Chronic thromboembolic pulmonary hypertension

Abstract

Chronic pulmonary hypertension in the course of venous thromboembolic disease (CTEPH) is defined as mean pulmonary artery pressure ≥ 25 mm Hg persistent for more than 6 months after the acute episode of pulmonary embolism. This relatively rare, but potentially serious disease is caused by a partial closure of the pulmonary arteries by organized thrombus. The clinical presentation is non-specific. As in other forms of pulmonary hypertension, the main symptom is progressive reduction in exercise tolerance due to dyspnoea or fatigability. The insidious and progressive course of the disease and a lack of awareness of CTEPH in both the medical community and among patients result in significant delay from onset of symptoms to diagnosis. Transthoracic echocardiography remains a valuable initial diagnostic tool in suspicion of pulmonary hypertension. Diagnostic gold standard in the diagnosis of chronic thromboembolic pulmonary hypertension is still pulmonary arteriography with right heart catheterization. In cases in which the thrombus is located in the proximal parts of the pulmonary artery and its branches there is a possibility of effective treatment with surgical pulmonary endarterectomy.