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Chronic thromboembolic pulmonary hypertension


Müller-Mottet, Séverine; Hasler, Elisabeth D; Opitz, Isabelle; Weder, Walter; Schuepbach, Reto A; Speich, Rudolf; Ulrich, Silvia (2014). Chronic thromboembolic pulmonary hypertension. Cardiovascular Medicine, 17(11):328-333.

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.

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.

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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Pneumology
Dewey Decimal Classification:610 Medicine & health
Uncontrolled Keywords:chronic thromboembolic pulmonary hypertension
Language:English
Date:November 2014
Deposited On:13 Jan 2015 09:46
Last Modified:05 Apr 2016 18:42
Publisher:EMH Swiss Medical Publishers
ISSN:1423-5528
Free access at:Official URL. An embargo period may apply.
Official URL:http://www.cardiovascmed.ch/docs/cvm/2014/11/en/cvm_00285.pdf
Related URLs:http://www.cardiovascmed.ch/

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