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Published online before print October 1, 2008, 10.1183/09031936.00092008
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Eur Respir J 2009; 33:332-338
Copyright ©ERS Journals Ltd 2009

Prognostic and aetiological factors in chronic thromboembolic pulmonary hypertension

R. Condliffe1,2, D. G. Kiely2, J. S. R. Gibbs3, P. A. Corris4,5, A. J. Peacock6, D. P. Jenkins1, K. Goldsmith1, J. G. Coghlan7 and J. Pepke-Zaba1

1 Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, 2 Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, 3 Dept of Cardiology, Hammersmith Hospital, 7 Dept of Cardiology, Royal Free Hospital, London, 4 Northern Vascular Unit, Freeman Hospital, 5 Institute of Cellular Medicine, Newcastle University, Newcastle-upon-Tyne, and 6 Scottish Pulmonary Vascular Unit, Western Infirmary, Glasgow, UK.

CORRESPONDENCE: J. Pepke-Zaba, Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, CB23 3RE, UK. Fax: 44 1480364367. E-mail: joanna.pepkezaba{at}papworth.nhs.uk

Keywords: Endarterectomy, prognosis, pulmonary hypertension, thromboembolism

Received: June 17, 2008
Accepted September 22, 2008

Several prognostic variables have previously been identified in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Specific medical conditions have also been associated with the development and prognosis of CTEPH. Using a national registry, the current authors have assessed the prognostic value of a larger number of variables and have also attempted to validate the clinical importance of previously identified aetiological factors.

Baseline information for all 469 CTEPH patients diagnosed in the UK pulmonary hypertension service between January 2001 and June 2006 was collected from hospital records.

Although univariate analysis confirmed the prognostic importance of pulmonary resistance, in multivariate analysis gas transfer and exercise capacity predicted pulmonary endarterectomy perioperative mortality. Cardiac index and exercise capacity independently predicted outcome in patients with nonoperable disease. Previous splenectomy was noted in 6.7% of patients, being significantly more common in patients with nonoperable than operable disease (13.7 versus 3.6%). Medical risk factors were not found to predict mortality.

In a large national cohort, predictors of outcome in patients with both operable and nonoperable chronic thromboembolic pulmonary hypertension have been identified. These may be useful in planning treatment. The aetiological importance of previously identified medical risk factors has been confirmed, although the current authors were unable to validate their prognostic strength.




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