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Pear-like tumour of the pericardium disappearing

The Clinical Case of the Month: April 2009

Myocardial Disease


Case Presentation:

A 52 years old patient was admitted to our hospital because of the recurrent large pericardial effusion, only four weeks after previous pericardiocentesis and drainage of 1600 ml of serous, initially idiopathic pericardial effusion. Five months before, he felt slowly progressing fatigue, dyspnoea on effort and productive cough (but no haemoptysis). ECG revealed atrial fibrillation with low voltage and tachycardia and echocardiography demonstrated signs of an imminent cardiac tamponade and a foreign mass on the visceral layer of the pericardium in front of the right ventricle. Computed tomography verified epicardial infiltration of benign features in front of the right ventricle. The patient was initially stable after the first pericardiocentesis. However, soon after the hospital discharge, a large pericardial effusion slowly recurred, followed with dyspnoea on effort. His medical history also includes arterial hypertension, well treated with ACE inhibitors.

Physical examination on his second admission revealed no fever, distended neck veins, and distant heart sounds. His heart rate was rapid but now regular (110 b/min, no atrial fibrillation any more) and there was no severe hypotension (BP 120/80 mmHg). In addition to the rapid heart rate, ECG revealed low voltage and electrical alternans, with a leftward axis deviation (QRS vector -26°). After repeated pericardiocentesis his heart rate returned to 70-80/min. However, a brief paroxysm of atrial fibrillation with a rapid ventricular rate of 180 b/min occurred again immediately after pericardiocentesis.
Laboratory analyses revealed increased sedimentation rate during the course of hospitalisation (46...38...80...50...100...69) with a slight sideropenic anaemia (haemoglobin 111-122, serum iron 7.3). All other routine laboratory analyses were normal including serum cholesterol 4.19, HDL 1.41, LDL 2.22 and triglycerides. Although other parameters of the liver function were normal, gamma GT was significantly increased (132) probably due to the previous alcohol abuse.

Chest X-ray demonstrated small pleural effusion at the left side and an enlarged "water-bottle" heart shadow. Echocardiography revealed a very large, Horowitz type D pericardial effusion with the diastolic separation of pericardial layers of 24-30 mm in front of the right ventricle and 33-36 mm behind the left ventricle. Interestingly, there was a 15-20 mm thick, pear-like formation at the visceral pericardium in front of the right ventricle. Despite the huge size of the effusion, there were no signs of cardiac tamponade. Remaining echocardiography findings were normal, with the normal size and contractility of all cardiac structures.
 
Abdominal ultrasonography has shown normal size and shape of the liver with no focal lesions, but diffusely non-homogenous structure. Portal vein was normal, with no thrombosis. Cholecyst was of normal size and position, but bended, with increased wall thickness and with an infundibular content of high density but no calculosis. Choledochus was of regular luminal size, with no calculosis. Pancreas was normal, as well as the spleen and the kidneys. No ascites could be detected.
As a part of the initial management, pericardiocentesis and drainage of 1700 ml of hemorrhagic pericardial effusion was performed using fluoroscopic control in the cardiac catheterization laboratory, a flexible 7F catheter and the subxiphoid approach. Pericardial fluid analyses revealed the following findings: glucose 2.6, total proteins 44, albumins 27, alpha amylase 37, LDH 745 U/l, pH 8. All cultures for aerobic and anaerobic bacteria remained sterile. Direct microscopy and cultures for tuberculosis were negative as well. Cytological examination of the pericardial effusion has demonstrated coagulated proteins, scarce lymphocytes, neutrophil granulocytes, and rare solitary cells with week expression of anisomorphism.
Cardiac catheterization was performed in the second session, in order to exclude presence of any pathological vascularisation of the tumorous formation on the visceral layer of the pericardium in front of the right ventricle. However, coronary arteries were normal, as well as the left ventricle with the LVEF of 65%. Right-heart catheterization revealed normal pressures in the pulmonary circulation and the right-heart chambers.
In order to additionally visualize the tumour formation, intracardiac echocardiography was performed (Accu-Nav, Siemens, Germany). Scanning from the right atrium, it was possible to visualize pear-like tumour on the visceral pericardium in front of the right ventricle, 3x5 cm, arising from the epicardium on the wide basis, with distinct borders and no signs of infiltration of the myocardium. Small, thick, residual pericardial effusion was also detectable, despite the previous drainage of the effusion and the presence of a functional pericardial catheter.

In an attempt to clarify the etiological diagnosis, after 12 days of prolonged drainage of the pericardial effusion with a daily drainage of 200-300 ml of sero-haemorrhagic effusion, the patient underwent pericardioscopy and pericardial biopsy: (16.5 F Olympus HYF-1T flexible endoscope)(Figure 1). Endoscopic evaluation revealed signs of haemorrhagic inflammation on both pericardial layers. As expected according to the echocardiography and computed tomography findings, a large, solitary tumour formation, with macroscopic features of a lipoma, was visualized on the epicardial layer in front of the right ventricle. Six biopsy samples were taken from the borderline area of the tumour and surrounding epicardium and pericardium. The procedure was completed without any complications. Pathohistological examination has revealed lymphocyte infiltration and specific changes depicted in the (Figure 1).


Figure 1.

Figure 1. Pericardioscopy and pericardial biopsy in a patient with recurring cardiac tamponade and extensive prolonged production of sero-haemorrhagic pericardial effusion of unclear aetiology.

1) What would be your diagnosis in this phase and
2) What would be your recommendation for the further management

Answers:

Due to the continuous production of large amounts of sero-haemorrhagic fluid the patient was referred to pericardiectomy. Subtotal pericardiectomy was performed, but surprisingly 5x3 cm large tumour, previously confirmed by echocardiography, computed tomography, intracardiac echocardiography, and pericardioscopy was not present at the time of surgery any longer. Perhaps, a lipomatous content of the tumour was drained after the biopsy. However, several similar tumours (Figure 2) were detected in the left pleura and removed during the same surgical procedure. In addition, 300 ml of thick chylous effusion was evacuated from the left pleura. The final diagnosis established from the biopsy samples taken by pericardioscopy and the samples taken during the surgery was cholesterol pericarditis with the chronic inflammation (foreign body granulomatous inflammation around the cholesterol crystals) as well as the benign pleural lipoma.
Pear-like infiltration of the visceral pericardium in front of the right ventricle was most probably also a lipoma which collapsed after taking several biopsy samples. After the surgery, there was no relapse of either pericardial or pleural effusion and the patient was discharged from the hospital on antihypertensive medication and statins. During the 24 months follow-up the patients was stable, with no symptoms, no pericardial or pleural effusion and no recurrences of lipomatous tumours. He was even able to return to his previous profession – acting in the classical theatre.

Figure 2Figure 3

Figure 2. Intraoperative findings demonstrating extensive inflammatory changes on the visceral and parietal pericardium, thickened parietal pericardium and infiltration in the left pleural space (white arrow). Surprisingly, no tumorous infiltration of the visceral pericardium could be visualized in front of the right ventricle any more. Right-sided image reveals histological examination from the pleural tumour – pleural lipoma.

Cholesterol pericarditis

Cholesterol pericarditis is a rare complication of chronic pericardial effusion or chronic scarring of the pericardium [1, 2] and is exacerbated by cholesterol crystals. Common underlying causes include tuberculous pericarditis, autoimmune rheumatic diseases, and pericardial trauma [2, 3]. When a pericardial effusion is relatively acute, its cholesterol content remains in solution. However, when the pericardial effusion is chronic, the normal ability to dissolve cholesterol is impaired and cholesterol crystals are deposited in the pericardium and effusion [4-6]. The fluid is clear, in contrast to chylopericardium, and classically is said to have a glittering "gold paint" appearance, as it was the case with our patient at the first pericardiocentesis [7, 8]. However, any other macroscopic appearance of pericardial effusion apart from the “gold paint” is not excluding the diagnosis.

The effusions tend to be large. The concentration of cholesterol equals or exceeds that of the blood, often attaining values above 500 mg/dL (13 mmol/L) [8]. Unfortunately, estimation of cholesterol level was not a part of our routine evaluation of the pericardial effusion, which turned out to be wrong in this specific case. The pericardial effusion associated with myxedema also has a high cholesterol concentration, but crystals are usually absent.
Blood associated with inflammation is thought to be the source of cholesterol in the pericardial fluid, and evidence of current or previous haemorrhage is usually evident. The pericardium is thicker than normal (Figure 2 left) and its inner surface is lined with plaques and cholesterol deposits. The histological findings include fibrosis, inflammatory cells, cholesterol clefts and crystals of variable geometry, and giant cell granulomata.
Treatment includes pericardiocentesis, which is seldom effective over the long-term because the effusions tend to recur and can cause tamponade at any time. This procedure also fails to address the thick, scarred pericardium and does not prevent the late development of constrictive pericarditis. Thus, optimal therapy is radical pericardiectomy with additional treatment of the underlying cause of chronic pericarditis [9].

 


References


1. Maisch B, Ristić AD. The classification of pericardial disease in the age of modern medicine. Curr Cardiol Rep 2002; 4(1): 13-21.
2. Maisch B, Soler-Soler J, Hatle L, Ristić AD. Pericardial diseases. In: Serruys PW, Camm AJ, Lüscher TF, eds. The ESC Textbook of Cardiovascular Medicine. Blackwell Publishing Ltd., London 2006, 517-34.
3. Pankuweit S, Ristić AD, Seferović PM, Maisch B. Bacterial pericarditis: diagnosis and management. Am J Cardiovasc Drugs 2005:5(2):103-12.
4. Raposo L, Andrade MJ, Ferreira J, Aguiar C, Abecasis M, Ramos S, Reis AB, Martins AP, Pais MJ, Gomes RS. Cholesterol pericarditis--relapsing pericardial effusion in a patient with rheumatoid arthritis. Rev Port Cardiol. 2005 May;24(5):737-45.
5. Ford EJ, Bear PA, Adams RW. Cholesterol pericarditis causing cardiac tamponade. Am Heart J. 1991 Sep;122(3 Pt 1):877-9.
6. Noiri E, Itoh T. Disorder of pericardial fluid cholesterol metabolism in cholesterol pericarditis. Jpn J Med. 1991 Jul-Aug;30(4):393.
7. Spodick, DH. The Pericardium. A comprehensive textbook, Marcel Dekker, New York 1997. p.84.
8. Brawley, RK, Vasko, JS, Morrow, AG. Cholesterol pericarditis. Considerations of its pathogenesis and treatment. Am J Med 1966; 41:235.
9. Maisch B, Seferović PM, Ristić AD, Erbel R, Rienmüller R, Adler Y, Tomkowski W, Thiene G, Yacoub MH. European Society of Cardiology Guidelines: Diagnosis and management of the pericardial diseases. Executive summary. Eur Heart J 2004; 25(7):587-610.

Notes to editor


Dr Arsen RisticBy Arsen Ristić, Petar Đukić, Vesna Božić, Bosiljka Vujisić-Tešić, Siniša Stojković, Petar M. Seferović.
Department of Cardiology, Department of Cardiac Surgery and Department of Pathology, Institute of Cardiovascular Diseases, Clinical Centre of Serbia and Belgrade University School of Medicine, Belgrade, Serbia
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.

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