Prognostic and Aetiological Factors in Chronic Thromboembolic Pulmonary Hypertension

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

^* To whom correspondence should be addressed. E-mail: joanna.pepkezaba{at}papworth.nhs.uk.

	Abstract

Several prognostic variables have been previously 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 we have assessed the prognostic value of a larger number of variables. We 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 non-operable disease. Previous splenectomy was noted in 6.7% of patients, being significantly more common in patients with non-operable than operable disease (13.7% v 3.6%, p<0.001). We did not find that medical risk factors predicted mortality.

In this large national cohort, predictors of outcome in patients with both operable and non-operable CTEPH have been identified. These may be useful in planning treatment. The aetiological importance of previously identified medical risk factors has been confirmed, although we were unable to validate their prognostic strength.