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Semin Respir Crit Care Med 2012; 33(02): 199-204
DOI: 10.1055/s-0032-1311800
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Chronic Pulmonary Embolism and Pulmonary Hypertension

Frederikus A. Klok
1   Department of Thrombosis and Haemostasis, Leiden University Medical Center (LUMC), Leiden, The Netherlands.
2   Department of General Internal Medicine, Bronovo Hospital, Den Haag, The Netherlands.
,
Inge C.M. Mos
1   Department of Thrombosis and Haemostasis, Leiden University Medical Center (LUMC), Leiden, The Netherlands.
3   Department of Internal Medicine, MC Haaglanden, Den Haag, The Netherlands.
,
Klaus W. van Kralingen
4   Department of Pulmonary Medicine, Leiden University Medical Center, Leiden, The Netherlands.
,
Jelmer E. Vahl
2   Department of General Internal Medicine, Bronovo Hospital, Den Haag, The Netherlands.
5   Department of Pulmonary Medicine, HAGA Ziekenhuis, Den Haag, The Netherlands.
,
Menno V. Huisman
1   Department of Thrombosis and Haemostasis, Leiden University Medical Center (LUMC), Leiden, The Netherlands.
› Author Affiliations
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Publication History

Publication Date:
30 May 2012 (online)

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Abstract

Incomplete resolution of acute pulmonary embolism (PE) is frequently observed after acute PE and may rarely result in chronic thromboembolic pulmonary hypertension (CTEPH). The underlying pathophysiological mechanism is largely unknown. Evidence underlines the concept of a dual pulmonary vascular compartment model consisting of increased pulmonary vascular resistance by both large vessel obstruction and distal small vessel obliteration, the latter initiated by pathological vascular remodeling. Up to 40% of patients with established CTEPH have no prior history of symptomatic venous thromboembolism. CTEPH is associated with a poor prognosis if left untreated. Therefore, the diagnostic approach of CTEPH aims at assessing the location and extent of the embolic obstruction, establishing the operability and prognosis of the patients and ruling out other variations of pulmonary hypertension with distinct indicated treatment. Heart catheterization for invasive pressure measurements and pulmonary catheter angiography is obligatory for the final diagnosis. Pulmonary thromboendarterectomy is the treatment of choice. In certain patients with persistent or recurrent pulmonary hypertension after surgery or with inoperable disease, pharmacotherapy might be beneficial.

Keywords

pulmonary embolism - chronic thromboembolic pulmonary hypertension - diagnosis - therapy - prognosis