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Chronic pulmonary thromboembolic disease is an insidious, life threatening condition that develops as a long term complication of the incomplete resolution of pulmonary embolism. Patients present with progressive breathlessness on exertion and the prognosis is poor owing to the development of pulmonary hypertension, with less than 30% five year survival. Pulmonary thromboendarterectomy has emerged as an effective treatment in selected patients.
Our patient was a 64 year old man with a five year history of progressive dyspnoea on effort following documented pulmonary embolism. He presented with severe exercise limitation (New York Heart Association functional class III) and signs of right ventricular hypertrophy. Invasive studies revealed a mean pulmonary arterial pressure of 65 mm Hg and a cardiac index of 2.06 1/min/m2. Contrast enhanced three dimensional magnetic resonance angiography (MRA) (below left) confirmed pulmonary thromboembolic disease with multiple abrupt occlusions of segmental pulmonary arterial branches in both lower lobes and in the right upper lobe (arrows), proximal arterial dilatation, and vessel tortuosity. Pulmonary thromboendarterectomy was carried out under hypothermic circulatory arrest and an extensive cast of organised clot and thrombus was removed from the pulmonary arterial tree. The patient has made an uneventful recovery and at three months after surgery is asymptomatic, walking up to three miles daily. Mean pulmonary arterial pressure has fallen to 22 mm Hg and the cardiac index has improved (2.92 l/min/m2). Repeat MRA demonstrates dramatic improvement in the pulmonary circulation with enhanced blood flow detected in subsegmental pulmonary arterial branches and in the pulmonary veins (below right).