Left Ventricular Cardiac Hemangioma Presenting With Atypical Chest Pain

Abstract

HomeCirculationVol. 117, No. 22Left Ventricular Cardiac Hemangioma Presenting With Atypical Chest Pain Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessReview ArticlePDF/EPUBLeft Ventricular Cardiac Hemangioma Presenting With Atypical Chest Pain Mattias Roser, MD, Ashraf Hamdan, MD, Takeshi Komoda, MD, Charalampos Kriatselis, MD, Philipp Stawowy, MD, Rudolf Meyer, MD, Roland Hetzer, MD, Christoph Knosalla, MD and Ingo Paetsch, MD Mattias RoserMattias Roser From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author , Ashraf HamdanAshraf Hamdan From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author , Takeshi KomodaTakeshi Komoda From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author , Charalampos KriatselisCharalampos Kriatselis From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author , Philipp StawowyPhilipp Stawowy From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author , Rudolf MeyerRudolf Meyer From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author , Roland HetzerRoland Hetzer From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author , Christoph KnosallaChristoph Knosalla From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author and Ingo PaetschIngo Paetsch From the Department of Internal Medicine/Cardiology (M.R., A.H., C. Kriatselis, P.S., I.P.), Department of Cardiothoracic & Vascular Surgery (T.K., R.H., C. Knosalla), and Department of Cardiovascular Pathology (R.M.), German Heart Institute Berlin, Berlin, Germany. Search for more papers by this author Originally published3 Jun 2008https://doi.org/10.1161/CIRCULATIONAHA.107.737486Circulation. 2008;117:2958–2960A 54-year-old male patient presented with atypical chest pain and exertional dyspnea. The physical examination and chest x-ray were unremarkable; the ECG showed sinus rhythm with left-axis deviation and a pathological Sokolow-Index with concomitant ST-depression in leads I, aVL, and V5–V6. Transthoracic echocardiography revealed the presence of a homogenously echodense, mobile left ventricular mass (dimensions: 15×12 mm) attached to the intraventricular septum (Figure, E). Cardiac magnetic resonance imaging was performed for tissue characterization: On T1-weighted imaging (Figure, A), the mass was isointense compared with the left ventricular myocardium, whereas on T2-weighted imaging (Figure, B), homogeneously bright signal intensity was found. No signs of infiltrative growth were seen. During first-pass infusion of a gadolinium-containing contrast agent, no relevant signal intensity increase could be detected; however, on postcontrast imaging (delayed enhancement technique), some contrast agent uptake was detected. Download figureDownload PowerPointFigure. A, T1-weighted imaging with fast-spin echo and blackblood suppression in 4-chamber view. The tumor has an intermediate signal intensity, similar to myocardium. B, T2-weighted imaging with fast-spin echo and blackblood suppression in 4-chamber view showing high signal intensity of the tumor (arrow). C and D, Coronary angiography in right (C) and left (D) anterior oblique view demonstrating the vascular supply of the tumor via 2 septal branches of the left anterior descending coronary artery. Vascular lakes are also visible within the tumor (arrows). E, Transthoracic echocardiogram (apical 4-chamber view) showing a homogeneous, globular cardiac mass attached to the interventricular septum of the left ventricle. F, Surgeon’s view through open aortic valve demonstrated a smooth-walled, purple nodule 15 mm in diameter. G and H, Microscopic examination revealed large, endothelial-lined, blood-containing spaces with thick fibroblastic walls. Areas of capillary-type vessels were present. The channels were separated by sparse connective tissue. Immunohistochemical staining with the endothelial marker CD31 confirmed the presence of an internal layer of thin endothelial cells. G, CD31 staining, magnification ×10. H, Hematoxylin-eosin staining, magnification ×10.A differential diagnosis of benign cardiac tumors with high signal intensity on T2-weighted imaging includes hemangioma and highly vascularized myxoma. Although myxoma are rarely located on the ventricular level and tend to show a heterogeneous signal intensity distribution resulting from cystic-regressive and necrotic areas, the homogeneously bright signal on T2-weighted imaging (light bulb sign) together with left ventricular localization favored the diagnosis of hemangioma. Invasive coronary angiography (Figure, C and D) excluded obstructive epicardial coronary disease; however, septal branches of the left ascending artery gave rise to an extensive capillary-like vascular net, and a typical tumor blush could be seen. Surgical resection of the mass was performed, and histopathologic evaluation (Figure, F through H) revealed mostly capillary-type vessels separated by sparse connective tissue. Immunohistochemically, a thin internal layer of cells with endothelial origin was established (endothelial marker CD31), thereby corroborating the diagnosis of capillary-type hemangioma (Figure, G). Our patient recovered uneventfully and was discharged home in good condition.Primary tumors of the heart are rare and often diagnosed postmortem because of a mostly asymptomatic clinical course. The frequency of primary cardiac tumors seen at autopsy is ≈0.02%.1 Three quarters of cardiac tumors can be classified histologically as benign, with myxoma being the most frequent entity. Hemangiomas of the heart are extremely rare, accounting for only 2% to 3% of all benign primary cardiac tumors. Hemangiomas can present at all ages, though the diagnosis is preferentially made during the 5th decade of life.2 Although cardiac hemangiomas are often asymptomatic, the main symptoms include dyspnea, palpitation, atypical chest pain, and arrhythmia. Other symptoms may result from compression of surrounding structures, obstruction of the outflow tracts, pericardial effusion, or embolization.3 Echocardiography represents the diagnostic imaging modality of choice to appropriately screen for cardiac tumors.4 Computed tomography and magnetic resonance imaging are complementary methods in the diagnostic workup of cardiac tumors. Magnetic resonance imaging is highly flexible with regard to the selection of imaging planes, which may prove helpful during preoperative planning. In addition, its inherently high soft-tissue contrast using different weightings usually provides a more distinct tissue characterization of the mass. In the present case, the cardiac hemangioma appeared isointense compared with the surrounding myocardium on native T1-weighted images, whereas it was homogeneously hyperintense on T2-weighted images (so called light bulb sign, which is due to the higher signal arising from the slowly flowing blood within the capillarized tumor).5 Coronary angiography will usually be an integral part of the preoperative assessment to exclude obstructive coronary artery disease and to evaluate the feeding arteries or reveal encasement of coronary vessels by the tumor. Surgical resection is the treatment of choice for symptomatic cardiac hemangioma and is considered to be curative in most cases.6,7The online-only Data Supplement, which contains a movie, is available with this article at http://circ.ahajournals.org/cgi/content/full/118/22/2958/DC1.DisclosuresNone.FootnotesCorrespondence to Mattias Roser, MD, Internal Medicine/Cardiology, German Heart Institute Berlin, Augustenburger Platz 1, 13353 Berlin, Germany. E-mail [email protected]References1 Reynen K. Frequency of primary tumors of the heart. Am J Cardiol. 1996; 77: 107.CrossrefMedlineGoogle Scholar2 Thomas JE, Eror AT, Kenney M, Caravalho J Jr. Asymptomatic right atrial cavernous hemangioma: a case report and review of the literature. Cardiovasc Pathol. 2004; 13: 341–344.CrossrefMedlineGoogle Scholar3 Cunningham T, Lawrie GM, Stavinoha J Jr, Quinones MA, Zoghbi WA. Cavernous hemangioma of the right ventricle: echocardiographic-pathologic correlates. J Am Soc Echocardiogr. 1993; 6: 335–340.CrossrefMedlineGoogle Scholar4 Brizard C, Latremouille C, Jebara VA, Acar C, Fabiani JN, Deloche A, Carpentier AF. Cardiac hemangiomas. Ann Thorac Surg. 1993; 56: 390–394.CrossrefMedlineGoogle Scholar5 Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin JR. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics. 2000; 20: 1073–1103.CrossrefMedlineGoogle Scholar6 Soberman MS, Plauth WH, Winn KJ, Forest GC, Hatcher CR Jr, Sink JD. Hemangioma of the right ventricle causing outflow tract obstruction. J Thorac Cardiovasc Surg. 1988; 96: 307–309.CrossrefMedlineGoogle Scholar7 Kipfer B, Englberger L, Stauffer E, Carrel T. Rare presentation of cardiac hemangiomas. Ann Thorac Surg. 2000; 70: 977–979.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Sun Z, Wang B, Li H, Zhang L, Li Y and Xie M (2020) Left ventricular hemangioma and quadricuspid aortic valve: a rare combination, The International Journal of Cardiovascular Imaging, 10.1007/s10554-019-01757-9, 36:4, (623-625), Online publication date: 1-Apr-2020. Wang Y, Liu S, Yang J, Gu T and Zhang L (2017) Cardiac hemangioma caused ventricular arrhythmia: A rare case and literature review, Journal of Electrocardiology, 10.1016/j.jelectrocard.2017.04.014, 50:5, (667-670), Online publication date: 1-Sep-2017. Jeong S, Seol S, Park D, Sa-Kong H, Song Y, Min H and Kim J (2017) Asymptomatic left ventricular hemangioma, Radiology Case Reports, 10.1016/j.radcr.2017.03.021, 12:2, (240-243), Online publication date: 1-Jun-2017. Li W, Teng P, Xu H, Ma L and Ni Y (2015) Cardiac Hemangioma: A Comprehensive Analysis of 200 Cases, The Annals of Thoracic Surgery, 10.1016/j.athoracsur.2015.02.064, 99:6, (2246-2252), Online publication date: 1-Jun-2015. Fathala A (2012) Left Ventricular Cardiac Cyst, Circulation, 10.1161/CIRCULATIONAHA.111.050492, 125:17, (2171-2172), Online publication date: 1-May-2012. Para M, Lairez O, Guilbeau-Frugier C and Marcheix B (2011) Hémangiome caverneux de l’oreillette droite, Annales de Cardiologie et d'Angéiologie, 10.1016/j.ancard.2010.12.023, 60:3, (173-175), Online publication date: 1-Jun-2011. Martínez-Rodríguez I, Banzo I, Quirce R, Jiménez-Bonilla J, Medina-Quiroz P, Rubio-Vassallo A and Carril J (2010) F-18 FDG PET/CT Uptake by a Cardiac Hemangioma, Clinical Nuclear Medicine, 10.1097/RLU.0b013e3181d626af, 35:5, (330-331), Online publication date: 1-May-2010. June 3, 2008Vol 117, Issue 22 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.107.737486PMID: 18519864 Originally publishedJune 3, 2008 PDF download Advertisement SubjectsCardiovascular SurgeryComputerized Tomography (CT)