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The risk of developing chronic thromboembolic pulmonary hypertension (CTPH) following pulmonary embolism (PE) has not been clearly defined. In this prospective study 223 patients diagnosed with a first symptomatic PE were followed for a median period of 94.3 months. Patients with conditions predisposing to non-thromboembolic pulmonary hypertension were excluded from the study, as were those with a previous PE or deep vein thrombosis (DVT). Patients who developed unexplained breathlessness during follow up were investigated with transthoracic echocardiography. V/Q scanning and pulmonary angiography followed when echocardiography suggested CTPH. CTPH was diagnosed on the basis of pulmonary artery pressures and angiographic findings. Seven patients developed CTPH. The cumulative incidence of CTPH at 6 months was 1%, at 1 year 3.1%, and at 2 years 3.8%. No cases of CTPH presented more than 2 years after the initial diagnosis of PE.
A further 82 patients with acute PE and previous thromboembolism were included in an analysis of risk factors for CTPH; 11 of these patients developed CTPH. Risk factors included previous PE, or PE presenting at a younger age, with a larger perfusion defect, or in the absence of an identifiable risk factor.
This study suggests that CTPH occurs earlier and more frequently than previously estimated. As a serious and relatively common complication of PE, more attention should be focused on prevention, early detection, and treatment. Prevention of recurrent PE by improvements in the treatment of PE and DVT should reduce the incidence of CTPH.
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