Published Online
on April 9, 2007

A more recent version of this article appeared on April 24, 2007

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Submitted on August 28, 2006
Accepted on March 2, 2007

Predictors of Outcome in Chronic Thromboembolic Pulmonary Hypertension

Diana Bonderman MD, Nika Skoro-Sajer MD, Johannes Jakowitsch PhD, Christopher Adlbrecht MD, Daniela Dunkler MSc, Sharokh Taghavi MD, Walter Klepetko MD, Meinhard Kneussl MD, and Irene M. Lang MD^*

From the Departments of Cardiology (D.B., N.S.-S., J.J., C.A., I.M.L.), Core Unit for Medical Statistics and Informatics (D.D.), and Cardiothoracic Surgery (S.T., W.K.), Medical University of Vienna, and the Department of Pulmonology, Wilhelminenspital (M.K.), Vienna, Austria.

^* To whom correspondence should be addressed. E-mail: irene.lang{at}meduniwien.ac.at.

Background--Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by intraluminal thrombus organization and fibrous obliteration of pulmonary arteries. Recently, associated medical conditions such as splenectomy, ventriculoatrial shunt for the treatment of hydrocephalus, permanent central intravenous lines, inflammatory bowel disease, and osteomyelitis were found to be associated with the development of CTEPH. The study aim was to define the impact of these novel risk factors on survival.

Methods and Results--Between January 1992 and December 2006, 181 patients diagnosed with CTEPH were tracked with the use of our center’s customized computer database. A Cox regression model was used to examine relations between survival and associated medical conditions, age, sex, hemodynamic parameters, modified New York Heart Association functional class at diagnosis, CTEPH type, pulmonary endarterectomy, and anti-cardiolipin antibodies/lupus anticoagulant. During a median observation time of 22.1 (range, 0.03 to 152) months, the clinical end point of cardiovascular death or lung transplantation occurred in 48 cases (27%). Pulmonary endarterectomy (hazard ratio, 0.14; 95% CI, 0.05 to 0.41; P=0.0003), associated medical conditions (hazard ratio, 3.17; 95% CI, 1.70 to 5.92; P=0.0003), and pulmonary vascular resistance (hazard ratio, 1.02; 95% CI, 1.00 to 1.04; P=0.04) were predictors of survival. Thirty-day postoperative mortality (24% versus 9%) and the incidence of postoperative pulmonary hypertension (92% versus 20%) were substantially higher in patients with associated medical conditions.

Conclusions--CTEPH-predisposing medical conditions, such as splenectomy, permanent central intravenous lines, and certain inflammatory disorders, predict poor survival in CTEPH.

Key words: hypertension, pulmonary • survival • thrombosis

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