peak exercise. Figure 1 Conclusion Invasive exercise haemodynamic examination in CTEPH patients demonstrates that after otherwise successful PEA surgery, more than 50% of patients have a significant increase in exercise mPAP, and the CO reserve remains compromised 12 months after PEA. Improvement in physical capacity is correlated with ΔCO. Acknowledgement/Funding Danish Heart Foundation, Aarhus University, Arvid Nilssons Fond, Eva & Henry Frænkels Mindefond, and Snedkermester Sophus Hustru Astrid Jacobsens Fond

scholarly journals Comprehensive analysis of inflammatory markers in chronic thromboembolic pulmonary hypertension patients

2014 ◽  
Vol 44 (4) ◽  
pp. 951-962 ◽  
Author(s):  
Diana Zabini ◽  
Akos Heinemann ◽  
Vasile Foris ◽  
Chandran Nagaraj ◽  
Patrick Nierlich ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Serum Levels ◽  
Interferon Γ ◽  
Walking Distance ◽  
Healthy Controls ◽  
Monocyte Chemoattractant Protein 1 ◽  
Inflammatory Protein ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is associated with chronic inflammation but the pathological mechanisms are largely unknown. Our study aimed to simultaneously profile a broad range of cytokines in the supernatant of pulmonary endarterectomy (PEA) surgical material, as well as prospectively in patients with CTEPH to investigate whether circulating cytokines are associated with haemodynamic and physical characteristics of CTEPH patients.Herein, we show that PEA specimens revealed a significant upregulation of interleukin (IL)-6, monocyte chemoattractant protein-1, interferon-γ-induced protein-10 (IP)-10, macrophage inflammatory protein (MIP)1α and RANTES compared to lung tissue from healthy controls.In prospectively collected serum, levels of IL-6, IL-8, IP-10, monokine induced by interferon-γ (MIG) and MIP1α were significantly elevated in CTEPH patients compared to age- and sex-matched healthy controls. In serum of idiopathic pulmonary arterial hypertension (IPAH) patients, only IP-10 and MIG were significantly increased. In CTEPH but not in IPAH, IP-10 was negatively correlated with cardiac index, 6-min walking distance and carbon monoxide diffusion capacity. In vitro, IP-10 significantly increased migration of freshly isolated adventitial fibroblasts.Our study is the first to show that IP-10 secretion is associated with poor pulmonary haemodynamics and physical capacity in CTEPH and might be involved in the pathological mechanism of PEA tissue formation.

scholarly journals The pathophysiology of chronic thromboembolic pulmonary hypertension

2017 ◽  
Vol 26 (143) ◽  
pp. 160112 ◽  
Author(s):  
Gérald Simonneau ◽  
Adam Torbicki ◽  
Peter Dorfmüller ◽  
Nick Kim
Keyword(s):  
Pulmonary Hypertension ◽  
Small Vessel Disease ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Small Vessel ◽  
Treatment Modalities ◽  
Pulmonary Vasculature ◽  
Pulmonary Vascular Disease ◽  
Substantial Impact ◽  
Vessel Disease ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive pulmonary vascular disease that is usually a consequence of prior acute pulmonary embolism. CTEPH usually begins with persistent obstruction of large and/or middle-sized pulmonary arteries by organised thrombi. Failure of thrombi to resolve may be related to abnormal fibrinolysis or underlying haematological or autoimmune disorders. It is now known that small-vessel abnormalities also contribute to haemodynamic compromise, functional impairment and disease progression in CTEPH. Small-vessel disease can occur in obstructed areas, possibly triggered by unresolved thrombotic material, and downstream from occlusions, possibly because of excessive collateral blood supply from high-pressure bronchial and systemic arteries. The molecular processes underlying small-vessel disease are not completely understood and further research is needed in this area. The degree of small-vessel disease has a substantial impact on the severity of CTEPH and postsurgical outcomes. Interventional and medical treatment of CTEPH should aim to restore normal flow distribution within the pulmonary vasculature, unload the right ventricle and prevent or treat small-vessel disease. It requires early, reliable identification of patients with CTEPH and use of optimal treatment modalities in expert centres.

Chronic thromboembolic pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2550-2554
Author(s):  
Yu Taniguchi ◽  
Xavier Jaïs ◽  
Gérald Simonneau ◽  
Marc Humbert
Keyword(s):  
Pulmonary Hypertension ◽  
Small Vessel Disease ◽  
Pulmonary Arteries ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Flow Distribution ◽  
Small Vessel ◽  
Pulmonary Vasculature ◽  
Pulmonary Vascular Disease ◽  
Vessel Disease ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and progressive pulmonary vascular disease that is usually the consequence of prior acute pulmonary embolism. CTEPH usually begins with persistent obstruction of large and/or middle-sized pulmonary arteries by organized thrombi. Failure of thrombi to resolve may be related to abnormal fibrinolysis, and underlying haematological or autoimmune disorders, including splenectomy and antiphospholipid antibodies. Small pulmonary vessel remodelling also contributes to haemodynamic compromise, functional impairment, and disease progression in CTEPH. Small vessel disease can occur in non-obstructed areas as well as downstream from occlusions (possibly because of excessive collateral blood supply from high-pressure bronchial and systemic arteries). The degree of small vessel disease has a substantial impact on the severity of CTEPH and post-surgical outcomes. Surgical, interventional, and medical treatment of CTEPH aim to restore normal flow distribution within the pulmonary vasculature, unload the right ventricle, and prevent or treat small vessel disease.

scholarly journals The ADAMTS13–VWF axis is dysregulated in chronic thromboembolic pulmonary hypertension

2019 ◽  
Vol 53 (3) ◽  
pp. 1801805 ◽  
Author(s):  
Michael Newnham ◽  
Kieron South ◽  
Marta Bleda ◽  
William R. Auger ◽  
Joan A. Barberà ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Hypertension ◽  
Disease Severity ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Healthy Controls ◽  
Thromboembolic Pulmonary Hypertension ◽  
Healthy Control ◽  
Pulmonary Arterial ◽  
Chronic Thromboembolic Disease ◽  
Von Willebrand

Chronic thromboembolic pulmonary hypertension (CTEPH) is an important consequence of pulmonary embolism that is associated with abnormalities in haemostasis. We investigated the ADAMTS13–von Willebrand factor (VWF) axis in CTEPH, including its relationship with disease severity, inflammation,ABOgroups andADAMTS13genetic variants.ADAMTS13 and VWF plasma antigen levels were measured in patients with CTEPH (n=208), chronic thromboembolic disease without pulmonary hypertension (CTED) (n=35), resolved pulmonary embolism (n=28), idiopathic pulmonary arterial hypertension (n=30) and healthy controls (n=68). CTEPH geneticABOassociations and protein quantitative trait loci were investigated. ADAMTS13–VWF axis abnormalities were assessed in CTEPH and healthy control subsets by measuring ADAMTS13 activity, D-dimers and VWF multimeric size.Patients with CTEPH had decreased ADAMTS13 (adjusted β −23.4%, 95% CI −30.9– −15.1%, p<0.001) and increased VWF levels (β +75.5%, 95% CI 44.8–113%, p<0.001) compared to healthy controls. ADAMTS13 levels remained low after reversal of pulmonary hypertension by pulmonary endarterectomy surgery and were equally reduced in CTED. We identified a genetic variant near theADAMTS13gene associated with ADAMTS13 protein that accounted for ∼8% of the variation in levels.The ADAMTS13–VWF axis is dysregulated in CTEPH. This is unrelated to pulmonary hypertension, disease severity or markers of systemic inflammation and implicates the ADAMTS13–VWF axis in CTEPH pathobiology.

Pulmonary hypertension: clinical classification

ESC CardioMed ◽  
2018 ◽  
pp. 2500-2502
Author(s):  
Francisca A. Gavilanes Oleas ◽  
Caio J. C. Fernandes ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Management Strategies ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Vascular Disease ◽  
Left Heart ◽  
Clinical Classification ◽  
Current Classification ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Clinical Conditions

Pulmonary hypertension is a clinical and haemodynamic condition associated with several baseline clinical conditions or existing as a pure pulmonary vascular disease. The current classification groups aetiologies of pulmonary hypertension that share similarities in terms of clinical presentation, pathological findings, and haemodynamic profiles, in an attempt to guide management strategies in these distinct conditions. As a consequence, pulmonary hypertension is classified into five different groups: pulmonary arterial hypertension, pulmonary hypertension due to left heart disease, pulmonary hypertension due to lung disease and/or hypoxia, chronic thromboembolic pulmonary hypertension, and pulmonary hypertension with unclear multifactorial mechanisms. This chapter reviews the rational supporting the current classification system alluding also to the existing evidence for specific therapies in each one of the groups.

scholarly journals Plasma adrenomedullin peptides and precursor levels in pulmonary arterial hypertension disease severity and risk stratification

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093131
Author(s):  
Habib Bouzina ◽  
Göran Rådegran
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Early Treatment ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Risk Scores ◽  
Healthy Controls ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Adrenomedullin is a potent vasodilatory peptide, linked to pulmonary arterial hypertension pathology. Proximity extension assays were utilized to study plasma biomarkers related to vasoregulation, with focus on adrenomedullin peptides and precursor levels, collectively referred to as ADM. ADM was measured in 48 treatment-naïve pulmonary arterial hypertension patients at diagnosis, and in 31 of them at an early treatment follow-up. Plasma ADM was additionally assessed in patients with chronic thromboembolic pulmonary hypertension ( n = 20) and pulmonary hypertension due to heart failure with preserved (HFpEF(PH)) ( n = 33) or reduced (HFrEF(PH)) ( n = 36) ejection fraction, as well as healthy controls ( n = 16). ADM was studied in relation to pulmonary arterial hypertension hemodynamics, risk assessment, prognosis, treatment response, and differentiation. Plasma ADM levels in pulmonary arterial hypertension patients at diagnosis were higher than in healthy controls ( p < 0.001), similar as in chronic thromboembolic pulmonary hypertension patients ( p = ns), but lower compared to HFpEF(PH) ( p < 0.03) and HFrEF(PH) ( p < 0.001). In pulmonary arterial hypertension, specifically, plasma ADM at diagnosis correlated mainly to mean right atrial pressure ( r = 0.73, p < 0.001), N-terminal prohormone of brain natriuretic peptide ( r = 0.75, p < 0.001), six-minute walking distance ( r = –0.57, p < 0.001), and venous oxygen saturation ( r = –0.57, p < 0.001). ADM also correlated to the ECS/ERS- ( r = 0.74, p < 0.001) and REVEAL risk scores ( r = 0.54, p < 0.001) at pulmonary arterial hypertension diagnosis. Plasma ADM in pulmonary arterial hypertension patients was unaltered at early treatment follow-up compared to baseline ( p = ns). Pulmonary arterial hypertension patients with supra-median ADM at diagnosis showed worse overall survival than those with infra-median levels (median survival 34 versus 66 months, p = 0.0077). In conclusion, the present results suggest that baseline plasma ADM levels mirror disease severity, correlating to both ECS/ERS- and the REVEAL risk scores.

scholarly journals Revisiting a Distinct Entity in Pulmonary Vascular Disease: Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

Medicina ◽  
2021 ◽  
Vol 57 (4) ◽  
pp. 355
Author(s):  
Munish Sharma ◽  
Deborah Jo Levine
Keyword(s):  
Pulmonary Hypertension ◽  
Medical Therapy ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Progressive Increase ◽  
Pulmonary Vasculature ◽  
Pulmonary Vascular Disease ◽  
Pulmonary Thromboendarterectomy ◽  
Percutaneous Balloon ◽  
Definitive Therapy ◽  
Thromboembolic Pulmonary Hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a specific type of pulmonary hypertension (PH) and the major component of Group 4 pulmonary hypertension (PH). It is caused by pulmonary vasculature obstruction that leads to a progressive increase in pulmonary vascular resistance and, ultimately, to failure of the right ventricle. Pulmonary thromboendarterectomy (PEA) is the only definitive therapy, so a timely diagnosis and early referral to a specialized PEA center to determine candidacy is prudent for a favorable outcome. Percutaneous balloon pulmonary angioplasty (BPA) has a potential role in patients unsuitable for PEA. Medical therapy with riociguat is the only PH-specific medical therapy currently approved for the treatment of inoperable or persistent CTEPH. This review article aims to revisit CTEPH succinctly with a review of prevailing literature.

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