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Published ahead of print on February 21, 2008, doi:10.1164/rccm.200712-1841OC

Am. J. Respir. Crit. Care Med., Volume 177, Number 10, May 2008, 1122-1127

A more recent version of this article appeared on May 15, 2008
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Submitted on December 17, 2007
Accepted on February 21, 2008

Improved Outcomes in Medically and Surgically Treated Chronic Thromboembolic Pulmonary Hypertension

Robin Condliffe1, David G Kiely2, J Simon R Gibbs3, Paul A Corris4, Andrew J Peacock5, David P Jenkins6, Denise Hodgkins6, Kim Goldsmith6, Rodney J Hughes6, Karen Sheares6, Steven SL Tsui6, Iain J Armstrong2, Chantal Torpy3, Rachel Crackett7, Christopher M Carlin5, Clare Das8, J Gerry Coghlan8, and Joanna Pepke-Zaba6*

1 Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, United Kingdom; Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom, 2 Pulmonary Vascular Disease Unit, Royal Hallamshire Hospital, Sheffield, United Kingdom, 3 Department of Cardiology, Hammersmith Hospital, London, United Kingdom, 4 Northern Pulmonary Vascular Unit, Freeman Hospital, Newcastle-upon-Tyne, United Kingdom; 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 Northern Pulmonary Vascular Unit, Freeman Hospital, Newcastle-upon-Tyne, United Kingdom, 8 Department of Cardiology, Royal Free Hospital, London, United Kingdom

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

Rationale: The management of chronic thromboembolic pulmonary hypertension (CTEPH) has changed over recent years with the growth of pulmonary endarterectomy surgery and the availability of disease-modifying therapies. Objectives: To investigate the prognosis of CTEPH in a national setting during this recent period. Methods: All incident cases diagnosed in one of the 5 pulmonary hypertension centres in the UK between January 2001 and June 2006 were identified prospectively. Information regarding baseline characteristics, treatment and follow-up was subsequently collected from hospital records. Measurements and Main Results: 469 patients were diagnosed of which 148 (32%) had distal, non-surgical disease. One and three year survival from diagnosis was 82% and 70% for patients with non-surgical disease and 88% and 76% for those treated surgically (p=0.023). Initial functional improvement in patients with non-surgical disease was noted but did not persist at 2 years. Significant functional and haemodynamic improvements were seen in surgically-treated patients with an increase in six minute walk distance of 105 m (p<0.001) at 3 months. Five year survival from surgery in the 35% of patients who survived to three months but had persistent pulmonary hypertension was 94%. Conclusions: The prognosis in non-surgical disease has improved. We have confirmed the previously described good outcome in surgically-treated disease. However, we have also demonstrated that the long term prognosis for patients who have persistent pulmonary hypertension at 3 months following surgery is good. The observed improvements in outcome during the modern treatment era reinforce the importance of identifying patients with this increasingly treatable condition.
Key words: pulmonary hypertension, thromboembolism, endarterectomy, survival, drug therapy




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