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Chronic thromboembolic pulmonary hypertension is a "new" condition (in terms of our recognition and understanding of it), whose true prevalence, natural history, and response to therapies continue to be elucidated. Meanwhile, specialized surgical centers continually make thromboendarterectomy safer, providing definitive cures that are nothing short of miraculous for those affected by this otherwise usually fatal disease.
Pepke-Zaba et al report results from 679 patients with a new diagnosis of CTEPH over 2 years (2007-2009) in an international registry in Europe and Canada (the great Madani et al at the Sulpizio Center at San Diego was not included). All patients were thoroughly worked-up, with diagnoses made by right heart catheterization, computed tomography, ventilation-perfusion imaging, and/or pulmonary angiography. The highlights included:
- 25% had no history of acute pulmonary embolism.
- Most had gone undiagnosed more than one year since the onset of symptoms.
- After work-up, 63% were deemed operable and 36% were non-operable. A judgment of operability was more likely based on the distribution of disease (with central being operable, distal not), as the non-operable patients were no sicker than the operable patients on the measured variables (NYHA class and hemodynamics).
- 57% underwent pulmonary endarterectomy (386, or 90% of those considered operable). They had a mortality rate of 4.7%.
- A majority of the non-operable patients (54%) were given vasodilator therapy (only 24% of the operable patients got drug treatment). There's no clear evidence of benefit of vasodilator therapy in CTEPH, but it may help some people.
San Diego recently reported a mortality rate of only 2.2% in patients treated from 2006-2010, along with excellent outcomes in very ill patients. If you have a U.S. patient you suspect might have CTEPH, work them up and refer them to UCSD.
Pepke-Zaba et al. Chronic thromboembolic pulmonary hypertension (CTEPH): Results from an international prospective registry. Circulation 2011; 124:1973-1981.