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Improved Outcomes in Medically and Surgically Treated Chronic Thromboembolic Pulmonary Hypertension

¹ Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge, 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; ⁵ Institute of Cellular Medicine, Newcastle University, Newcastle-upon-Tyne, United Kingdom; ⁶ Scottish Pulmonary Vascular Unit, Western Infirmary, Glasgow, United Kingdom; and ⁷ Department of Cardiology, Royal Free Hospital, London, United Kingdom

Correspondence and requests for reprints should be addressed to Dr. Joanna Pepke-Zaba, Ph.D., Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge CB23 3RE, UK. 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 recent years.

Methods: All incident cases diagnosed in one of the five pulmonary hypertension centers in the United Kingdom 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: A total of 469 patients received a diagnosis, of whom 148 (32%) had distal, nonsurgical disease. One- and three-year survival from diagnosis was 82 and 70% for patients with nonsurgical disease and 88 and 76% for those treated surgically (P = 0.023). Initial functional improvement in patients with nonsurgical disease was noted but did not persist at 2 years. Significant functional and hemodynamic 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 3 months but had persistent pulmonary hypertension was 94%.

Conclusions: The prognosis in nonsurgical 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 after 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

AT A GLANCE COMMENTARY Scientific Knowledge on the Subject Chronic thromboembolic pulmonary hypertension (CTEPH) occurs in up to 3.8% of patients after an acute pulmonary embolism. Historically, outcome was poor, but it has improved significantly in patients suitable for pulmonary endarterectomy. What This Study Adds to the Field Prognosis in nonsurgical CTEPH has improved. Long-term outcome in patients with persistent pulmonary hypertension at follow-up after pulmonary endarterectomy is good.