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Pulmonary Hypertension After PE More Common Than Previously Thought

Jason Cota, Pharm.D. Student at the University of Texas at Austin
Henry I. Bussey, Pharm.D.
October 2004

A recent study and accompanying editorial found that chronic thromboembolic pulmonary hypertension (CTHP) occurs in more than 3% of patients with pulmonary embolism (PE). Further, risk factors for CTHP appear to be younger age, previous PE, idiopathic cause, and size of PE.

The Study

This prospective, long-term follow-up study evaluated 223 patients presenting with an acute PE without previous thromboembolic events in order to estimate the incidence of chronic thromboembolic pulmonary hypertension (CTPH). Investigators diagnosed CTPH in 7 of 223 patients (3.1%) with a mean time from qualifying PE to CTPH diagnosis of 9 months (range of 3 to 21 months). Kaplan-Meier analysis showed the cumulative incidence of CTPH was 3.8% at 2 years.

For risk factor analysis, the investigators also included an additional 82 PE patients who had a history of previous PE or DVT for a total of 305 patients. Of these 305, 18 patients (5.9%) developed CTPH and the 287 patients who did not develop CTPH were termed control patients. The mean age of the CTPH group was 48.6 years compared to 62 years in the control group. Previous PE was identified in 8 of 18 CTPH patients (44.4%) as compared to 16 of 287 (5.6%) control patients. Ten patients in the CTPH group (55.6%) presented with idiopathic PE compared to 55 (19.2%) of the controls. Finally, mean perfusion defect in CTPH group was 62.6% compared to 33.7% in the control group. These patient characteristics were identified as risk factors for CTPH.

The Editorial

Lang points out that the 3.8% incidence of CTPH at 2 years actually may be higher because the initial thromboembolic event in CTPH can be asymptomatic in most patients. Although the presence of lupus anticoagulant, anticardiolipin antibodies or elevated factor VIII levels (termed "permanent risk factors" in the study) were not identified as risk factors for CTPH, 55.6% of CTPH patients had an idiopathic PE and may have had these underlying abnormalities. The authors conclude that CTPH following PE is relatively common and perhaps recognizing those patients at risk, in addition to achieving better anticoagulation, would prevent the development of CTPH.


1. Pengo V, Lensing AW, Prins MH, et al. Incidence of Chronic Thromboembolic Pulmonary Hypertension after Pulmonary Embolism. New England Journal of Medicine 2004; 350:2257-2264.

2. (accompanying editorial): Lang IM. Chronic thromboembolic pulmonary hypertension--Not so rare after all. New England Journal of Medicine 2004; 350:2236-38

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