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Initial Presentation of an Accessory Left Ventricle in a Patient With Syncope

2010; Lippincott Williams & Wilkins; Volume: 121; Issue: 19 Linguagem: Inglês

10.1161/cir.0b013e3181e036bf

1524-4539

Sara L. Partington, Bilal Ali, Ryan Daly, Bruce A. Koplan, Leonard S. Lilly, Scott D. Solomon, Raymond Y. Kwong, Ron Blankstein,

Cardiac Structural Anomalies and Repair

HomeCirculationVol. 121, No. 19Initial Presentation of an Accessory Left Ventricle in a Patient With Syncope Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessReview ArticlePDF/EPUBInitial Presentation of an Accessory Left Ventricle in a Patient With Syncope Sara L. Partington, MD, Bilal Ali, MD, Ryan P. Daly, MD, Bruce A. Koplan, MD, Leonard S. Lilly, MD, Scott D. Solomon, MD, Raymond Y. Kwong, MD and Ron Blankstein, MD Sara L. PartingtonSara L. Partington From the Noninvasive Cardiovascular Imaging Program, Department of Medicine and Radiology (S.L.P., B.Z., R.P.D., R.Y.K., R.B.), and Division of Cardiovascular Medicine, Department of Medicine (B.A.K., L.S.L., S.D.S., R.Y.K., R.B.), Brigham and Women's Hospital, Boston Mass. , Bilal AliBilal Ali From the Noninvasive Cardiovascular Imaging Program, Department of Medicine and Radiology (S.L.P., B.Z., R.P.D., R.Y.K., R.B.), and Division of Cardiovascular Medicine, Department of Medicine (B.A.K., L.S.L., S.D.S., R.Y.K., R.B.), Brigham and Women's Hospital, Boston Mass. , Ryan P. DalyRyan P. Daly From the Noninvasive Cardiovascular Imaging Program, Department of Medicine and Radiology (S.L.P., B.Z., R.P.D., R.Y.K., R.B.), and Division of Cardiovascular Medicine, Department of Medicine (B.A.K., L.S.L., S.D.S., R.Y.K., R.B.), Brigham and Women's Hospital, Boston Mass. , Bruce A. KoplanBruce A. Koplan From the Noninvasive Cardiovascular Imaging Program, Department of Medicine and Radiology (S.L.P., B.Z., R.P.D., R.Y.K., R.B.), and Division of Cardiovascular Medicine, Department of Medicine (B.A.K., L.S.L., S.D.S., R.Y.K., R.B.), Brigham and Women's Hospital, Boston Mass. , Leonard S. LillyLeonard S. Lilly From the Noninvasive Cardiovascular Imaging Program, Department of Medicine and Radiology (S.L.P., B.Z., R.P.D., R.Y.K., R.B.), and Division of Cardiovascular Medicine, Department of Medicine (B.A.K., L.S.L., S.D.S., R.Y.K., R.B.), Brigham and Women's Hospital, Boston Mass. , Scott D. SolomonScott D. Solomon From the Noninvasive Cardiovascular Imaging Program, Department of Medicine and Radiology (S.L.P., B.Z., R.P.D., R.Y.K., R.B.), and Division of Cardiovascular Medicine, Department of Medicine (B.A.K., L.S.L., S.D.S., R.Y.K., R.B.), Brigham and Women's Hospital, Boston Mass. , Raymond Y. KwongRaymond Y. Kwong From the Noninvasive Cardiovascular Imaging Program, Department of Medicine and Radiology (S.L.P., B.Z., R.P.D., R.Y.K., R.B.), and Division of Cardiovascular Medicine, Department of Medicine (B.A.K., L.S.L., S.D.S., R.Y.K., R.B.), Brigham and Women's Hospital, Boston Mass. and Ron BlanksteinRon Blankstein From the Noninvasive Cardiovascular Imaging Program, Department of Medicine and Radiology (S.L.P., B.Z., R.P.D., R.Y.K., R.B.), and Division of Cardiovascular Medicine, Department of Medicine (B.A.K., L.S.L., S.D.S., R.Y.K., R.B.), Brigham and Women's Hospital, Boston Mass. Originally published18 May 2010https://doi.org/10.1161/CIR.0b013e3181e036bfCirculation. 2010;121:e401–e403A 39-year-old woman was referred for a transthoracic echocardiogram after presenting to her primary care provider following an episode of unexplained loss of consciousness. Her past medical history was notable for fibromyalgia and migraine headaches. She had a normal cardiac and neurological physical examination with no extra heart sounds or murmurs.Her transthoracic echocardiogram (Movie I of the online-only Data Supplement) revealed a large outpouching of the lateral wall of the left ventricle (LV) that contracted in synchrony with the ventricle (Figure 1A and 1B). The initial differential for this finding included an LV diverticulum or an accessory ventricle. An LV pseudoaneurysm was considered unlikely because of the synchronous contraction of the outpouching with the rest of the ventricle. Download figureDownload PowerPointFigure 1. Transthoracic echocardiogram demonstrating the apical 4-chamber view of the LV. The outpouching (arrow) originating from the lateral aspect of the midventricular wall is shown in both diastole (A) and systole (B). The outpouching contracted synchronously with the LV.To further evaluate this abnormality, cardiac magnetic resonance imagine (MRI) was performed (Movie II of the online-only Data Supplement). This study demonstrated a large, irregular, multilobed, contracting outpouching of the mid to distal lateral wall of the LV (Figure 2). The neck measured 1.5×1.8 cm in diameter; the depth measured 2.5 cm. There was normal rest perfusion of the myocardium overlying this outpouching (Figure 3C). On late-enhancement imaging, there was no evidence of scar or infarction surrounding this area, thus eliminating the possibility of a prior infarct or scar leading to a pseudoaneurysm (Figure 3B and 3E). Notably, there were small projections within the wall of the outpouching, and in some of these areas (Figure 3D), the myocardium appeared particularly thin. Within the wall of the outpouching, there were very small areas of myocardial hyperenhancement enhancement most likely consistent with minimal fibrosis (Figure 3E). Cardiac MRI was also helpful in ruling out other cardiac causes of syncope such as infiltrative heart disease, myocardial scar, or other structural abnormalities. Download figureDownload PowerPointFigure 2. Cardiac MRI balanced steady-state free-procession image in the 4-chamber view demonstrating the multilobulated LV outpouching along the lateral wall. The presence of a partial accessory septum (arrow) was noted and supported the diagnosis of an accessory chamber rather than a diverticulum.Download figureDownload PowerPointFigure 3. A, Cardiac MRI gradient-echo image in the 4-chamber view of the LV. The white line demonstrates the plane used to obtain the short-axis views of the LV outpouching shown in B through E. B, A dual-inversion-recovery T2-weighted fast spin-echo short-axis image demonstrating no evidence of edema. The areas of increased signal (arrows) correspond to blood pooling within the LV and within the small projection off the LV outpouching. C, Imaging during first-pass perfusion with gadolinium demonstrating normal resting perfusion of the myocardium. These images also show the contiguous blood pool within the LV, the accessory chamber, and a small projection originating from the outpouching (arrow). D, Balanced steady-state free-procession postgadolinium image demonstrating small projections originating from the accessory chamber (arrows). The myocardium surrounding these projections was notably thin. E, Late-gadolinium-enhancement images demonstrating the absence of prior infarction or infiltration of the myocardium. Of note, minimal areas of myocardial hyperenhancement (arrow) were present, most likely consistent with mild fibrosis within the wall of the accessory LV.The differential diagnosis for a LV outpouching that contracts synchronously with the rest of the ventricle includes an LV diverticulum or an accessory chamber. A diverticulum contains all 3 layers of cardiac tissue but has a narrow connection to the ventricle. An accessory chamber contains all 3 layers of cardiac tissue but has a wide connection to the ventricle1 and may have an anomalous septum or muscle bundle that divides the ventricular cavity into 2 chambers.2 The imaging features identified in our case, including a large outpouching with a relatively wide neck, multilobed morphology, and a partial accessory septum (Figure 2), all favored the diagnosis of an accessory ventricle.Literature regarding accessory ventricular chambers in adult populations is sparse, given that the majority of cases have been reported in children and seem to be associated with cardiac systolic dysfunction or other cardiac abnormalities.1–3 However, patients who present with isolated LV outpouchings tend to remain asymptomatic and experience no complications, although ventricular arrhythmias, cardiac rupture, and sudden death have been reported. Thrombus formation within the outpouching causing systemic emboli has also been reported but is exceedingly rare because of the contractile function of the diverticulum or accessory chamber.4The finding of an accessory ventricular chamber in our patient, coupled with the presentation of unexplained loss of consciousness, led to an electrophysiology study. Although no ventricular arrhythmias could be induced, an implantable cardiac defibrillator was placed owing to the unexplained syncope and the presence of a cardiac structural abnormality known to cause ventricular arrhythmias.Guest Editor for this article was Leon Axel, MD, PhD.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/cgi/content/full/121/19/e401/DC1.DisclosuresNone.FootnotesCorrespondence to Ron Blankstein, MD, Brigham and Women's Hospital, 75 Francis St, Boston, MA 02115. E-mail [email protected]References1 Awad S, Patel A, Polimenakos A, Braun R, Abdulla R. Left ventricular accessory chamber. Pediatr Cardiol. 2009; 30: 1022–1025.CrossrefMedlineGoogle Scholar2 Harikrishnan S, Sivasankaran S, Tharakan J. Double chambered left ventricle. Int J Cardiol. 2002; 82: 59–61.CrossrefMedlineGoogle Scholar3 Kay PH, Rigby M, Mulholland HC. Congenital double chambered left ventricle treated by exclusion of the accessory chamber. Br Heart J. 1983; 49: 195–198.CrossrefMedlineGoogle Scholar4 Cianciulli TF, Colaso DCG, Saccheri MC, Lax JA, Redruello HJ, Guerra JE, Prezioso HA, Vidal LA. Left ventricular diverticulum, a rare echocardiographic finding. Cardiol J. 2009; 16: 76–81.MedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Park W, Kim D, Kwak Y and Cho S (2020) Double-chambered left ventricle discovered in patient with ruptured left ventricular aneurysm after myocardial infarction, The Journal of Thoracic and Cardiovascular Surgery, 10.1016/j.jtcvs.2019.06.092, 159:3, (e191-e194), Online publication date: 1-Mar-2020. Anderson R and Gufler H (2020) Commentary: What makes the morphologically left ventricle double chambered?, The Journal of Thoracic and Cardiovascular Surgery, 10.1016/j.jtcvs.2019.07.012, 159:3, (e195-e196), Online publication date: 1-Mar-2020. Wu J (2019) Mechanical Complications of Myocardial Infarction Essential Echocardiography, 10.1016/B978-0-323-39226-6.00019-9, (200-203.e1), . A D, C T, A T, P Z, N K, J P and D B (2018) An unusual case of double-chambered left ventricle, Journal of Echocardiography, 10.1007/s12574-018-0393-5, 17:3, (167-168), Online publication date: 1-Sep-2019. Zhang W, Chang D, Huang J, Maimaitiaili A, Ermek T, Yisireyili M and Zhang Z (2018) Double-chambered left ventricle: A rare case in a child, Echocardiography, 10.1111/echo.14193, 36:1, (192-195), Online publication date: 1-Jan-2019. Saavedra L, Lucena E and Michelena H (2017) "Aberrant" Papillary Muscle in a High-Endurance Athlete? The Importance of Advanced Cardiac Imaging, Canadian Journal of Cardiology, 10.1016/j.cjca.2016.08.008, 33:2, (293.e7-293.e8), Online publication date: 1-Feb-2017. Malakan Rad E, Awad S and Hijazi Z (2014) Congenital Left Ventricular Outpouchings: A Systematic Review of 839 Cases and Introduction of a Novel Classification after Two Centuries, Congenital Heart Disease, 10.1111/chd.12214, 9:6, (498-511), Online publication date: 1-Nov-2014. Mordi I, Carrick D and Tzemos N (2013) Diagnosis of Double-Chambered Left Ventricle Using Advanced Cardiovascular Imaging, Echocardiography, 10.1111/echo.12244, 30:7, (E206-E208), Online publication date: 1-Aug-2013. Nacif M, Mello R, Lacerda Junior O, Sibley C, Machado R and Marchiori E (2010) Double-chambered left ventricle in an adult: diagnosis by CMRI, Clinics, 10.1590/S1807-59322010001200028, 65:12, (1393-1395), Online publication date: 1-Jan-2010. Carretero Bellon J, Pérez Casares A, Brunet García L, Prada F and Sánchez de Toledo J (2020) Diagnóstico ante una deformación sacular del ventrículo izquierdo en niños. No todo es lo que parece, Revista de ecocardiografía práctica y otras técnicas de imagen cardíaca, 10.37615/retic.v3n3a14, 3:3, (6-9) May 18, 2010Vol 121, Issue 19 Advertisement Article InformationMetrics https://doi.org/10.1161/CIR.0b013e3181e036bfPMID: 20479159 Originally publishedMay 18, 2010 PDF download Advertisement SubjectsComputerized Tomography (CT)Developmental BiologyEchocardiographyImaging