Clinical manifestations and diagnosis of chronic thromboembolic pulmonary hypertension
- Peter F Fedullo, MD
Peter F Fedullo, MD
- Clinical Professor of Medicine
- University of California San Diego Medical Center
Improvement following acute pulmonary embolism is usually sufficient to restore normal pulmonary hemodynamics, gas exchange, and exercise tolerance [1-3]. However, a minority of patients develop chronic thromboembolic pulmonary hypertension (CTEPH) following an acute pulmonary embolism .
The incidence, possible etiologies, presentation, clinical features, diagnosis, and differential diagnosis of CTEPH are reviewed here. Treatment of CTEPH is discussed separately. (See "Overview of the treatment of chronic thromboembolic pulmonary hypertension" and "Chronic thromboembolic pulmonary hypertension: Surgical treatment" and "Chronic thromboembolic pulmonary hypertension: Medical treatment".)
The true incidence of chronic thromboembolic pulmonary hypertension (CTEPH) is unknown but estimated to be between 1 and 5 percent among survivors of acute pulmonary embolism (PE) [5-10]. One retrospective series reported that among 899 patients with PE, approximately 1 percent developed CTEPH . In contrast, two prospective series reported that between 3.8 and 4.8 percent of patients developed CTEPH in a two-year period following the diagnosis of PE [6,7]. Another review of 16 studies suggested a prevalence of 0.5 percent in the general population that increases to 3 percent in survivors of PE .The development of CTEPH after two years is rare.
It is thought that CTEPH may represent the final manifestation of unresolved pulmonary embolism. With improved understanding of CTEPH, a population of patients has been identified with post-embolic exercise intolerance in the absence of resting pulmonary hypertension. This condition has variably been referred to as "chronic thromboembolic disease (CTED)" or "post-PE syndrome." Exercise intolerance may be due to increased dead space ventilation related to pulmonary vascular obstruction and/or limitation of maximal cardiac output with exercise [11,12]. A lower threshold to look for CTEPH in this population is appropriate.
It is unknown why some patients with acute pulmonary embolism develop chronic thromboembolic pulmonary hypertension (CTEPH) and others do not .
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