Giant Intramural Left Ventricular Rhabdomyoma in a Newborn
2011; Lippincott Williams & Wilkins; Volume: 124; Issue: 20 Linguagem: Inglês
10.1161/circulationaha.111.017897
ISSN1524-4539
AutoresMassimo A. Padalino, Vladimiro L. Vida, Anil Bhattarai, Elena Reffo, Ornella Milanesi, Gaetano Thiene, Giovanni Stellin, Cristina Basso,
Tópico(s)Cardiac Structural Anomalies and Repair
ResumoHomeCirculationVol. 124, No. 20Giant Intramural Left Ventricular Rhabdomyoma in a Newborn Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessBrief ReportPDF/EPUBGiant Intramural Left Ventricular Rhabdomyoma in a Newborn Massimo A. Padalino, Vladimiro L. Vida, Anil Bhattarai, Elena Reffo, Ornella Milanesi, Gaetano Thiene, Giovanni Stellin and Cristina Basso Massimo A. PadalinoMassimo A. Padalino From the Paediatric and Congenital Cardiac Surgery Unit (A.B.), Department of Cardiac, Thoracic and Vascular Sciences (M.A.P., V.L.V., G.S.); Division of Paediatric Cardiology, Department of Paediatrics (E.R., O.M.); and Department of Medical and Diagnostic Sciences and Special Therapies (G.T., C.B.), University of Padova Medical School, Padova, Italy. , Vladimiro L. VidaVladimiro L. Vida From the Paediatric and Congenital Cardiac Surgery Unit (A.B.), Department of Cardiac, Thoracic and Vascular Sciences (M.A.P., V.L.V., G.S.); Division of Paediatric Cardiology, Department of Paediatrics (E.R., O.M.); and Department of Medical and Diagnostic Sciences and Special Therapies (G.T., C.B.), University of Padova Medical School, Padova, Italy. , Anil BhattaraiAnil Bhattarai From the Paediatric and Congenital Cardiac Surgery Unit (A.B.), Department of Cardiac, Thoracic and Vascular Sciences (M.A.P., V.L.V., G.S.); Division of Paediatric Cardiology, Department of Paediatrics (E.R., O.M.); and Department of Medical and Diagnostic Sciences and Special Therapies (G.T., C.B.), University of Padova Medical School, Padova, Italy. , Elena ReffoElena Reffo From the Paediatric and Congenital Cardiac Surgery Unit (A.B.), Department of Cardiac, Thoracic and Vascular Sciences (M.A.P., V.L.V., G.S.); Division of Paediatric Cardiology, Department of Paediatrics (E.R., O.M.); and Department of Medical and Diagnostic Sciences and Special Therapies (G.T., C.B.), University of Padova Medical School, Padova, Italy. , Ornella MilanesiOrnella Milanesi From the Paediatric and Congenital Cardiac Surgery Unit (A.B.), Department of Cardiac, Thoracic and Vascular Sciences (M.A.P., V.L.V., G.S.); Division of Paediatric Cardiology, Department of Paediatrics (E.R., O.M.); and Department of Medical and Diagnostic Sciences and Special Therapies (G.T., C.B.), University of Padova Medical School, Padova, Italy. , Gaetano ThieneGaetano Thiene From the Paediatric and Congenital Cardiac Surgery Unit (A.B.), Department of Cardiac, Thoracic and Vascular Sciences (M.A.P., V.L.V., G.S.); Division of Paediatric Cardiology, Department of Paediatrics (E.R., O.M.); and Department of Medical and Diagnostic Sciences and Special Therapies (G.T., C.B.), University of Padova Medical School, Padova, Italy. , Giovanni StellinGiovanni Stellin From the Paediatric and Congenital Cardiac Surgery Unit (A.B.), Department of Cardiac, Thoracic and Vascular Sciences (M.A.P., V.L.V., G.S.); Division of Paediatric Cardiology, Department of Paediatrics (E.R., O.M.); and Department of Medical and Diagnostic Sciences and Special Therapies (G.T., C.B.), University of Padova Medical School, Padova, Italy. and Cristina BassoCristina Basso From the Paediatric and Congenital Cardiac Surgery Unit (A.B.), Department of Cardiac, Thoracic and Vascular Sciences (M.A.P., V.L.V., G.S.); Division of Paediatric Cardiology, Department of Paediatrics (E.R., O.M.); and Department of Medical and Diagnostic Sciences and Special Therapies (G.T., C.B.), University of Padova Medical School, Padova, Italy. Originally published15 Nov 2011https://doi.org/10.1161/CIRCULATIONAHA.111.017897Circulation. 2011;124:2275–2277A 1-day-old newborn was referred with prenatal echocardiographic diagnosis of cardiac mass. Born by caesarean section at 38+3 weeks of pregnancy, he presented with cyanosis and bradycardia at birth, requiring immediate intubation and mechanical ventilation. Chest x-ray demonstrated cardiomegaly with a cardiothoracic index of 0.7 (Figure 1). Echocardiographic examination (Figure 2 and Movie I in the online-only Data Supplement) confirmed the diagnosis of a large cardiac mass located at the posterior wall and at the apex of the left ventricle (LV). The child presented with several episodes of paroxysmal supraventricular tachycardia, which were treated with DC shock and various pharmacological agents, such as amiodarone, adenosine, flecainide, digoxin, and metoprolol, and 1 episode of ventricular fibrillation with cardiac arrest requiring resuscitation. MRI showed a single large mass (5.5×5 cm) arising from the left atrioventricular groove and extending to the LV lateral wall and apex, up to the interventricular septum, with no LV inflow or outflow obstruction, and was suggestive for cardiac fibroma (Figure 3A through 3C and Movie II in the online-only Data Supplement). The left bronchus was partially obstructed by external compression from the mass. However, further MRI tissue characterization was compatible with a LV rhabdomyoma, because the gradient echo sequences showed signal intensity of the mass similar to that of the myocardium. In addition, turbo-spin echo sequences with fat saturation ruled out the presence of fatty tissue. Finally, the images obtained after gadolinium chelate injection (Figure 3D) did not show any enhancement of the tumor, and this finding was significant enough to rule out a fibroma.Download figureDownload PowerPointFigure 1. Postnatal chest x-ray. The cardiac silhouette is dilated (cardiothoracic index, 0.7). Note the endotracheal tube and umbilical line.Download figureDownload PowerPointFigure 2. Postnatal echocardiographic examination, showing a large cardiac mass, located at the posterior wall and apex of the left ventricle, and involving the interventricular septum. LV indicates left ventricle; RV, right ventricle.Download figureDownload PowerPointFigure 3. Cardiac MRI investigation. A, ECG-triggered breath-hold proton density T1-weighted fast spin echo in coronal plane, showing a large homogeneous isointense mass involving the LV wall. B, ECG-triggered breath-hold cine steady-state free procession in axial plane, showing the cardiac mass involving the interventricular septum. C, ECG-triggered breath-hold cine steady state free procession in 4-chamber view, showing no intracardiac obstruction. D, After gadolinium injection, no mass hyperenhancement is visible. Ao indicates aorta; RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle.Further preoperative evaluation of the coronary arteries was not performed. Finally, neurological investigation excluded the association with tuberous sclerosis.At 14 days of life, the patient was scheduled for surgical resection of the cardiac mass. After median sternotomy, on cardiopulmonary bypass, the large solid mass (Figure 4A) was partially resected (Figure 4B) to preserve the coronary branches that were running close to it, as evidenced intraoperatively. The postoperative course in the intensive care unit was unremarkable, on low-dose inotropes, without supraventricular tachycardia recurrence. The patient required mechanical ventilation for 4 days, and was discharged from the intensive care unit on the 8th postoperative day and eventually discharged from the hospital on the 17th postoperative day. Histopathologic examination revealed the presence of enlarged, vacuolated cells with sparse cytoplasm and so-called spider cells, ie, cells characterized by a typical centrally located nucleus with radial extensions to the cell periphery. Immunohistochemical studies showed features of striated muscle cells (myoglobin positive) confirming the diagnosis of cardiac rhabdomyoma (Figure 5). Ten months after the operation, the child is alive and in good clinical condition, with no oral medications. Follow-up ECG Holter monitoring documents sinus rhythm, and echocardiographic assessment demonstrates a good LV function (shortening fraction 37%) and only a small residual mass in the apical portion of the interventricular septum, not hemodynamically significant.Download figureDownload PowerPointFigure 4. Intraoperative view. A, The large solid mass is exposed after median sternotomy. B, Operative view of the tumor during resection. LV indicates left ventricle; RV, right ventricle.Download figureDownload PowerPointFigure 5. Histopathologic study. A, Enlarged swollen myocytes with clear cytoplasm and centrally located nucleus, connected with the periphery of the cell by strands of cytoplasm (spider cells) (hematoxylin-eosin stain, ×80). B, the enlarged cells are vacuolated because of the abundant glycogen deposits in the cytoplasm (Alcian PAS stain ×160); C) immunohistochemistry reveals striated muscular cells in keeping with cardiac rhabdomyoma (myosin ×160).Primary cardiac tumors are rare entities, occurring with a lifetime incidence of 0.0017% to 0.02%.1 In the pediatric population, cardiac tumors have been identified in 0.02% to 0.04%,2,3 and the majority of them are benign rhabdomyomas. Early identification with fetal echocardiography, associated with postnatal MRI tissue characterization, enables prompt postnatal treatment with lifesaving surgical resection of the mass when significant hemodynamic compromise or life-threatening arrhythmias occur.4DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.111.017897/-/DC1.Correspondence to Massimo A. Padalino, MD, PhD, Pediatric and Congenital Cardiac Surgery Unit, Centro "V. Gallucci," Via Giustiniani 2, 35128 Padova, Italy. E-mail massimo.[email protected]itReferences1. Thiene G, Valente M, Lombardi M, Basso C. Tumours of the Heart. In: , Camm JA, Luscher TF, Serruys PV. ESC Textbook of Cardiovascular Medicine. New York, NY: Oxford University Press; 2009. CrossrefGoogle Scholar2. Padalino MA, Basso C, Milanesi O, Vida VL, Moreolo GS, Thiene G, Stellin G. Surgically treated primary cardiac tumors in early infancy and childhood. J Thorac Cardiovasc Surg. 2005; 129:1358–1363.CrossrefMedlineGoogle Scholar3. Freedom RM, Lee KJ, MacDonald C, Taylor G. Selected aspects of cardiac tumors in infancy and childhood. Pediatr Cardiol. 2000; 21:299–316.CrossrefMedlineGoogle Scholar4. De Rosa G, De Carolis MP, Pardeo M, Bersani I, Tempera A, De Nisco A, Caforio L, Romagnoli C, Piastra M. Neonatal emergencies associated with cardiac rhabdomyomas: an 8-year experience. Fetal Diagn Ther. 2011; 29:169–177.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Cavalcante C, Pinto Junior V, Pompeu R, Teles A, Bandeira J, Maia I, Tavora F, Cavalcante M, Zamarian A, Araujo Júnior E and Castello Branco K (2019) Perinatal unusual rhabdomyoma location – case report and systematic reviews of the literature, The Journal of Maternal-Fetal & Neonatal Medicine, 10.1080/14767058.2019.1597044, 34:1, (137-151), Online publication date: 2-Jan-2021. Msaad H, Drissa M, Hakim K and Ourda F (2018) Epidemiology and outcome of primary cardiac tumours prenatally, in neonatesneonnates and children: A single center experience from tunis, The Egyptian Heart Journal, 10.1016/j.ehj.2018.09.009, 70:4, (279-282), Online publication date: 1-Dec-2018. Pelella G, Ramaraj R, Dhannapuneni R and Guerrero R (2016) The Use of Video-Assisted Cardioscopy for Neonatal Left Ventricular Tumor Resection, World Journal for Pediatric and Congenital Heart Surgery, 10.1177/2150135116634073, 9:4, (463-466), Online publication date: 1-Jul-2018. Alshammari B and Shah D (2018) Cardiac Masses Cancer and Cardiovascular Disease, 10.1007/978-3-319-62088-6_12, (155-165), . Basso C, Rizzo S, Valente M and Thiene G (2016) Cardiac masses and tumours, Heart, 10.1136/heartjnl-2014-306364, 102:15, (1230-1245), Online publication date: 1-Aug-2016. Riegler J, Tiburcy M, Ebert A, Tzatzalos E, Raaz U, Abilez O, Shen Q, Kooreman N, Neofytou E, Chen V, Wang M, Meyer T, Tsao P, Connolly A, Couture L, Gold J, Zimmermann W and Wu J (2015) Human Engineered Heart Muscles Engraft and Survive Long Term in a Rodent Myocardial Infarction Model, Circulation Research, 117:8, (720-730), Online publication date: 25-Sep-2015. Stamatian F, Kovacs T, Hăşmăşanu M, Opriţa S, Andreica S, Mátyás M, Decean E and Zaharie G (2015) Cardiac Tumors in the Neonatal Period: Clinical Features and Echocardiographic Evaluation, Donald School Journal of Ultrasound in Obstetrics and Gynecology, 10.5005/jp-journals-10009-1355, 8:2, (201-207), Online publication date: 1-Jun-2014. Burch M and Dedieu N (2013) Almanac 2012: Congenital heart disease. The national society journals present selected research that has driven recent advances in clinical cardiology, Revista Portuguesa de Cardiologia (English Edition), 10.1016/j.repce.2013.04.008, 32:3, (269-277), Online publication date: 1-Mar-2013. Burch M and Dedieu N (2013) Almanac 2012: Congenital heart disease. The national society journals present selected research that has driven recent advances in clinical cardiology, Revista Portuguesa de Cardiologia, 10.1016/j.repc.2012.10.008, 32:3, (269-277), Online publication date: 1-Mar-2013. Burch M and Dedieu N (2013) Almanac 2012: Congenital heart disease. The national society journals present selected research that has driven recent advances in clinical cardiology, The Egyptian Heart Journal, 10.1016/j.ehj.2012.10.004, 65:1, (21-29), Online publication date: 1-Mar-2013. Burch M and Dedieu N (2013) Almanac 2012: Congenital heart disease, Archivos de Cardiología de México, 10.1016/j.acmx.2013.01.004, 83:1, (55-63), Online publication date: 1-Jan-2013. Thiene G, Valente M and Basso C (2013) Cardiac Tumors: From Autoptic Observations to Surgical Pathology in the Era of Advanced Cardiac Imaging Cardiac Tumor Pathology, 10.1007/978-1-62703-143-1_1, (1-22), . November 15, 2011Vol 124, Issue 20 Advertisement Article InformationMetrics © 2011 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.111.017897PMID: 22083149 Originally publishedNovember 15, 2011 PDF download Advertisement SubjectsCongenital Heart DiseaseImaging
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