Chronic Thromboembolic Pulmonary Hypertension-Therapeutic Options

Authors: Seyfarth, Hans-Jurgen; Hammerschmidt, Stefan; Gessner, Christian; Halank, Michael; Wirtz, Hubert

Source: Current Respiratory Medicine Reviews, Volume 2, Number 4, November 2006 , pp. 431-438(8)

Publisher: Bentham Science Publishers

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Abstract:

Approximately 4% of all individuals that develop pulmonary embolism will go on to have chronic thromboembolic pulmonary hypertension (CTEPH). Patients with severe CTEPH will eventually be evaluated for thrombendarterectomy, the only curative treatment for this disease. However, only a subgroup of these patients is eligible for this invasive procedure. In addition, a number of patients will not benefit tremendously from thrombendarterectomy. Finally, thrombendarterectomy may not be possible for functional reasons. In these situations medical treatment, i.e. diuretics, oxygen and anticoagulation remains and is recommended. However, no proven specific e.g. vasodilating medical treatment has been demonstrated in CTEPH. Patients with CTEPH exhibit the same symptoms and a comparable prognosis compared with pulmonary arterial hypertension (PAH) patients. Although the occlusion of pulmonary vessels in CTEPH is caused by repetitive embolism histological similarities to PAH were observed in the pulmonary vascular bed, possibly a reaction to the developing high vascular pressure. Considering these similarities it would certainly make sense to study medical PAH treatment in patients with CTEPH. A few smaller studies exist, which do suggest beneficial effects of endothelin receptor antagonists, prostanoids and phosphodiesterase-5 antagonists in CTEPH. The evidence for the various forms of treatment of for CTEPH will be reviewed.

Keywords: Pulmonary embolism; pulmonary hypertension; thrombendarterectomy; drug therapy; lung transplantation

Document Type: Research article

DOI: http://dx.doi.org/10.2174/157339806778777186

Affiliations: 1: Medizinische Universitatsklinik I, Johannisallee 32, D-04103 Leipzig, Germany.

Publication date: 2006-11-01

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  • Current Respiratory Medicine Reviews publishes frontier reviews on all the latest advances on respiratory diseases and its related areas e.g. pharmacology, pathogenesis, clinical care, and therapy. The journal's aim is to publish the highest quality review articles dedicated to clinical research in the field. The journal is essential reading for all researchers and clinicians in respiratory medicine.
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