ARTICLE IN PRESS doi:10.1510/icvts.2011.266494
Interactive CardioVascular and Thoracic Surgery 12 (2011) 1046–1047 www.icvts.org
Case report - Cardiac general
Occult malignancy presenting as constrictive pericarditis Darren Porter*, Mehmood Jadoon, Damian McGrogan, Onyekwelu Nzewi Department of Cardiothoracic Surgery, Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA, Northern Ireland, UK Received 19 January 2011; accepted 8 February 2011
Abstract 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. 䊚 2011 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. Keywords: Cardiac; Constrictive pericarditis; Metastatic lung adenocarcinoma
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). *Corresponding author. 49 Diamond Gardens, Finaghy, Belfast BT10 0HE, Northern Ireland, UK. Tel.: q44-75-1002439; fax: q44-28-602678. E-mail addresses:
[email protected],
[email protected] (D. Porter). 䊚 2011 Published by European Association for Cardio-Thoracic Surgery
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.
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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 pericar-
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Fig. 2. Thickened pericardium following pericardectomy.
w1x Cameron J, Oesterle SN, Baldwin JC, Hancock WW. The etiologic spectrum of constrictive pericarditis. Am Heart J 1987;113:354–360. w2x Bertog SC, Thambidorai SK, Parakh K, Schoenhagen P, Ozduran V, Houghtaling PL, Lytle BW, Blackstone EH, Lauer MS, Klein AL. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J Am Coll Cardiol 2004;43:1445–1452. w3x Abraham KP, Reddy V, Gattuso P. Neoplasm metastatic to the heart: review of 3314 consecutive autopsies. Am J Cardiovasc Pathol 1990; 3:195–198. w4x Llewellyn MJ, Atkinson MW, Fabri B. Pericardial constriction caused by primary mesothelioma. Br Heart J 1987;57;54–57. w5x Klatt EC, Heitz DR. Cardiac metastases. Cancer 1990;65:1456–1459. w6x Chiles C, Woodard P, Gutierrez F, Link K. Metastatic involvement of the heart and pericardium: CT and MR imaging. Radiographics 2001;21:439– 449. w7x Wakamatsu T, Koizumi T, Urushihata K, Fujimoto K, Uchikawa S, Kubo K, Iwamura A, Yazawa M. Direct pericardial involvement of non-small cell lung cancer rapidly developing pericardial constriction. Jpn J Clin Oncol 2004;34:627–629. w8x Caballero LM, Asensio JMN, Gonzalez RA, Romero JJG, Fernandez RG, Fernandez AMP. Constrictive pericarditis as the first sign of lung cancer. Arch Bronconeumol 2006;42:608–610. w9x Balghith M, Taylor DA, Jugdutt BI. Cardiac tamponade as the first clinical manifestation of metastatic adenocarcinoma of the lung. Can J Cardiol 2000;16:925–927. w10x Tsolakis EJ, Charitos CE, Mitsibounas D, Nanas JN. Cardiac tamponade rapidly evolving toward constrictive pericarditis and shock as a first manifestation of non-cardiac cancer. J Card Surg 2004;19:134–135.
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dial 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 location of the tumour depends on the path of dissemination. Lymphatic spread is usually to the pericardium and this is the most common metastatic pathway. In contrast, haematogenous spread usually produces myocardial metastasis and, much less commonly, endocardial involvement w6 x . Few case reports of metastatic lung cancer causing constrictive pericarditis have been reported w7x and no case reports could be found in the literature that describe constrictive pericarditis in the absence of pericardial effusion as the first manifestation of the neoplastic process w8x. Metastatic tumour progression to the pericardium is generally characterised by pericardial effusion, which can result in cardiac tamponade w9x. Pericardial constriction by diffuse pericardial thickening of metastatic origin is a rare complication, and it would be very unusual for such constriction to be the first manifestation of the neoplastic process w10x. In the literature only one case has described constrictive pericarditis secondary to metastatic lung cancer; however, this case described an effusive constrictive pericarditis which was diagnosed at post mortem w8x. We believe that the case described in our centre is the only reported case to describe constrictive pericarditis in the absence of a pericardial effusion, a diagnosis that was made ante mortem and which unfortunately was the primary presentation of metastatic adenocarcinoma of the lung.
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Fig. 1. Cardiac magnetic resonance imaging: Pericardial thickening (arrow).
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