Predictors of outcome in chronic thromboembolic pulmonary hypertension

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.