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Article ; Conference proceedings: Eine seltene aber wichtige zystische Lungenerkrankung – Das Birt-Hogg-Dubé-Syndrom

Asbach, J / Hinterthaner, M / Dorfmüller, P / Hammerl, P / Andreas, S

2024 Volume 78, Issue S 01

Event/congress	64. Kongress der Deutschen Gesellschaft für Pneumologie und Beatmungsmedizin e. V., Mannheim, 2024-03-20
Language	German
Publishing date	2024-03-01
Publisher	Georg Thieme Verlag
Publishing place	Stuttgart ; New York
Document type	Article ; Conference proceedings
ZDB-ID	607630-0
ISSN	1438-8790 ; 0934-8387
ISSN (online)	1438-8790
ISSN	0934-8387
DOI	10.1055/s-0044-1778902
Database	Thieme publisher's database

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Uh II Zs.142: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Clinical-radiological-pathological correlation in pulmonary hypertension with unclear and/or multifactorial mechanisms.

Lichtblau, Mona / Mayer, Laura / Gopalan, Deepa / Dorfmüller, Peter / Ulrich, Silvia

European respiratory review : an official journal of the European Respiratory Society

2023 Volume 32, Issue 170

Abstract: Ever since the second world symposium on pulmonary hypertension (PH) held in Evian, France, in 1998, PH has been classified into five major clinical groups. Group 5 PH includes a variety of distinct conditions with unclear and/or multifactorial ... ...

Abstract	Ever since the second world symposium on pulmonary hypertension (PH) held in Evian, France, in 1998, PH has been classified into five major clinical groups. Group 5 PH includes a variety of distinct conditions with unclear and/or multifactorial underlying pathologies. Management of these patients is challenging as the number of patients within these groups is often small, not all individuals with certain underlying conditions are affected by PH and patients exhibit distinct symptoms due to different underlying diseases. Studies and clinical trials in these groups are largely lacking and mostly restricted to case series and registry reports. Nonetheless, the worldwide burden of group 5 PH is estimated to be significant in terms of the prevalence of some associated diseases. Group 5 PH encompasses six subgroups, including haematological disorders (inherited and acquired chronic haemolytic anaemia and chronic myeloproliferative disorders), systemic disorders (sarcoidosis, pulmonary Langerhans's cell histiocytosis and neurofibromatosis type 1), metabolic disorders (glycogen storage diseases and Gaucher disease), chronic renal failure with or without haemodialysis, pulmonary tumour thrombotic microangiopathy and fibrosing mediastinitis.
MeSH term(s)	Humans ; Hypertension, Pulmonary/diagnostic imaging ; Hypertension, Pulmonary/etiology ; Prevalence
Language	English
Publishing date	2023-12-20
Publishing country	England
Document type	Journal Article
ZDB-ID	1077620-5
ISSN	1600-0617 ; 0905-9180
ISSN (online)	1600-0617
ISSN	0905-9180
DOI	10.1183/16000617.0119-2023
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Zs.A 3265: Show issues

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Article ; Online: Clinical-imaging-pathological correlation in pulmonary hypertension associated with left heart disease.

van de Veerdonk, Marielle C / Roosma, Lize / Trip, Pia / Gopalan, Deepa / Vonk Noordegraaf, Anton / Dorfmüller, Peter / Nossent, Esther J

European respiratory review : an official journal of the European Respiratory Society

2024 Volume 33, Issue 171

Abstract: Pulmonary hypertension (PH) is highly prevalent in patients with left heart disease (LHD) and negatively impacts prognosis. The most common causes of PH associated with LHD (PH-LHD) are left heart failure and valvular heart disease. In LHD, passive ... ...

Abstract	Pulmonary hypertension (PH) is highly prevalent in patients with left heart disease (LHD) and negatively impacts prognosis. The most common causes of PH associated with LHD (PH-LHD) are left heart failure and valvular heart disease. In LHD, passive backward transmission of increased left-sided filling pressures leads to isolated post-capillary PH. Additional pulmonary vasoconstriction and remodelling lead to a higher vascular load and combined pre- and post-capillary PH. The increased afterload leads to right ventricular dysfunction and failure. Multimodality imaging of the heart plays a central role in the diagnostic work-up and follow-up of patients with PH-LHD. Echocardiography provides information about the estimated pulmonary artery pressure, morphology and function of the left and right side of the heart, and valvular abnormalities. Cardiac magnetic resonance imaging is the gold standard for volumetric measurements and provides myocardial tissue characterisation. Computed tomography of the thorax may show general features of PH and/or LHD and is helpful in excluding other PH causes. Histopathology reveals a spectrum of pre- and post-capillary vasculopathy, including intimal fibrosis, media smooth muscle cell hyperplasia, adventitial fibrosis and capillary congestion. In this paper, we provide an overview of clinical, imaging and histopathological findings in PH-LHD based on three clinical cases.
MeSH term(s)	Humans ; Hypertension, Pulmonary/diagnostic imaging ; Hypertension, Pulmonary/etiology ; Heart Diseases/complications ; Heart Failure ; Pulmonary Arterial Hypertension ; Fibrosis
Language	English
Publishing date	2024-02-28
Publishing country	England
Document type	Journal Article
ZDB-ID	1077620-5
ISSN	1600-0617 ; 0905-9180
ISSN (online)	1600-0617
ISSN	0905-9180
DOI	10.1183/16000617.0144-2023
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Zs.A 3265: Show issues

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Article ; Online: Pulmonary veno-occlusive disease: illustrative cases and literature review.

Lechartier, Benoit / Boucly, Athénaïs / Solinas, Sabina / Gopalan, Deepa / Dorfmüller, Peter / Radonic, Teodora / Sitbon, Olivier / Montani, David

European respiratory review : an official journal of the European Respiratory Society

2024 Volume 33, Issue 171

Abstract: Pulmonary veno-occlusive disease (PVOD), also known as "pulmonary arterial hypertension (PAH) with overt features of venous/capillary involvement", is a rare cause of PAH characterised by substantial small pulmonary vein and capillary involvement, ... ...

Abstract	Pulmonary veno-occlusive disease (PVOD), also known as "pulmonary arterial hypertension (PAH) with overt features of venous/capillary involvement", is a rare cause of PAH characterised by substantial small pulmonary vein and capillary involvement, leading to increased pulmonary vascular resistance and right ventricular failure. Environmental risk factors have been associated with the development of PVOD, such as occupational exposure to organic solvents and chemotherapy, notably mitomycin. PVOD may also be associated with a mutation in the
MeSH term(s)	Humans ; Pulmonary Veno-Occlusive Disease/etiology ; Pulmonary Veno-Occlusive Disease/genetics ; Lung/diagnostic imaging ; Familial Primary Pulmonary Hypertension ; Tomography, X-Ray Computed/methods ; Lung Transplantation/adverse effects ; Pulmonary Arterial Hypertension ; Protein Serine-Threonine Kinases
Chemical Substances	EIF2AK4 protein, human (EC 2.7.11.1) ; Protein Serine-Threonine Kinases (EC 2.7.11.1)
Language	English
Publishing date	2024-01-17
Publishing country	England
Document type	Review ; Journal Article
ZDB-ID	1077620-5
ISSN	1600-0617 ; 0905-9180
ISSN (online)	1600-0617
ISSN	0905-9180
DOI	10.1183/16000617.0156-2023
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Zs.A 3265: Show issues

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Article ; Online: A Novel Rat Model of Mild Pulmonary Hypertension Associated with Pulmonary Venous Congestion Induced by Left Pulmonary Vein Banding.

Münks, Jonas / Yogeswaran, Athiththan / Antoine, Tobiah Kevin / Blumrich, Leonhard Anton / Dorfmüller, Peter / Ghofrani, Hossein Ardeschir / Assmus, Birgit / Schermuly, Ralph Theo / Sydykov, Akylbek

International journal of molecular sciences

2024 Volume 25, Issue 5

Abstract: Pulmonary hypertension (PH) associated with left heart disease (PH-LHD) is the most common form of PH. In PH-LHD, changes in the pulmonary vasculature are assumed to be mainly caused by pulmonary venous congestion. However, the underlying mechanisms of ... ...

Abstract	Pulmonary hypertension (PH) associated with left heart disease (PH-LHD) is the most common form of PH. In PH-LHD, changes in the pulmonary vasculature are assumed to be mainly caused by pulmonary venous congestion. However, the underlying mechanisms of this form of PH are poorly understood. We aimed to establish a model of PH associated with pulmonary venous congestion. Wistar-Kyoto rats underwent partial occlusion of the left pulmonary vein to induce pulmonary venous congestion or sham surgery and were assessed at various time points post-surgery (3, 6, 9, 12 weeks). In vivo cardiopulmonary phenotyping was performed by using echocardiography along with heart catheterization. Histomorphometry methods were used to assess pulmonary vascular remodeling (e.g., wall thickness, degree of muscularization). Left pulmonary vein banding (PVB) resulted in mildly elevated right ventricular systolic pressure and moderate right ventricular hypertrophy. In PVB rats, small- and medium-sized pulmonary vessels in the left lung were characterized by increased wall thickness and muscularization. Taken together, our data demonstrate that left PVB-induced pulmonary venous congestion is associated with pulmonary vascular remodeling and mild PH.
MeSH term(s)	Rats ; Animals ; Hypertension, Pulmonary ; Pulmonary Veins ; Vascular Remodeling ; Hyperemia ; Rats, Inbred WKY
Language	English
Publishing date	2024-02-29
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2019364-6
ISSN	1422-0067 ; 1422-0067 ; 1661-6596
ISSN (online)	1422-0067
ISSN	1422-0067 ; 1661-6596
DOI	10.3390/ijms25052827
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: Chronic thromboembolic pulmonary hypertension: the magic of pathophysiology.

Simonneau, Gérald / Dorfmüller, Peter / Guignabert, Christophe / Mercier, Olaf / Humbert, Marc

Annals of cardiothoracic surgery

2022 Volume 11, Issue 2, Page(s) 106–119

Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and underdiagnosed complication of acute pulmonary embolism (APE). CTEPH is a common cause of pulmonary hypertension (PH) with distinct management strategy including pulmonary endarterectomy, ...

Abstract	Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and underdiagnosed complication of acute pulmonary embolism (APE). CTEPH is a common cause of pulmonary hypertension (PH) with distinct management strategy including pulmonary endarterectomy, balloon pulmonary angioplasty, long-term anticoagulation and PH drugs targeting endothelial cell dysfunction. Initially, PH in chronic thromboembolic pulmonary disease (CTEPD) was thought to be due exclusively to the intravascular obstruction of pulmonary arteries by unresolved fibrotic clots. However, it is now well accepted that pulmonary vascular remodelling can include significant pulmonary microvasculopathy, which plays a role in the development of CTEPH. The histological description and clinical consequences of CTEPH microvasculopathy are now better understood. These lesions may involve not only small muscular pulmonary arteries <500 µm, but also pulmonary capillaries and veins. In addition, enlargement and proliferation of systemic bronchial arteries as well as anastomoses between the systemic and pulmonary circulations contribute to the development of microvasculopathy. In this review, we discuss the recent advances in the understanding of the pathophysiology of CTEPH.
Language	English
Publishing date	2022-03-19
Publishing country	China
Document type	Journal Article ; Review
ZDB-ID	2713627-9
ISSN	2304-1021 ; 2225-319X
ISSN (online)	2304-1021
ISSN	2225-319X
DOI	10.21037/acs-2021-pte-10
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Smouldering fire or conflagration? An illustrated update on the concept of inflammation in pulmonary arterial hypertension.

Perros, Frédéric / Humbert, Marc / Dorfmüller, Peter

European respiratory review : an official journal of the European Respiratory Society

2021 Volume 30, Issue 162

Abstract: Pulmonary arterial hypertension (PAH) is a rare condition that is characterised by a progressive increase of pulmonary vascular resistances that leads to right ventricular failure and death, if untreated. The underlying narrowing of the pulmonary ... ...

Abstract	Pulmonary arterial hypertension (PAH) is a rare condition that is characterised by a progressive increase of pulmonary vascular resistances that leads to right ventricular failure and death, if untreated. The underlying narrowing of the pulmonary vasculature relies on several independent and interdependent biological pathways, such as genetic predisposition and epigenetic changes, imbalance of vasodilating and vasoconstrictive mediators, as well as dysimmunity and inflammation that will trigger endothelial dysfunction, smooth muscle cell proliferation, fibroblast activation and collagen deposition. Progressive constriction of the pulmonary vasculature, in turn, initiates and sustains hypertrophic and maladaptive myocardial remodelling of the right ventricle. In this review, we focus on the role of inflammation and dysimmunity in PAH which is generally accepted today, although existing PAH-specific medical therapies still lack targeted immune-modulating approaches.
MeSH term(s)	Familial Primary Pulmonary Hypertension ; Heart Ventricles ; Humans ; Inflammation/diagnosis ; Pulmonary Arterial Hypertension ; Pulmonary Artery
Language	English
Publishing date	2021-12-22
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	1077620-5
ISSN	1600-0617 ; 0905-9180
ISSN (online)	1600-0617
ISSN	0905-9180
DOI	10.1183/16000617.0161-2021
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: Pulmonary hypertension: pathology.

Dorfmüller, Peter

Handbook of experimental pharmacology

2013 Volume 218, Page(s) 59–75

Abstract: Pulmonary hypertension (PH) is a life-threatening and often fatal disease, characterized by elevated pulmonary vascular resistance and secondary right ventricular failure. Since etiologies of PH are multiple and its pathogenesis is complex, histology ... ...

Abstract	Pulmonary hypertension (PH) is a life-threatening and often fatal disease, characterized by elevated pulmonary vascular resistance and secondary right ventricular failure. Since etiologies of PH are multiple and its pathogenesis is complex, histology from lungs of patients with PH may help us to determine different etiological factors of the disease. The degree of involvement of various cell types and structures within the lung tissue represents an important indicator of the pathophysiologal process. So even if the role for pathologists in routine management of PH is limited, lessons can be learned from morphology. The present chapter outlines the current understanding of this disease from the pathologist's point of view.
MeSH term(s)	Animals ; Connective Tissue Diseases/pathology ; Fibrosis ; Humans ; Hypertension, Pulmonary/pathology ; Hypertrophy ; Pulmonary Artery/pathology ; Pulmonary Veno-Occlusive Disease/pathology
Language	English
Publishing date	2013
Publishing country	Germany
Document type	Journal Article ; Review
ISSN	0171-2004
ISSN	0171-2004
DOI	10.1007/978-3-642-38664-0_3
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Group 3 Pulmonary Hypertension: From Bench to Bedside.

Singh, Navneet / Dorfmüller, Peter / Shlobin, Oksana A / Ventetuolo, Corey E

Circulation research

2022 Volume 130, Issue 9, Page(s) 1404–1422

Abstract: Pulmonary hypertension (PH) because of chronic lung disease is categorized as Group 3 PH in the most recent classification system. Prevalence of these diseases is increasing over time, creating a growing need for effective therapeutic options. Recent ... ...

Abstract	Pulmonary hypertension (PH) because of chronic lung disease is categorized as Group 3 PH in the most recent classification system. Prevalence of these diseases is increasing over time, creating a growing need for effective therapeutic options. Recent approval of the first pulmonary arterial hypertension therapy for the treatment of Group 3 PH related to interstitial lung disease represents an encouraging advancement. This review focuses on molecular mechanisms contributing to pulmonary vasculopathy in chronic hypoxia, the pathology and epidemiology of Group 3 PH, the right ventricular dysfunction observed in this population and clinical trial data that inform the use of pulmonary vasodilators in Group 3 PH.
MeSH term(s)	Humans ; Hypertension, Pulmonary/drug therapy ; Hypertension, Pulmonary/epidemiology ; Lung ; Lung Diseases, Interstitial/complications ; Lung Diseases, Interstitial/drug therapy ; Vasodilator Agents ; Ventricular Dysfunction, Right/drug therapy
Chemical Substances	Vasodilator Agents
Language	English
Publishing date	2022-04-28
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	80100-8
ISSN	1524-4571 ; 0009-7330 ; 0931-6876
ISSN (online)	1524-4571
ISSN	0009-7330 ; 0931-6876
DOI	10.1161/CIRCRESAHA.121.319970
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Ui IV Zs.70: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
Ui IV Zs.60: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)

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Article ; Online: Physiopathologie et traitements de l’hypertension artérielle pulmonaire.

Perros, Frédéric / Jutant, Étienne-Marie / Savale, Laurent / Dorfmüller, Peter / Humbert, Marc / Montani, David

Medecine sciences : M/S

2023 Volume 39, Issue 4, Page(s) 359–369

Abstract: Pulmonary arterial hypertension (PAH) is a rare disease affecting mainly the pre-capillary pulmonary vascular bed. However, some forms of the disease have venous/capillary involvement. It is an obstructive remodelling of the pulmonary arterioles coupled ... ...

Title translation	Physiopathology and treatment of pulmonary arterial hypertension.
Abstract	Pulmonary arterial hypertension (PAH) is a rare disease affecting mainly the pre-capillary pulmonary vascular bed. However, some forms of the disease have venous/capillary involvement. It is an obstructive remodelling of the pulmonary arterioles coupled with vascular pruning, increasing right ventricular afterload and leading to right heart failure. PAH has a complex pathogeny that is detailed in this review. Current specific treatments target endothelial dysfunction, and primarily aim at vasodilatation. Promising innovative treatments targeting the pulmonary artery remodelling are under development.
MeSH term(s)	Humans ; Heart Failure ; Hypertension, Pulmonary ; Pulmonary Arterial Hypertension/physiopathology ; Pulmonary Arterial Hypertension/therapy
Language	French
Publishing date	2023-04-24
Publishing country	France
Document type	Review ; English Abstract ; Journal Article
ZDB-ID	632733-3
ISSN	1958-5381 ; 0767-0974
ISSN (online)	1958-5381
ISSN	0767-0974
DOI	10.1051/medsci/2023053
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