Occult malignancy presenting as constrictive pericarditis

Interactive CardioVascular and Thoracic Surgery, Jun 2011

Metastatic tumour progression to the pericardium is generally characterised by an effusional pericarditis. It is extremely rare for tumour to metastasise to the pericardium and cause constrictive pericarditis in the absence of a pericardial effusion. We report the recent case of a patient who was referred to our centre with constrictive pericarditis. Following pericardectomy and histopathological analysis this was found to be secondary to an occult metastatic adenocarcinoma.

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Occult malignancy presenting as constrictive pericarditis

Darren Porter 0 Mehmood Jadoon 0 Damian McGrogan 0 Onyekwelu Nzewi 0 0 Department of Cardiothoracic Surgery, Royal Victoria Hospital , Grosvenor Road, Belfast BT12 6BA, Northern Ireland, UK Metastatic tumour progression to the pericardium is generally characterised by an effusional pericarditis. It is extremely rare for tumour to metastasise to the pericardium and cause constrictive pericarditis in the absence of a pericardial effusion. We report the recent case of a patient who was referred to our centre with constrictive pericarditis. Following pericardectomy and histopathological analysis this was found to be secondary to an occult metastatic adenocarcinoma. 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. 1. Introduction Constrictive pericarditis is most often idiopathic or it occurs following cardiothoracic surgery or radiation therapy to the mediastinum, it is rarely caused by malignancy. When metastatic cancer involves the pericardium, it typically causes pericardial effusion (with or without tamponade). Few case reports of metastatic lung cancer causing constrictive pericarditis have been reported and fewer still describe constrictive pericarditis as the first manifestation of the neoplastic process. We report the case of a 33-yearold patient with an occult malignancy presenting as constrictive pericarditis. 2. Case report A 33-year-old patient presented with a six months history of dyspnoea, general malaise, weight loss, ascites and ankle oedema. Chest X-ray demonstrated bilateral pleural effusions. Autoimmune and vasculitic screens were normal and tumour markers were negative. Computed tomography pulmonary angiogram (CTPA) revealed bilateral pleural effusions, pericardial thickening and a small sub-segmental pulmonary embolism with no obvious lung pathology. A pleural tap was performed and this was negative for TB, infection or malignancy. Transthoracic echocardiography demonstrated a thickened pericardium, a dilated superior vena cava (SVC) and inferior vena cava (IVC) which raised the possibility of constrictive pericarditis. A cardiac magnetic resonance imaging (MRI) confirmed this diagnosis with classical septal bouncing with respiratory motion and markedly thickened pericardium (Fig. 1). Radical pericardectomy was carried out through a conventional median sternotomy incision. An unusual finding was uniformly marked thickening that completely encased the SVC, the IVC and the pulmonary trunk, and even beyond the phrenic nerves. The phrenic nerves were dissected out carefully and the entire posterior thickened pericardium was removed to free the heart completely (Fig. 2). Following removal of the pericardium there was an immediate improvement in blood pressure and a drop in central venous pressure (CVP) from 29 mmHg to 19 mmHg. Histopathology of pericardium indicated a differential diagnosis of metastatic adenocarcinoma or mesothelioma; however, malignant mesothelioma was excluded using immunohistochemistry and metastatic lung adenocarcinoma was confirmed. Anterior hilar nodes and thymic tissue were also extensively replaced with metastatic adenocarcinoma. With a working diagnosis of occult metastatic lung adenocarcinoma, the patient underwent a CT chest, abdomen and pelvis on day 6 postoperatively which revealed no gross lesion. Despite the marked improvement and resolution of the symptoms of constrictive pericarditis the patient unfortunately died 29 days postoperatively from complications secondary to repeated pulmonary emboli. 3. Discussion Constrictive pericarditis is most commonly idiopathic or it occurs secondary to mediastinal radiotherapy or following cardiac surgery. Less common aetiologies include infection, connective tissue disorders, malignancy, uraemia, and sarcoidosis w1x, tuberculosis is a rare cause of constrictive pericarditis in the Western world w2x. Constrictive pericarditis can rarely be caused by malignancy, however, malignancy may also manifest as pericardial effusion (with or without tamponade) or an encased heart with thickening of both visceral and parietal layers, resulting in a constrictive physiology. D. Porter et al. / Interactive CardioVascular and Thoracic Surgery 12 (2011) 10461047 Fig. 1. Cardiac magnetic resonance imaging: Pericardial thickening (arrow). Fig. 2. Thickened pericardium following pericardectomy. Metastatic involvement of the heart is much more common than primary tumours. The prevalence of such metastatic involvement has been reported as ranging from 15% to 30% in autopsies performed for cases of neoplastic disease and 4% of general autopsies w3x. Lung and breast cancers are the most frequent causes of malignant pericardial disease; however, lymphoma and mesothelioma can also involve the pericardium w4x. The most common cell type to metastasise to the heart is adenocarcinoma w5x. Cardiac involvement can develop by retrograde lymphatic, haematogenous, direct or transverse extension. The cardiac (...truncated)


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Darren Porter, Mehmood Jadoon, Damian McGrogan, Onyekwelu Nzewi. Occult malignancy presenting as constrictive pericarditis, Interactive CardioVascular and Thoracic Surgery, 2011, pp. 1046-1047, 12/6, DOI: 10.1510/icvts.2011.266494