Asian Cardiovasc Thorac Ann 2000;8:381-383
© 2000 Asia Publishing EXchange Pte Ltd
Diffuse Cardiac Lipomatosis Presenting as Recurrent Pericardial Effusion
Ashok K Srivastava, MCh,
Anil K Agarwal, MS,
Aditya Kapoor, DM,1,
Prabhat Tiwari, MD,2,
Rakesh Pandey, MD,3
Department of Cardiovascular & Thoracic Surgery
1 Department of Cardiology
2 Department of Anaesthesia
3 Department of Pathology
Sanjay Gandhi Post-Graduate Institute of Medical Sciences
Lucknow, India
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For reprint information contact: Ashok K Srivastava, MCh Tel: 91 522 44 0963 Fax: 91 522 44 0017 email: ashok{at}sgpgi.ac.in. Department of Cardiovascular & Thoracic Surgery, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Raebareli Road, Lucknow 226014, India.
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Abstract
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A 48-year-old man undergoing open pericardiotomy for recurrent pericardial effusion was found to have extensive diffuse cardiac lipomatosis. The mode of presentation, preoperative evaluation, and surgical management of this rare condition are described.
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Introduction
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Diffuse lipomatosis usually involves an extensive portion of the trunk and extremities, and this condition may clinically simulate liposarcoma.1 Microscopically, the tissue resembles that of an ordinary lipoma. There have been occasional reports of this tumor involving the interatrial septum but diffuse infiltration of the myo-cardium and epicardium by mature adipocytes has not been described.
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Case Report
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A 48-year-old man had dyspnea on exertion, easy fatigability, and cough with expectoration for one year. There was no history of weight loss or fever. He had undergone surgery for a duodenal lipoma 20 years previously. Before presenting to our institution, he had undergone pericardiocentesis 4 times within 4 months. The pericardial fluid was noted to be a nonspecific chronic inflammatory exudate. Because tuberculosis is a common cause of pericarditis in developing countries, antitubercular treatment had been prescribed. On referral to our institution, the patient was in New York Heart Association functional class III, his jugular venous pressure was elevated to 10 cm above the sternal angle, and there was a pulsus paradoxus. Chest radiography showed a cardio-thoracic ratio of 80%. Echocardiography revealed a large echolucent space encasing the entire heart with areas of varying echo texture that had an echodensity similar to myocardium and mimicked pericardial effusion. Although magnetic resonance imaging (MRI) was available, it was not usually employed for effusion and constriction. The patient was therefore referred for pericardiotomy.
Through a left anterolateral thoracotomy under general anesthesia, the pericardium was found to be filled with a massive amount of fatty tissue and clear fluid. The thoracotomy incision was extended towards the contra-lateral side after bisecting the sternum for better exposure. While inspecting the origin of the lipomatous tissue, a major part of the lipoma was accidentally avulsed from the right ventricular outflow tract (RVOT). Emergency cardiopulmonary bypass (CPB) was instituted and the aorta was crossclamped. On further exploration, it was found that the fatty tissue was attached to the RVOT and was densely intermingled with the entire myocardium, the aorta, and the pulmonary arteries. The RVOT defect was closed using a Dacron patch covered with autologous pericardium to prevent bleeding. In the absence of tough tissue to hold the stitches, blood continued to leak from the suture lines despite adequate reversal of heparin. The bleeding could not be controlled and the patient died on the first postoperative day.
The excised mass had a smooth lobulated surface, it measured 22 x 15 x 10 cm, and weighed 3.5 kg. Histopathology was carried out on tissue samples from the superior vena cava, inferior vena cava, right atrial junction, left and right ventricles, the aorta, and the pulmonary artery. These revealed a marked increase in fat content of the epicardium and transmural infiltration of the myocardium by mature adipocytes. The myocardial fibers were splayed by intervening adipocytes. The adipocytes showed degenerative cytoplasmic changes and bizarre nuclei (Figures 1 and 2
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Figure 1. Section from the superior vena caval-right atrial junction, showing the endocardium and part of the myocardium extensively infiltrated by mature adipocytes (hematoxylin and eosin stain, original magnification x100).
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Figure 2. Section from the right ventricular wall showing splaying of myocardial fibers and infiltration of mature adipocytes between myocardial fibers (hematoxylin and eosin stain, original magnification x200).
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Discussion
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Cardiac lipomas are rare and account for approximately 4% to 14% of all benign cardiac tumors.2,3 They may be localized intracavitary or subepicardially. Although generally asymptomatic, presentation usually depends on the size and location of the tumor. Intracavitary lipomas usually produce early symptoms such as congestive heart failure, supraventricular or ventricular arrhythmias, syncope, or sudden cardiac death.4 Subepicardial lipomas usually grow slowly and often achieve enormous size before producing any symptoms. They are frequently detected incidentally at autopsy, although anginal pain due to coronary arterial compression, atrial arrhythmias, and effort intolerance have been reported.5 Our patient presented with the unusual symptom of recurrent peri-cardial effusion.
Chest radiography usually does not differentiate between pericardial effusion and subepicardial lipoma. Echo-cardiography accurately defines the location and extent of the mass. However, the acoustic appearance of an intrapericardial lipoma is not diagnostic. Intracardiac lipomas are generally echodense, while subepicardial lipomas usually appear relatively echolucent and may be erroneously interpreted as pericardial fluid, as in this case.5,6 Computed tomography and MRI can demonstrate lipomatous tumors. Computed tomography can differen-tiate lipomatous tumors that have low radiodensity (52 to 150 Hounsfield units) from other tumors that have higher radiodensity, such as myxomas. MRI can distinguish between lipomas and other tumors with fatty contents, such as liposarcomas. MRI provides direct multiplanar images with clear definition of blood vessels so that location, size, and extension of cardiac tumors to the great vessels, pericardium, and lungs can be precisely determined. These are important factors in determining tumor resectability and planning the approach.
Since the first successful removal of an intrapericardial lipoma by Maurer7 in 1952, several sporadic cases have been reported. Eng and colleagues8 reported resection of an epicardial lipoma weighing 1.36 kg, Doshi and colleagues5 resected one weighing 2.15 kg. Benign lipomas can be excised completely with low morbidity and mortality and excellent long-term results. In the majority of cases, lipomas may be removed without the need for CPB. However, CPB may be required for recurrence of a lipoma with intracardiac extension. Our case seems to be exceptional as there was massive infiltration of the entire myocardium with lipomatous tissue. Benign systemic lipomatosis, also called Madelung's disease or Launois-Bensaude syndrome, is characterized by a slow massive accumulation of mature fatty tissue in the cervical and supraclavicular region, causing swelling above the neck and at times causing severe distress.1 In this case, extensive diffuse lipomatosis made the myocardium extremely friable and even with the aid of CPB, the defect in the RVOT could not be satisfactorily closed and the patient continued to bleed. We postulate that cardiac transplantation might play a role in the treatment of cardiac lipomatosis in the future.
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References
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Hugo NE, Conway H. Benign symmetrical lipomatosis. Plast Recons Surg 1966;37:69–71.
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Lam KY, Dickens P, Chan AC. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies. Arch Pathol Lab Med 1993;117:1027–31.[Medline]
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McAllister HA, Enoglio JJ. Tumors of the cardiovascular system. Atlas of tumor pathology. 2nd series, fascicle 15. Washington: Armed Forces Institute of Pathology, 1978: 44–6.
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Reynen K, Rein J, Wittekind C, von der Emde J . Surgical removal of a lipoma of the heart. Int J Cardiol 1993;40: 67–8.[Medline]
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Doshi S, Halim M, Singh H, Patel R. Massive intra-pericardial lipoma, a rare cause of breathlessness. Investigation and management. Int J Cardiol 1998;66: 211–5.[Medline]
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Shumacker HB Jr, Leshnower AC. Extracavitary lipoma of the heart. Operative resection. Ann Thorac Surg 1974; 18:411–4.[Medline]
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Maurer ER. Successful removal of tumor of the heart. J Thorac Surg 1952;23:479.
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Eng J, Ruiz K, Kay PH. Giant epicardial lipoma. Int J Cardiol 1992;37:115–7.[Medline]
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Abstract
Introduction
