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The Right Heart in Chronic Thromboembolic Pulmonary Hypertension

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The Right Heart

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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon complication of pulmonary embolism that results from mechanical obstruction of proximal pulmonary vessels by non-resolving thromboemboli and distal arteriopahty due to non-occluded area overperfusion. Increased right ventricular (RV) afterload challenges the RV and leads to changes in its morphology and function: RV hypertrophy is followed by tricuspid regurgitation, dilatation and left-ward bowing of the interventricular septum. Sustained pressure overload results in RV failure and death.

Pulmonary endarterectomy (PEA) represents the treatment of choice. In a vast majority of affected patients, organized thrombotic material can effectively be removed by PEA, which in turn leads to a reduction in RV afterload. As a result, most of the RV hemodynamic and structural changes reverse to near normal. In parallel, patients’ clinical condition and long-term outcome improve. In some cases PEA can be insufficient due to accompanying small vessel disease, or not possible due to distal thrombus location and/or patients co-morbidities. For patients in whom surgery is not an option or insufficient, recent trials with beneficial pharmacotherapeutic effects have raised new hopes.

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Authors and Affiliations

  1. Department of Internal Medicine II, Medical University of Vienna, Währingergürtel 18-20, Vienna, 1090, Austria

    Stefan Aschauer MD, Irene M. Lang MD & Diana Bonderman MD

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  1. Stefan Aschauer MD
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  2. Irene M. Lang MD
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  3. Diana Bonderman MD
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Correspondence to Diana Bonderman MD .

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Editors and Affiliations

  1. National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland

    Sean P. Gaine

  2. Department of Cardiology, Erasme University Hospital, Brussels, Belgium

    Robert Naeije

  3. Scottish Pulmonary Vascular Unit, Regional Heart and Lung Centre, Glasgow, United Kingdom

    Andrew John Peacock

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Aschauer, S., Lang, I.M., Bonderman, D. (2014). The Right Heart in Chronic Thromboembolic Pulmonary Hypertension. In: Gaine, S., Naeije, R., Peacock, A. (eds) The Right Heart. Springer, London. https://doi.org/10.1007/978-1-4471-2398-9_15

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