Ann Thorac Surg 2004;78:e96-e97
© 2004 The Society of Thoracic Surgeons
Case report
Cardiac Varix in Relation to Right Atrial Free Wall Presenting as a Mass Compressing the Right Atrium and Mimicking a Pericardial Cyst
Abraham Oomman, MD, DMa,*,
Panchapakesan Ramachandran, MD, DMa,
Rajan Santhosham, MCha,
Louis Felix Sridhar, DNBa,
Binu Ramesh, MD, DMa,
Samy Jayaraman, DTCDa
a Apollo Hospital, Chennai, Tamil Nadu, India
Accepted for publication December 22, 2003.
* Address reprint requests to Dr Oomman, Apollo Hospital, Gream's Rd, Chennai, Tamil Nadu, India, PIN-600006
dr-oomman{at}lycos.com
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Abstract
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Cardiac varix is a rare entity. It is generally small and is in relation to the interatrial septum, often mistaken as cardiac myxoma. A 19-year-old girl, on evaluation of respiratory infection, was found to have a mass compressing the right atrium. Computed tomographic scan and magnetic resonance imaging suggested a differential diagnosis of pericardial cyst or hydatid cyst. Peroperatively, a large (8 x 6.5 x 5.5 cm) cystic lesion in relation to the right atrial free wall was found. The histopathology of the resected mass revealed it to be a cardiac varix. The case is notable for its large size and its location in relation to the right atrial free wall.
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Introduction
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Cardiac varices are endocardial, unilocular blood filled cysts lined by endothelial cells and filled with organizing thrombi. These are dilated thrombosed veins and are usually found in the right atrium. They are rare entities and are usually mistaken for myxomas. We report a case where the cardiac varix was large and in relation to the free wall of the right atrium.
AJ, a 19-year-old female college student with a history of episodic cough and wheeze, was evaluated by the primary care physician for respiratory tract infection. Roentgenogram chest showed cardiomegaly and the electrocardiogram was within normal limits. Echocardiogram was suggestive of an extra cardiac mass compressing the right atrium (RA) and, partly, the right ventricle. Computed tomography (CT) of the chest showed a nonenhancing cystic lesion adjacent to the right cardiac border, suggestive of a pericardial cyst (Fig 1). She was referred to our center for further management.
Clinical examination was within normal limits. Baseline biochemical and hematological examinations were also within normal limits. Magnetic resonance imaging (MRI) scan of the chest showed a well-defined intracardiac space occupying lesion with wall calcification involving the pericardium in the right lateral aspect of the cardia of mixed signal intensity. The possibilities considered were hemorrhagic pericardial cyst or hydatid cyst.
The patient was taken for surgery. An attempt was made to remove what was thought to be a pericardial cyst through a right thoracotomy. During surgery, it became apparent that this mass was attached to the RA free wall. The mass was large and obscuring the ascending aorta, making cannulation difficult. Hence, a decision was made to approach through median sternotomy. Median sternotomy and pericardiotomy were done. Bicaval ascending aortic cardiopulmonary bypass was established. Under mild to moderate hypothermia, the tumor was enucleated en masse. It was found to be attached to the RA free wall, was large, and had a well-developed capsule. The mass was not attached to the atrioventricular groove or the right coronary artery. The patient was weaned off bypass in sinus rhythm with no inotropic support. The patient had an uneventful postoperative course and was discharged on the seventh postoperative day.
The specimen was sent for histopathological examination. Macroscopically, the specimen was a cyst measuring 8 x 6.5 x 5.5 cm (Fig 2). The cut surface revealed a cyst containing brownish necrotic material. Microscopically, the section showed atrial wall with compressed muscle containing a thick walled cyst. The wall showed collagenization and some vascular proliferation with perivascular lymphoplasmocytic infiltrates amid many pigmented macrophages. Within the cyst, there was much hemorrhage with fibrin deposits. Cyst in foci showed endothelial lining. The final histopathological impression was that the lesion was consistent with a right atrial varix (venous malformation).
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Comment
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Right atrial varicies are rare entities. They were described for the first time by anatopathologists at the end of the nineteenth century and the beginning of the twentieth century [1]. Descriptions have been from postmortem studies, which have led to epidemiological analyses and have given rise to nosological controversies [1]. They are situated in the lower part of the interatrial septum and rarely exceed 2 cm in diameter. Transesophageal echocardiography, MRI, and CT scan have increased the chances of clinical differentiation of this entity which most often is a chance finding and commonly misdiagnosed as a myxoma. Harrity and colleagues [2] reported a case of two broad based masses along the right atrial septum in a 31-year-old human which were thought to represent partially calcified myxomas. However, at operation they were cystic structures filled with blood and calcific nodules. Histopathologically they were varices with phleboliths. The authors commented that angiography and ultrafast CT may help in the differentiation of this entity from myxomas and other intracavitary masses [2]. Remadi and colleagues [3] reported a case where an asymptomatic primary tumor in the right atrium was detected in a 76-year-old human during preoperative evaluation for prostate surgery. The histopathology of the resected mass turned out to be a varix of the heart. Thorsen and colleagues [4] have reported mobile intracardiac varix as a new echocardiographic entity.
Primary cardiac tumors are rare. Their incidence ranges from 0.001% to 0.28% according to a report of an autopsy series [5]. In 75% of cases they are benign, and the most frequent are myxomas, lipomas, and rhabdomyomas. The remaining 25% are malignant, and the most common are rhabdomyosarcomas and angiosarcomas. Cardiac tumors may involve the endocardium, the myocardium, the pericardium, and the emergence of great vessels.
The differential diagnosis, or rather the preoperative diagnosis in varix, is most often myxoma. The reported cases were intracardiac and in relation to the interatrial septum resulting in the diagnosis of myxoma. Our case was different. Echocardiogram, CT scan, and MRI were suggestive of a well-defined space occupying lesion adjacent to the right cardiac border, suggesting the possibilities of a pericardial cyst or a hydatid cyst. Other rare possibilities include the following: lipoma of the right atrium [6]; inflammatory pseudotumor of the RA [7]; intrapericardial pheochromocytoma [8]; a closed interatrial aneurysm filled with blood mimicking a tumor [9]; a right coronary artery aneurysm simulating a right intraatrial mass [10]; an intracardiac tuberculoma [11]; and a Eustachian valve mimicking a right atrial cystic tumor [12].
Although pericardial cysts are rare, they are the most common benign pericardial tumor. They are thought to represent the failure of fusion of one of the mesenchymal lacunae that normally come to lie in the region of the anterior cardiophrenic angle. Its removal is recommended if the diagnosis is in doubt, symptoms are present, or complications arise [13]. The most common presenting symptoms are vague chest pain and dyspnea. The complications include cardiac compression, cyst infection with or without cardiac erosion, and cyst rupture [13] or hemorrhage [14]. A case of extrinsic compression by a dissecting pericardial hematoma presenting as obstructive intracardiac mass has been reported [15]. Pericardial hydatid cyst is rare. Sakarya and colleagues [16] reported a case where MRI showed a pericardial cyst with low signal intensity noted on T1 weighted images. The signal intensities of folded parasitic membranes in the cyst were of lower intensity on T2 weighted images. Based on these images a pericardial hydatid cyst was diagnosed, which was surgically removed and confirmed histopathologically.
Our case had many interesting features. Cardiac varix is a rare entity. The reported cases show the varix to be in relation to the interatrial septum and rarely exceeds 2 cm in size [1]. In our patient it was 8 x 6.5 x 5.5 cm in size and was in relation to the right atrial free wall and presented as a mass compressing the right atrium, which was mistakenly thought to be a pericardial tumor. A Medline search did not reveal any case of cardiac varix of such a large size or at this location. We have reported a case of cardiac varix attached to the right atrial free wall presenting as a mass compressing the right atrium.
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References
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Abstract
Introduction
