Chronic thromboembolic pulmonary hypertension shares all of the clinical features of idiopathic pulmonary arterial hypertension and is similarly uncommon. In approximately half of the cases there is no history of antecedent acute pulmonary thromboembolism. A minority of patients who have a predisposing risk for thrombosis, such as a history of leg injuries, immobilization, heart failure, cancer or a circulating anticoagulant. A pulmonary angiogram can confirm the diagnosis.
Pulmonary angiogram
This is a pulmonary angiogram in the anterior posterior view from another patient with known chronic thromboembolic pulmonary hypertension. Note the following characteristics: there is enlargement of the central pulmonary artery; there are abrupt vascular cut-offs representing the thromboemboli; there are also stenoses, or vascular narrowings, indicative of old recanalized thrombus.
Operative specimen
This operative specimen was removed from a patient with chronic pulmonary thromboembolic disease. Note that the thromboemboli are well endothelialized and appears as a cast of the underlying pulmonary arterial tree. Because of the chronic nature of these thromboemboli, they are not amenable to thrombolysis with lytic agents and, thus, surgical removal is the only possible treatment.