© 2015, Wiley Periodicals, Inc. DOI: 10.1111/echo.12878
Echocardiography
An Unusual Mass of Tricuspid Valve in an Adult Patient: Blood-Filled Cyst € rsoy, M.D.,‡ Su € leyman Karakoyun, M.D.,§ Macit Kalcßık, M.D.,* Mahmut Yesin, M.D.,† Mustafa Ozan Gu € € ksal, M.D.,¶ Cemil _Izgi, M.D.,** and Mehmet Ozkan, Sinan Cersßit, M.D.,† Cengiz Ko M.D.†,§ *Department of Cardiology, _Iskilip Atıf Hoca State Hospital, C ß orum,Turkey; †Department of Cardiology, Kosuyolu Kartal Heart Training and Research Hospital, Istanbul, Turkey; ‡Department of Cardiology, Gaziemir State Hospital, _Izmir, Turkey; §Department of Cardiology, Faculty of Medicine, Kars Kafkas University, Kars, Turkey; ¶Department of Cardiovascular Surgery, Kosuyolu Kartal Heart Training and Research Hospital, Istanbul, Turkey; and **Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, United Kingdom
Blood-filled cysts (BFC) within the heart are common findings at postmortem examinations of fetuses and infants. However, such cysts are very rare entities in adolescents and adults. We report here an adult case of BFC attached to the posterior leaflet of the tricuspid valve, demonstrating the importance of multimodal diagnostic imaging combining both echocardiography and magnetic resonance imaging. (Echocardiography 2015;32:1199–1202) Key words: cardiac imaging, tricuspid valve, echocardiography, magnetic resonance imaging
Blood-filled cysts (BFC) are usually congenital in origin and located on the endocardium, particularly along the lines of closure of the heart valves. Their prevalence in postmortem examinations of fetuses and infants ranges from 25% to 100%.1 However, such cysts regress spontaneously in most of the patients and are rare in adults. The persistence of such cysts in the elderly may lead them to reach large diameters up to 40 mm and lead to cardiac dysfunction.2 Cyst wall consists of endothelial cells and a thin layer of fibrous tissue that contains nonorganized blood or serosanguineous fluid.3 Most of them are small and usually asymptomatic but, potential complications of larger BFCs include valve dysfunction, left ventricular outflow tract obstruction and embolic stroke. Here, we report an adult patient with a BFC attached to the posterior leaflet of the tricuspid valve which was treated with surgical excision. Case Presentation: A 65-year-old male patient who had a severe osteal left anterior descending (LAD) coronary artery stenosis (Fig. 1) was referred to our institution after a mass attached to the tricuspid valve Address for correspondence and reprint requests: Macit € u €er Sitesi Kalcik, M.D., Esentepe Mah., Milangaz Caddesi Unl B-Blok No: 22, Kartal, Istanbul, Turkey. Fax: (90)216 4596321; E-mail: [email protected]
was detected during routine transthoracic echocardiographic examination (Fig. 2A, movie clip S1). Physical examination did not reveal any pathological findings except for an elevated blood pressure. Transesophageal echocardiography (TEE) showed a cystic structure of 1.5 9 1.5 cm attached to the posterior leaflet of the tricuspid valve without relevant tricuspid regurgitation and no right ventricular inflow tract obstruction (Fig. 2B, movie clip S2). Real time three-dimensional TEE confirmed the presence of a semimobile pedunculated mass on the posterior tricuspid leaflet (Fig. 2C, movie clip S3). Subsequent cardiac magnetic resonance imaging (MRI) revealed a 15 9 18 mm possibly cystic mass attached to the posterior leaflet of the tricuspid valve which was iso-intense compared to myocardium on T1-weighted images (Fig. 3A). The cystic nature of the mass was confirmed by T2-weighted images as the central core was hyper-intense (Fig. 3B). After discussion of the findings within the heart team, operative resection of the mass was decided. Following median sternotomy and induction of cardiopulmonary bypass, the mass was reached via a left atrial and transseptal approach. When the surgeon holds the mass with forceps, fresh blood emerged from the tumor (Fig. 4A). Excision of the mass (Fig. 4B) and associated leaflet was followed by bicuspidization of tricuspid valve. The operation was accomplished after completion of the distal 1199
Kalcßık, et al.
Figure 1. Coronary angiography showed A. a severe osteal left anterior descending coronary artery stenosis with B. normal circumflex and nondominant right coronary artery. Cx = circumflex coronary artery; LAD = left anterior descending coronary artery; RCA = right coronary artery.
Figure 2. A. Two-dimensional transthoracic echocardiography modified apical four-chamber view revealed a cystic mass attached to the tricuspid valve. B. Two-dimensional transesophageal echocardiography (TEE) showed a cystic structure attached to the posterior leaflet of the tricuspid valve and C. real time three-dimensional TEE confirmed the presence of a semimobile pedunculated mass on the posterior tricuspid leaflet. LV = left ventricle; RA = right atrium; RV = right ventricle; AL = anterior leaflet of tricuspid valve; PL = posterior leaflet of tricuspid valve; SL = septal leaflet of tricuspid valve. The arrows show the blood-filled cyst on tricuspid valve.
Figure 3. Cardiac magnetic resonance imaging (MRI) revealed a possibly cystic mass attached to the posterior leaflet of the tricuspid valve which was iso-intense compared to myocardium on A. T1-weighted images and B. the central core of the mass was high signal on T2-weighted image confirming its cystic nature.
bypass anastomoses of left internal mammary artery on the LAD. Differential postoperative histological examination showed a blood-filled cystic cavity surrounded by a fibromyxoid wall with an internal lining of endothelial cells. Any malignancy, fibroelastoma or myxoma, was ruled out. The further postoperative course was
1200
uneventful, and the patient was discharged in a stable condition. Discussion: Intracardiac BFC are typically asymptomatic, usually congenital in origin, seen predominantly in infants. These cysts have been described on all
Blood-Filled Cyst on the Tricuspid Valve
Figure 4. A. Intraoperative photo revealing the blood emerging from the ruptured cystic mass while the surgeon holds it with forceps. B. Postoperative macroscopic specimen of the excised ruptured cystic mass. (The arrow shows the blood filled cyst on tricuspid valve)
cardiac valves and attached to the surfaces of all cardiac chambers.4 There are various hypotheses on the development of blood cysts, including malformations during valve development, hematoma formation in the subvalvular region, changes in remnants of primitive pericardial mesothelium and simply ectasia of blood vessels.5 Hauser et al. presented the first echocardiographic description of blood cysts attached to papillary muscles in 1983. The echocardiographic features of blood cysts consist of an echo-free space surrounded by a thin wall.6 Differential diagnosis should include myxoma, vegetation, hydatic cyst, thrombus, or malignancy. However, these entities tend to present as echo dense structures or structures with variable echogenicities. TEE is considered by most physicians as the method of choice if optimal visualization of valvular morphology is desired.7 Cardiac MRI also could be valuable to differentiate a BFC from other masses.8,9 On precontrast examinations, BFC are iso-intense compared to myocardium on T1-weighted images, and hyperintense on T2-weighted images. On the other hand, myxomas appear hypointense to myocardium on T1-weighted images and hyperintense on T2weighted images while fibromas appear hypointense on T2-weighted images and iso-intense on T1-weighted images. It is common to find regions of low signal intensity on T2-weighted images within the myxoma due to the presence calcification.9 Our case underlines the importance of an adequate preoperative diagnostic workup of such an intracardiac mass, combining echocardiography and cardiac MRI. There is no consensus or any guidelines regarding the optimal management of asymptomatic cysts. According to very few case reports (fewer than 50), it seems reasonable to adopt a conservative approach in a patient with no symp-
toms and small cysts, while surgical resection should be considered if symptoms exist or if the cysts cause any cardiac outflow obstruction or embolic strokes.10 Despite the asymptomatic state of the patient, the size and mobility of the cyst, with a relevant risk of potentially embolization, as well as the concomitant osteal LAD disease, led to the indication for a surgical approach in our patient. Therapeutic decision making should take into account the size, location and mobility of the cyst as well as relevant cardiac comorbidities and apply multimodal diagnostic imaging. References 1. Zimmerman KG, Paplanus SH, Dong S, et al: Congenital blood cysts of the heart valves. Hum Pathol 1983;14:699–703. 2. Abreu A, Galrinho A, Sa EP, et al: Hamartoma of the mitral valve with blood cysts: A rare tumor detected by echocardiography. J Am Soc Echocardiogr 1998;11:832– 836. 3. Boyd TAB: Blood cysts on the heart valves of infants. Am J Pathol 1949;25:757–759. 4. Dencker M, Jexmark T, Hansen F, et al: Bileaflet blood cysts on the mitral valve in an adult. J Am Soc Echocardiogr 2009;22:1085.e5–1085.e8. 5. Minneci C, Casolo G, Popoff G, et al: A rare case of left ventricular outflow obstruction. Eur J Echocardiogr 2004;5:72–75. 6. Hauser AM, Rathod K, McGill J, et al: Blood cyst of the papillary muscle. Clinical, echocardiographic and anatomic observations. Am J Cardiol 1983;51:612–613. 7. Miglioranza MH, Leiria TL, Haertel JC, et al: The role of three-dimensional echocardiography in interventricular mass evaluation. Echocardiography 2013;30:E125–E127. 8. Kochav J, Simprini L, Weinsaft JW: Imaging of the right heart-CT and CMR. Echocardiography 2015;32 (Suppl. 1): S53–68. 9. Altbach MI, Squire SW, Kudithipudi V, et al: Cardiac MRI is complementary to echocardiography in the assessment of cardiac masses. Echocardiography 2007; 24:286–300. 10. Lodha A, Patel J, Haran M, et al: Blood cyst of the mitral valve: A rare cause of stroke. Echocardiography 2009;26:736–738.
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Kalcßık, et al.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Two-dimensional transthoracic echocardiography modified apical fourchamber view revealed a cystic mass attached to the tricuspid valve. LV = left ventricle; RA = right atrium; RV = right ventricle. The arrow shows the blood filled cyst on tricuspid valve. Movie clip S2. Two-dimensional transesophageal echocardiography showed a cystic structure attached to the posterior leaflet of the
1202
tricuspid valve. LA = left atrium; RA = right atrium; RV = right ventricle. The arrow shows the blood filled cyst on tricuspid valve. Movie clip S3. Real time three-dimensional transesophageal echocardiography confirmed the presence of a semimobile pedunculated mass on the posterior tricuspid leaflet. MV = mitral valve; PL = posterior leaflet of tricuspid valve; SL = septal leaflet of tricuspid valve. The arrow shows the blood filled cyst on posterior leaflet of tricuspid valve.
Echocardiography
An Unusual Mass of Tricuspid Valve in an Adult Patient: Blood-Filled Cyst € rsoy, M.D.,‡ Su € leyman Karakoyun, M.D.,§ Macit Kalcßık, M.D.,* Mahmut Yesin, M.D.,† Mustafa Ozan Gu € € ksal, M.D.,¶ Cemil _Izgi, M.D.,** and Mehmet Ozkan, Sinan Cersßit, M.D.,† Cengiz Ko M.D.†,§ *Department of Cardiology, _Iskilip Atıf Hoca State Hospital, C ß orum,Turkey; †Department of Cardiology, Kosuyolu Kartal Heart Training and Research Hospital, Istanbul, Turkey; ‡Department of Cardiology, Gaziemir State Hospital, _Izmir, Turkey; §Department of Cardiology, Faculty of Medicine, Kars Kafkas University, Kars, Turkey; ¶Department of Cardiovascular Surgery, Kosuyolu Kartal Heart Training and Research Hospital, Istanbul, Turkey; and **Cardiovascular Magnetic Resonance Unit, Royal Brompton Hospital, London, United Kingdom
Blood-filled cysts (BFC) within the heart are common findings at postmortem examinations of fetuses and infants. However, such cysts are very rare entities in adolescents and adults. We report here an adult case of BFC attached to the posterior leaflet of the tricuspid valve, demonstrating the importance of multimodal diagnostic imaging combining both echocardiography and magnetic resonance imaging. (Echocardiography 2015;32:1199–1202) Key words: cardiac imaging, tricuspid valve, echocardiography, magnetic resonance imaging
Blood-filled cysts (BFC) are usually congenital in origin and located on the endocardium, particularly along the lines of closure of the heart valves. Their prevalence in postmortem examinations of fetuses and infants ranges from 25% to 100%.1 However, such cysts regress spontaneously in most of the patients and are rare in adults. The persistence of such cysts in the elderly may lead them to reach large diameters up to 40 mm and lead to cardiac dysfunction.2 Cyst wall consists of endothelial cells and a thin layer of fibrous tissue that contains nonorganized blood or serosanguineous fluid.3 Most of them are small and usually asymptomatic but, potential complications of larger BFCs include valve dysfunction, left ventricular outflow tract obstruction and embolic stroke. Here, we report an adult patient with a BFC attached to the posterior leaflet of the tricuspid valve which was treated with surgical excision. Case Presentation: A 65-year-old male patient who had a severe osteal left anterior descending (LAD) coronary artery stenosis (Fig. 1) was referred to our institution after a mass attached to the tricuspid valve Address for correspondence and reprint requests: Macit € u €er Sitesi Kalcik, M.D., Esentepe Mah., Milangaz Caddesi Unl B-Blok No: 22, Kartal, Istanbul, Turkey. Fax: (90)216 4596321; E-mail: [email protected]
was detected during routine transthoracic echocardiographic examination (Fig. 2A, movie clip S1). Physical examination did not reveal any pathological findings except for an elevated blood pressure. Transesophageal echocardiography (TEE) showed a cystic structure of 1.5 9 1.5 cm attached to the posterior leaflet of the tricuspid valve without relevant tricuspid regurgitation and no right ventricular inflow tract obstruction (Fig. 2B, movie clip S2). Real time three-dimensional TEE confirmed the presence of a semimobile pedunculated mass on the posterior tricuspid leaflet (Fig. 2C, movie clip S3). Subsequent cardiac magnetic resonance imaging (MRI) revealed a 15 9 18 mm possibly cystic mass attached to the posterior leaflet of the tricuspid valve which was iso-intense compared to myocardium on T1-weighted images (Fig. 3A). The cystic nature of the mass was confirmed by T2-weighted images as the central core was hyper-intense (Fig. 3B). After discussion of the findings within the heart team, operative resection of the mass was decided. Following median sternotomy and induction of cardiopulmonary bypass, the mass was reached via a left atrial and transseptal approach. When the surgeon holds the mass with forceps, fresh blood emerged from the tumor (Fig. 4A). Excision of the mass (Fig. 4B) and associated leaflet was followed by bicuspidization of tricuspid valve. The operation was accomplished after completion of the distal 1199
Kalcßık, et al.
Figure 1. Coronary angiography showed A. a severe osteal left anterior descending coronary artery stenosis with B. normal circumflex and nondominant right coronary artery. Cx = circumflex coronary artery; LAD = left anterior descending coronary artery; RCA = right coronary artery.
Figure 2. A. Two-dimensional transthoracic echocardiography modified apical four-chamber view revealed a cystic mass attached to the tricuspid valve. B. Two-dimensional transesophageal echocardiography (TEE) showed a cystic structure attached to the posterior leaflet of the tricuspid valve and C. real time three-dimensional TEE confirmed the presence of a semimobile pedunculated mass on the posterior tricuspid leaflet. LV = left ventricle; RA = right atrium; RV = right ventricle; AL = anterior leaflet of tricuspid valve; PL = posterior leaflet of tricuspid valve; SL = septal leaflet of tricuspid valve. The arrows show the blood-filled cyst on tricuspid valve.
Figure 3. Cardiac magnetic resonance imaging (MRI) revealed a possibly cystic mass attached to the posterior leaflet of the tricuspid valve which was iso-intense compared to myocardium on A. T1-weighted images and B. the central core of the mass was high signal on T2-weighted image confirming its cystic nature.
bypass anastomoses of left internal mammary artery on the LAD. Differential postoperative histological examination showed a blood-filled cystic cavity surrounded by a fibromyxoid wall with an internal lining of endothelial cells. Any malignancy, fibroelastoma or myxoma, was ruled out. The further postoperative course was
1200
uneventful, and the patient was discharged in a stable condition. Discussion: Intracardiac BFC are typically asymptomatic, usually congenital in origin, seen predominantly in infants. These cysts have been described on all
Blood-Filled Cyst on the Tricuspid Valve
Figure 4. A. Intraoperative photo revealing the blood emerging from the ruptured cystic mass while the surgeon holds it with forceps. B. Postoperative macroscopic specimen of the excised ruptured cystic mass. (The arrow shows the blood filled cyst on tricuspid valve)
cardiac valves and attached to the surfaces of all cardiac chambers.4 There are various hypotheses on the development of blood cysts, including malformations during valve development, hematoma formation in the subvalvular region, changes in remnants of primitive pericardial mesothelium and simply ectasia of blood vessels.5 Hauser et al. presented the first echocardiographic description of blood cysts attached to papillary muscles in 1983. The echocardiographic features of blood cysts consist of an echo-free space surrounded by a thin wall.6 Differential diagnosis should include myxoma, vegetation, hydatic cyst, thrombus, or malignancy. However, these entities tend to present as echo dense structures or structures with variable echogenicities. TEE is considered by most physicians as the method of choice if optimal visualization of valvular morphology is desired.7 Cardiac MRI also could be valuable to differentiate a BFC from other masses.8,9 On precontrast examinations, BFC are iso-intense compared to myocardium on T1-weighted images, and hyperintense on T2-weighted images. On the other hand, myxomas appear hypointense to myocardium on T1-weighted images and hyperintense on T2weighted images while fibromas appear hypointense on T2-weighted images and iso-intense on T1-weighted images. It is common to find regions of low signal intensity on T2-weighted images within the myxoma due to the presence calcification.9 Our case underlines the importance of an adequate preoperative diagnostic workup of such an intracardiac mass, combining echocardiography and cardiac MRI. There is no consensus or any guidelines regarding the optimal management of asymptomatic cysts. According to very few case reports (fewer than 50), it seems reasonable to adopt a conservative approach in a patient with no symp-
toms and small cysts, while surgical resection should be considered if symptoms exist or if the cysts cause any cardiac outflow obstruction or embolic strokes.10 Despite the asymptomatic state of the patient, the size and mobility of the cyst, with a relevant risk of potentially embolization, as well as the concomitant osteal LAD disease, led to the indication for a surgical approach in our patient. Therapeutic decision making should take into account the size, location and mobility of the cyst as well as relevant cardiac comorbidities and apply multimodal diagnostic imaging. References 1. Zimmerman KG, Paplanus SH, Dong S, et al: Congenital blood cysts of the heart valves. Hum Pathol 1983;14:699–703. 2. Abreu A, Galrinho A, Sa EP, et al: Hamartoma of the mitral valve with blood cysts: A rare tumor detected by echocardiography. J Am Soc Echocardiogr 1998;11:832– 836. 3. Boyd TAB: Blood cysts on the heart valves of infants. Am J Pathol 1949;25:757–759. 4. Dencker M, Jexmark T, Hansen F, et al: Bileaflet blood cysts on the mitral valve in an adult. J Am Soc Echocardiogr 2009;22:1085.e5–1085.e8. 5. Minneci C, Casolo G, Popoff G, et al: A rare case of left ventricular outflow obstruction. Eur J Echocardiogr 2004;5:72–75. 6. Hauser AM, Rathod K, McGill J, et al: Blood cyst of the papillary muscle. Clinical, echocardiographic and anatomic observations. Am J Cardiol 1983;51:612–613. 7. Miglioranza MH, Leiria TL, Haertel JC, et al: The role of three-dimensional echocardiography in interventricular mass evaluation. Echocardiography 2013;30:E125–E127. 8. Kochav J, Simprini L, Weinsaft JW: Imaging of the right heart-CT and CMR. Echocardiography 2015;32 (Suppl. 1): S53–68. 9. Altbach MI, Squire SW, Kudithipudi V, et al: Cardiac MRI is complementary to echocardiography in the assessment of cardiac masses. Echocardiography 2007; 24:286–300. 10. Lodha A, Patel J, Haran M, et al: Blood cyst of the mitral valve: A rare cause of stroke. Echocardiography 2009;26:736–738.
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Kalcßık, et al.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Two-dimensional transthoracic echocardiography modified apical fourchamber view revealed a cystic mass attached to the tricuspid valve. LV = left ventricle; RA = right atrium; RV = right ventricle. The arrow shows the blood filled cyst on tricuspid valve. Movie clip S2. Two-dimensional transesophageal echocardiography showed a cystic structure attached to the posterior leaflet of the
1202
tricuspid valve. LA = left atrium; RA = right atrium; RV = right ventricle. The arrow shows the blood filled cyst on tricuspid valve. Movie clip S3. Real time three-dimensional transesophageal echocardiography confirmed the presence of a semimobile pedunculated mass on the posterior tricuspid leaflet. MV = mitral valve; PL = posterior leaflet of tricuspid valve; SL = septal leaflet of tricuspid valve. The arrow shows the blood filled cyst on posterior leaflet of tricuspid valve.
