Improved outcomes in medically and surgically treated chronic thromboembolic pulmonary hypertension.
BACKGROUND
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.


OBJECTIVE
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.


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.
Condliffe
Robin
R
Pulmonary Vascular Disease Unit, Papworth Hospital, Cambridge CB23 3RE, UK.
Kiely
David G
DG
Gibbs
J Simon R
JS
Corris
Paul A
PA
Peacock
Andrew J
AJ
Jenkins
David P
DP
Hodgkins
Denise
D
Goldsmith
Kim
K
Hughes
Rodney J
RJ
Sheares
Karen
K
Tsui
Steven S L
SS
Armstrong
Iain J
IJ
Torpy
Chantal
C
Crackett
Rachel
R
Carlin
Christopher M
CM
Das
Clare
C
Coghlan
J Gerry
JG
Pepke-Zaba
Joanna
J
D000328:Adult; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D002908:Chronic Disease; D015331:Cohort Studies; D004691:Endarterectomy / Q000534:rehabilitation*; D005080:Exercise Test; D017079:Exercise Tolerance / Q000187:drug effects; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use*; D011651:Pulmonary Artery / Q000473:pathology / Q000601:surgery; D011652:Pulmonary Circulation; D012189:Retrospective Studies; D016019:Survival Analysis; D013923:Thromboembolism / Q000150:complications / Q000401:mortality; D016896:Treatment Outcome; D006113:United Kingdom / Q000453:epidemiology
D000959:Antihypertensive Agents; D010975:Platelet Aggregation Inhibitors