Ann Thorac Surg 2009;87:638-640. doi:10.1016/j.athoracsur.2008.06.032
© 2009 The Society of Thoracic Surgeons
Case Reports
Blood Cyst of the Right Ventricle Presenting as Recurrent High Fever and Chills in an Adult
Mert Dumantepe, MD*,
Koray Ak, MD,
Ufuk Mungan, MD,
Ibrahim Alp, MD,
Bilal Kaan Inan, MD,
Ahmet Turan Yilmaz, MD
Department of Cardiovascular Surgery, GATA Haydarpasa Education Hospital, Kadiköy, Istanbul, Turkey
Accepted for publication June 4, 2008.
* Address correspondence to Dr Dumantepe, At
f bey sokak, Derya 85 sitesi 2. Ksm, A blok, Kat: 3, D: 11, Acbadem, Istanbul, 34660, Turkey (Email: mdumantepe{at}gmail.com).
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Abstract
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Blood cysts are uncommon primary cardiac tumors, frequently encountered in pediatric patients, but extremely rare when found in adults. Due to the considerable risks of embolization and obstruction of the blood flow, surgical removal is preferred. Herein, we report a case of blood cyst of the right ventricle, presenting as recurrent fever and chills in an adult patient.
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Introduction
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Cardiac blood cysts, first defined by Elasser in 1844, are usually detected on atrioventricular heart valves in infancy and rarely reported in adults [1]. They are usually seen on the pulmonary, tricuspid, and mitral valves, and they rarely originate from the right ventricle, left ventricle, or right atrial endocardium [1]. Although several theories regarding the origin of blood cysts as heteroplastic changes in tissues originating from the primitive pericardial mesothelium or hypoxia, inflammation and bleeding resulting in endocardial hematoma have been proposed, still, it remains a debated issue [1, 2]. The common way of clinical presentation in patients with an intracardiac blood cyst has been reported to be through several findings related to either intracardiac occlusion of blood flow or peripheral embolization [2]. In this report, we presented a patient who had been admitted with the complaints of recurrent high fever and chill attacks, and whose final evaluation revealed a blood cyst of the right ventricle. We believe that recurrent high fever and chills related to a blood cyst of the right ventricle has not been previously reported.
A 40-year-old man suffering from recurrent high fever for the past 3 months was admitted to our hospital in September 2007. Each spike of his temperature lasted for approximately 2 to 3 hours and was usually associated with chills. His past medical history is not significant. Physical examination revealed stable vital signs with normal blood pressure, cardiac, pulmonary, abdominal, and neurologic examination that were within normal limits. The electrocardiogram and the chest roentgenogram did not demonstrate any specific changes. Complete blood count revealed a hematocrit of 42% and a leukocyte count of 17,200/µL. Biochemical tests, including electrolytes, liver function tests, creatinine, and blood urea were all within normal limits. As an acute phase reactant, the erythrocyte sedimentation rate was 46 mm/h and the C-reactive protein level was 76.4 mg/L. Repeated blood cultures withdrawn during the fever spikes were all negative. Transthoracic echocardiography showed a large (30 x 12 mm), slightly mobile, cystic mass attached to the apical part of the right ventricular septum (Fig 1). The right ventricular size, function, tricuspid valve, and pulmonary valve were all normal. Transesophageal echocardiographic and cardiac magnetic resonance imaging findings were consistent with those seen on the transthoracic echocardiography (ie, a 32 x 13 mm in size, circular, strongly echogenic, mobile cystic mass extending to the right ventricular outflow tract) (Fig 2). Lung ventilation-perfusion scintigraphy revealed a perfusion defect that was consistent with a moderate risk of pulmonary embolism (PE).
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Fig 1. Transthoracic echocardiography showing a 3-cm circular slightly strong echocardiographic contrast tumor extending to the right ventricular outflow tract (RVOT). (AV = aortic valve; LA = left atrium; PA = pulmonary artery; RA = right atrium.)
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Fig 2. (A) T2 weighted sagittal plane cardiac magnetic resonance image (MRI) showing blood cystic mass. (B) Movable tumor was observed in the right ventricular outflow tract at the time of heart beating. Arrows indicate blood cyst located to the apical part of the right ventricle in T2 weighted axial plane cardiac MRI.
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Differential diagnosis of the cystic mass included a blood cyst of the right ventricle, myxoma or vegetation related to infective endocarditis, with a working diagnosis of culture-negative infective endocarditis involving the right heart. The patient underwent surgery. After a median sternotomy and aorto-bi-caval cannulation, cardiopulmonary bypass was established. The patient was cooled to 33°C. Myocardial protection was achieved by antegrade cold blood cardioplegia during aortic cross clamping. A standard right atriotomy was used to expose the right ventricular mass. A pedunculated circular mass originating from the septal surface of the right ventricular outflow tract was detected. The surface of the mass was ruptured and partially covered with thrombotic material. To prevent recurrence, surgical excision had to include a substantial portion of the normal endomyocardium. Cardiopulmonary bypass and aortic cross-clamping times were 40 and 27 minutes, respectively. The rest of the procedure was completed in a routine fashion. The early postoperative course was uneventful and the patient was discharged on postoperative day 8. Pathologic examination of the mass revealed that the cyst contained organized and nonorganized blood or serosanguineous fluid, and it was internally lined by flattened, cobblestone-shaped endothelium and an outer thin layer of fibrous tissue. Culture of the pathologic specimen of the cystic material was also negative.
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Comment
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Cardiac blood cysts are considered a congenital pathology located in the endocardium, particularly close to the free margins of valve leaflets. They are most commonly seen in neonates and generally disappear 6 months after birth and are rarely found in adults. It was reported in the literature that the origin of cardiac blood cysts was mainly from cardiac valves, particularly the atrioventricular and pulmonary valves in adult cases [3]. This result was previously supported by Boyd's pathogenetic theory in which these cysts are formed by blood pressed into crevices on the ventricular surface of the valves [4]. The cysts may uncommonly originate from left and right ventricular endocardium [3–5]. In our case, the pedunculated cystic mass was attached tightly to the septum and extended down into the right ventricular outflow tract. Histologically the cyst was lined up by flattened endothelial cells and filled with nonorganized blood.
Blood cysts reported in the literature were mainly left-sided cardiac tumors and the usual method of clinical presentation was with the symptoms related to the left ventricular inflow or outflow occlusion and valvular dysfunction [6]. Moreover, Jacob and colleagues [7] reported cerebral embolization related to a left-sided blood cyst. Right-sided tumors originating from the tricuspid valve or right ventricle endocardium may theoretically manifest themselves with findings of the right ventricle inflow or outflow occlusion or PE.
We believe that pulmonary embolism related to the right ventricular blood cyst has not been reported. In our case, the cyst was ruptured and recurrent multiple PEs were believed to be the cause of the recurrent high fever. Fever has been recognized as a common sign or feature associated with PEs. Stein and colleagues [8] reported a temperature level (> 37.5°C) in 50% of patients with acute PE. The PE-related fever is usually low-grade, rarely exceeding 38.3°C, and it is short-lived, reaching its peak the same day on which the PE occurs, and gradually disappearing within 1 week. The pathogenesis of PE-related fever has not yet been fully clarified. It has been suggested that one or a combination of a variety of potential pyrogenic mechanisms occur with the PE, such as tissue necrosis, hemorrhage, inflammation, atelectasis, or self-limited occult super-infections. The presence of a slight inflammatory response is indirectly confirmed by the concomitant increase of serum markers of inflammation as the erythrocyte sedimentation rate and C-reactive protein [9]. In our patient, increased erythrocyte sedimentation rate and C-reactive protein level were linked to the rupture and multiple microembolization of the tumor tissue. The capsule and stalk of a blood cyst are weak; therefore right-sided blood cysts have a certain potential to rupture and result in PE.
Diagnosis of a cardiac blood cyst should include transthoracic echocardiography and cardiac magnetic resonance imaging, which may help to select the surgical approach. We applied a transatrial approach for the protection of right ventricular function, and surgical exposure was excellent. Tumors located on the outflow part of the right or left ventricle might necessitate an additional pulmonary arteriotomy or aortotomy. Differential diagnosis should be made with myxoma, other cardiac malignancies, vegetation related to endocarditis, hydatid cyst, and thrombus [1, 4, 6]. There is no consensus regarding the optimal management of a cardiac blood cyst. Pasaoglu and colleagues [10] recommended immediate surgical resection, even without any symptoms, but others have proposed a regular echocardiographic follow-up in patients without symptoms owing to the benign nature of the tumor [11].
In our opinion, taking the risks of rupture and embolization into consideration, urgent surgical removal of blood cysts in both symptomatic and asymptomatic patients should be the preferred option. The present case revealed that blood cyst of the right heart may manifest itself with several systemic findings such as recurrent fever and chills related to pulmonary embolism.
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References
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- Tanaka H, Ebato M, Narisawa T, et al. Atrial blood cysts with ischemic heart disease Circ J 2003;67:1-2.[Medline]
- Sakakibara S, Katsuhara K, Iida Y, Iida Y, Nishida H. Pulmonary subvalvular tumor Dis Chest 1967;51:637-642.
- Eugene KWS, May LW, King TT, Sze KS. Blood cysts of the tricuspid valve Ann Thorac Surg 1996;61:1012-1013.[Abstract/Free Full Text]
- Boyd TAB. Blood cyst on the heart valves of infant Am J Pathol 1949;25:757.[Medline]
- Ohmoto Y, Tsuchihashi K, Tanaka S, Shimamoto K, Iimura O. Giant endocardial blood cyst in left ventricle resected by transthoracic valve approach Chest 1993;103:965-966.[Abstract/Free Full Text]
- Zhang C, Hu J, Ni Y-M, Jiang Z-N, Xu H-Y. Giant blood cyst tumor in the left ventricular outflow tract Chin Med J (Engl) 2007;120:1109-1110.
- Jacob JJ, Jose J, John B, et al. Intracardiac blood-filled cysts of the heart: a rare cause of embolic stroke Singapore Med J 2007;48:e125.[Medline]
- Stein PD, Willis III PW, DeMets DL. History and physical examination in acute pulmonary embolism in patients without preexisting cardiac or pulmonary disease Am J Cardiol 1981;47:218-223.[Medline]
- Chirinos JA, Heresi GA, Velasquez H, et al. Elevation of endothelial microparticles, platelets, and leukocyte activation in patients with venous thromboembolism J Am Coll Cardiol 2005;45:1467-1471.[Abstract/Free Full Text]
- Pasaoglu I, Dogan R, Oram A, Ozyilmaz F, Bozer AY. Giant blood cyst of the left ventricle Jpn Heart J 1991;32:147-151.[Medline]
- Eugene KWS, May LW, King TT, Sze KS. Blood cysts of the tricuspid valve Ann Thorac Surg 1996;61:1012-1013.[Abstract/Free Full Text]
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Abstract
Introduction
