Tumoral pulmonary hypertension
File(s)
Published version
Author(s)
Price, Laura C
Seckl, Michael J
Dorfmüller, Peter
Wort, S John
Type
Journal Article
Abstract
Tumoral pulmonary hypertension (PH) comprises a variety of subtypes in patients with a current or previous malignancy. Tumoral PH principally includes the tumour-related pulmonary microvascular conditions pulmonary tumour microembolism and pulmonary tumour thrombotic microangiopathy. These inter-related conditions are frequently found in post mortem specimens but are notoriously difficult to diagnose ante mortem The outlook for patients remains extremely poor although there is some emerging evidence that pulmonary vasodilators and anti-inflammatory approaches may improve survival. Tumoral PH also includes pulmonary macroembolism and tumours that involve the proximal pulmonary vasculature, such as angiosarcoma; both may mimic pulmonary embolism and chronic thromboembolic PH. Finally, tumoral PH may develop in response to treatments of an underlying malignancy. There is increasing interest in pulmonary arterial hypertension induced by tyrosine kinase inhibitors, such as dasatanib. In addition, radiotherapy and chemotherapeutic agents such as mitomycin-C can cause pulmonary veno-occlusive disease. Tumoral PH should be considered in any patient presenting with unexplained PH, especially if it is poorly responsive to standard approaches or there is a history of malignancy. This article will describe subtypes of tumoral PH, their pathophysiology, investigation and management options in turn.
Date Issued
2019-03-31
Date Acceptance
2018-11-23
Citation
European Respiratory Review, 2019, 28 (151)
ISSN
0905-9180
Publisher
European Respiratory Society
Journal / Book Title
European Respiratory Review
Volume
28
Issue
151
Copyright Statement
©ERS 2019. ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0 (https://creativecommons.org/licenses/by-nc/4.0/)
Identifier
https://www.ncbi.nlm.nih.gov/pubmed/30728162
PII: 28/151/180065
Subjects
Respiratory System
Publication Status
Published
Coverage Spatial
England
Date Publish Online
2019-02-06