Source: Annual Congress 2008 - Mediastinal lesions; rare pathology
Disease area: Pulmonary vascular diseases

Abstract

Background. A myxoma is a rare tumour which is usually located in the heart, left atrium being the most common localization. CTEPH is an under-recognized disease resulting from chronic obstruction of pulmonary artery by organized thromboemboli leading to gradual increase of pulmonary vascular resistance, progressive pulmonary hypertension and eventually right heart failure. Despite the ongoing debate concerning natural history and pathogenesis there is a curative operation for this disease – pulmonary thromboendarterectomy (PTE).
Methods. We present a case report of a 47-yo woman who was admitted to our center complaining of dyspnoea on exertion, dry cough, fatigue and weakness. The symptoms first appeared three month prior to hospitalisation. Chest CT, ECHO, pulmonary angiography revealed what appeared to be a thrombus in the inferior vena cava, lung parenchimal changes typical for recurrent pulmonary embolism, pulmonary hypertension of 80/20 mm Hg and RV ejection fraction of 19% due to pulmonary artery obstruction by thrombotic masses.
Results. The operation was performed in deep hypothermia with 3 periods of circulatory arrest (12, 16, 2 mins) for removal of the inferior vena cava tumour, left PTE, and right pulmonary artery revision which revelaed no central changes. The postoperative period was uneventful. The control ECHO showed considerable decrease of pulmonary artery pressure to 27 mm Hg, RH ejection fraction=39%. The patient was discharged on 18-th postoperative day.
Conclusion. Caval myxoma can be the precipitating factor for CTEPH. The surgery is the only curative option for this potentially fatal combination.

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Citations should be made in the following way:
P. Yablonsky, V. Milyokhin, V. Golovin, E. Shloydo, E. Pavlushkov (Saint-Petersburg, Russian Federation). A rare combination of a myxoma of the inferior vena cava and chronic thromboembolic pulmonary hypertension (CTEPH). Eur Respir J 2008; 32: Suppl. 52, 2156

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