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Review

Chronic Thromboembolic Pulmonary Hypertension

by
Séverine Müller-Mottet
1,
Elisabeth Hasler
1,
Isabelle Opitz
2,
Walter Weder
2,
Reto A. Schuepbach
3,
Rudolf Speich
1 and
Silvia Ulrich
1,*
1
Clinic for Pulmonology, University Hospital, Zürich, Switzerland
2
Division of Thoracic Surgery, University Hospital, Zürich, Switzerland
3
Division of Surgical Intensive Care Medicine, University Hospital, Zürich, Switzerland
*
Author to whom correspondence should be addressed.
Cardiovasc. Med. 2014, 17(11), 328; https://doi.org/10.4414/cvm.2014.00285
Submission received: 19 August 2014 / Revised: 19 September 2014 / Accepted: 19 October 2014 / Published: 19 November 2014

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare and potentially fatal disease which occasionally occurs as a complication of acute pulmonary embolism. The mechanisms leading to failure of thrombus resolution are not completely understood with only some risk factors identified. Vascular alterations are present in the large and small vessel compartment. Signs and symptoms of CTEPH are nonspecific, rendering diagnosis challenging. A VQ scan followed by computed tomography, magnetic resonance or conventional pulmonary angiography is mandatory to confirm diagnosis and assess operability by a multidisciplinary team. Pulmonary endarterectomy remains the treatment of choice in operable patients and results in significantly improved haemodynamics and functional capacity. In inoperable patients medical treatment is well defined, and mainly includes the recently introduced pharmacological substances with impact on haemodynamics and functional performance.
Keywords: chronic thromboembolic pulmonary hypertension chronic thromboembolic pulmonary hypertension

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MDPI and ACS Style

Müller-Mottet, S.; Hasler, E.; Opitz, I.; Weder, W.; Schuepbach, R.A.; Speich, R.; Ulrich, S. Chronic Thromboembolic Pulmonary Hypertension. Cardiovasc. Med. 2014, 17, 328. https://doi.org/10.4414/cvm.2014.00285

AMA Style

Müller-Mottet S, Hasler E, Opitz I, Weder W, Schuepbach RA, Speich R, Ulrich S. Chronic Thromboembolic Pulmonary Hypertension. Cardiovascular Medicine. 2014; 17(11):328. https://doi.org/10.4414/cvm.2014.00285

Chicago/Turabian Style

Müller-Mottet, Séverine, Elisabeth Hasler, Isabelle Opitz, Walter Weder, Reto A. Schuepbach, Rudolf Speich, and Silvia Ulrich. 2014. "Chronic Thromboembolic Pulmonary Hypertension" Cardiovascular Medicine 17, no. 11: 328. https://doi.org/10.4414/cvm.2014.00285

APA Style

Müller-Mottet, S., Hasler, E., Opitz, I., Weder, W., Schuepbach, R. A., Speich, R., & Ulrich, S. (2014). Chronic Thromboembolic Pulmonary Hypertension. Cardiovascular Medicine, 17(11), 328. https://doi.org/10.4414/cvm.2014.00285

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