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Standard PAH therapy improves long term survival in CTEPH patients

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Abstract

Background

Chronic thromboembolic pulmonary hypertension (CTEPH), subsequent to pulmonary embolism is a relatively frequent cause of pulmonary hypertension. Similar to patients with pulmonary arterial hypertension (PAH), CTEPH carries a poor prognosis. There is no hard evidence for any other therapy except pulmonary endarterectomy and none for those patients that are not eligible for this procedure.

Patients and methods

Fifty patients with confirmed, inoperable CTEPH receiving specific vasodilative therapy (prostanoids, endothelin receptor antagonists, PDE 5-inhibitors or combination) were included in this retrospective study (mean age 55 years, range 16–76 years; 36 female, 14 male). Kaplan–Meier plots of these patients were compared with Kaplan–Meier plots of two historical CTEPH patient groups without any specific vasodilative treatment by log rank tests.

Results

CTEPH patients treated with specific vasodilative compounds as used for therapy of PAH were followed up for 52 ± 30 months and had a significantly improved survival compared with patients treated without PAH type vasodilators (p ≤ 0.0002).

Conclusion

Our data may generate the hypothesis that specific vasodilative treatment improves outcome in patients with inoperable CTEPH.

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Abbreviations

CI:

Cardiac index

CTEPH:

Chronic thromboembolic pulmonary hypertension

IPAH:

Idiopathic pulmonary arterial hypertension

mPAP:

Mean pulmonary arterial pressure

PAH:

Pulmonary arterial hypertension

PEA:

Pulmonary endarterectomy

PVR:

Pulmonary vascular resistance

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Acknowledgments

Dr. Seyfarth, Dr. Halank, Dr. Wilkens, Dr. Wirtz and Dr. Ewert have received speaker’s honorariums from Actelion Pharmaceuticals and Schering. The present study was not funded by a third party.

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Authors and Affiliations

  1. Department of Respiratory Medicine, University of Leipzig, Liebigstrasse 20, 04103, Leipzig, Germany

    Hans-Juergen Seyfarth, Hans Pankau, Stefan Hammerschmidt & Hubert Wirtz

  2. Internal Medicine I, University of Dresden, Dresden, Germany

    Michael Halank

  3. Universitätsklinikum Homburg/Saar, Homburg, Germany

    Heinrike Wilkens & Hans-Joachim Schäfers

  4. University of Greifswald, Greifswald, Germany

    Ralf Ewert

  5. Deutsches Herzzentrum, Klinikum Der TU München, Munich, Germany

    Martin Riedel

  6. Institut for Medical Informatics, Statistics and Epidemiology, University of Leipzig, Leipzig, Germany

    Ernst Schuster

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  1. Hans-Juergen Seyfarth
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  2. Michael Halank
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  3. Heinrike Wilkens
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  4. Hans-Joachim Schäfers
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  7. Ernst Schuster
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  8. Hans Pankau
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Correspondence to Hans-Juergen Seyfarth.

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Seyfarth, HJ., Halank, M., Wilkens, H. et al. Standard PAH therapy improves long term survival in CTEPH patients. Clin Res Cardiol 99, 553–556 (2010). https://doi.org/10.1007/s00392-010-0156-4

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  • DOI: https://doi.org/10.1007/s00392-010-0156-4

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