Next Article in Journal
A Control-Theoretic Model of Atherosclerosis
Previous Article in Journal
Neonicotinoid Insecticides Alter the Transcriptome of Soybean and Decrease Plant Resistance
Previous Article in Special Issue
Macrophage Migration Inhibitory Factor (MIF) Inhibition in a Murine Model of Bleomycin-Induced Pulmonary Fibrosis
Article Menu
Issue 3 (February-1) cover image

Export Article

Open AccessReview
Int. J. Mol. Sci. 2019, 20(3), 784;

Molecular Research in Chronic Thromboembolic Pulmonary Hypertension

Department of Thoracic Surgery, University Hospital Zurich, 8091 Zurich, Switzerland
Author to whom correspondence should be addressed.
Received: 31 December 2018 / Revised: 4 February 2019 / Accepted: 6 February 2019 / Published: 12 February 2019
(This article belongs to the Special Issue Molecular Research on Pulmonary Hypertension)
Full-Text   |   PDF [720 KB, uploaded 12 February 2019]   |  


Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a debilitating disease, for which the underlying pathophysiological mechanisms have yet to be fully elucidated. Occurrence of a pulmonary embolism (PE) is a major risk factor for the development of CTEPH, with non-resolution of the thrombus being considered the main cause of CTEPH. Polymorphisms in the α-chain of fibrinogen have been linked to resistance to fibrinolysis in CTEPH patients, and could be responsible for development and disease progression. However, it is likely that additional genetic predisposition, as well as genetic and molecular alterations occurring as a consequence of tissue remodeling in the pulmonary arteries following a persistent PE, also play an important role in CTEPH. This review summarises the current knowledge regarding genetic differences between CTEPH patients and controls (with or without pulmonary hypertension). Mutations in BMPR2, differential gene and microRNA expression, and the transcription factor FoxO1 have been suggested to be involved in the processes underlying the development of CTEPH. While these studies provide the first indications regarding important dysregulated pathways in CTEPH (e.g., TGF-β and PI3K signaling), additional in-depth investigations are required to fully understand the complex processes leading to CTEPH. View Full-Text
Keywords: chronic thromboembolic pulmonary hypertension; pathophysiology; genetic alterations; molecular factors; microRNAs; mutations; biomarkers chronic thromboembolic pulmonary hypertension; pathophysiology; genetic alterations; molecular factors; microRNAs; mutations; biomarkers

Figure 1

This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).

Share & Cite This Article

MDPI and ACS Style

Opitz, I.; Kirschner, M.B. Molecular Research in Chronic Thromboembolic Pulmonary Hypertension. Int. J. Mol. Sci. 2019, 20, 784.

Show more citation formats Show less citations formats

Note that from the first issue of 2016, MDPI journals use article numbers instead of page numbers. See further details here.

Related Articles

Article Metrics

Article Access Statistics



[Return to top]
Int. J. Mol. Sci. EISSN 1422-0067 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
Back to Top