Echocardiographic History of an Asymptomatic Congenital Cardiac Tumor
2006; Lippincott Williams & Wilkins; Volume: 114; Issue: 21 Linguagem: Inglês
10.1161/circulationaha.106.637116
ISSN1524-4539
AutoresAndrea Motto, Piercarlo Ballo, Arianna Bocelli, Silvana Gramenzi, Maurizio de Martino,
Tópico(s)Congenital Diaphragmatic Hernia Studies
ResumoHomeCirculationVol. 114, No. 21Echocardiographic History of an Asymptomatic Congenital Cardiac Tumor Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBEchocardiographic History of an Asymptomatic Congenital Cardiac TumorNo Changes in Mass Dimensions During a 14-Year Follow-Up Andrea Motto, MD, Piercarlo Ballo, MD, Arianna Bocelli, MD, Silvana Gramenzi, MD and Maurizio de Martino, MD Andrea MottoAndrea Motto From the Cardiology Operative Unit, S. Andrea Hospital, La Spezia (A.M., P.B.); Department of Pediatrics, University of Florence, Anna Meyer Children's Hospital, Florence (A.B., M.d.M.); and Department of Cardiology, Fatebenefratelli ed Oftalmico Hospital, Milano (S.G.), Italy. , Piercarlo BalloPiercarlo Ballo From the Cardiology Operative Unit, S. Andrea Hospital, La Spezia (A.M., P.B.); Department of Pediatrics, University of Florence, Anna Meyer Children's Hospital, Florence (A.B., M.d.M.); and Department of Cardiology, Fatebenefratelli ed Oftalmico Hospital, Milano (S.G.), Italy. , Arianna BocelliArianna Bocelli From the Cardiology Operative Unit, S. Andrea Hospital, La Spezia (A.M., P.B.); Department of Pediatrics, University of Florence, Anna Meyer Children's Hospital, Florence (A.B., M.d.M.); and Department of Cardiology, Fatebenefratelli ed Oftalmico Hospital, Milano (S.G.), Italy. , Silvana GramenziSilvana Gramenzi From the Cardiology Operative Unit, S. Andrea Hospital, La Spezia (A.M., P.B.); Department of Pediatrics, University of Florence, Anna Meyer Children's Hospital, Florence (A.B., M.d.M.); and Department of Cardiology, Fatebenefratelli ed Oftalmico Hospital, Milano (S.G.), Italy. and Maurizio de MartinoMaurizio de Martino From the Cardiology Operative Unit, S. Andrea Hospital, La Spezia (A.M., P.B.); Department of Pediatrics, University of Florence, Anna Meyer Children's Hospital, Florence (A.B., M.d.M.); and Department of Cardiology, Fatebenefratelli ed Oftalmico Hospital, Milano (S.G.), Italy. Originally published21 Nov 2006https://doi.org/10.1161/CIRCULATIONAHA.106.637116Circulation. 2006;114:e591–e593A 14-year-old girl with a history of a congenital cardiac tumor presented to our echocardiography laboratory for a routine reexamination. The diagnosis had been made in our laboratory in 1991 on her seventh day of life using an old-generation ultrasound machine (SSH-40A, Toshiba Medical Systems, Tokyo, Japan). The newborn had undergone echocardiographic evaluation because of a systolic murmur. Despite the low technical quality of the images (Figure 1 and Movie I), a large mass involving the interventricular septum (4.2×1.5 cm, as measured from the apical 4-chamber view), suggestive of a congenital cardiac tumor, was visualized. Subsequent analyses had supported the diagnosis of cardiac rhabdomyoma. No other significant comorbidities, including tuberous sclerosis—a genetic condition often associated with the presence of cardiac rhabdomyoma1—had been discovered. Because of the absence of symptoms, a conservative approach was adopted. Clinical and echocardiographic follow-up during the subsequent years showed substantial tumor stability, with no development of symptoms. Download figureDownload PowerPointFigure 1. Original echocardiographic image obtained in 1991 using an old-generation ultrasound machine and stored using a VHS videotape. Despite the poor quality of the image, a large mass, highly suggestive of a congenital cardiac tumor, was detected within the interventricular septum (white arrow). LV indicates left ventricle; RV, right ventricle.At the present examination, the girl was asymptomatic and in good condition. Two-dimensional echocardiography (Sonos 7500 Live 3D Echo, Philips Medical Systems, Andover, Mass) showed an inhomogeneous echogenic mass arising from the mid-apical portion of the interventricular septum (Figures 2 to 4). The dimensions of the mass at end-diastole were similar to those measured at birth (4.4 cm, 1.6 cm, and 3.0 cm in the craniocaudal, transverse, and anteroposterior axis, respectively). In accordance with the muscular nature of the tumor, the mass showed a slight thickening throughout systole (Movies II to IV). The rest of the heart was structurally normal, and no functional abnormalities were found. Download figureDownload PowerPointFigure 2. Echocardiographic parasternal long-axis view of the tumor (white arrow) at end-diastole. LV indicates left ventricle; RV, right ventricle.Download figureDownload PowerPointFigure 3. Echocardiographic off-axis apical view, obtained by modifying a standard 4-chamber view to optimize visualization of the tumor (white arrow) at end-diastole. LV indicates left ventricle; RV, right ventricle.Download figureDownload PowerPointFigure 4. Echocardiographic parasternal short-axis view of the tumor (white arrow) at end-diastole. LV indicates left ventricle; RV, right ventricle.Real-time 3-dimensional imaging and offline quantitative analysis of data (3DQ-QLAB advanced quantification software, version 3.0, Philips Ultrasound) allowed detailed visualization of the mass, revealing an irregular multilobed architecture with inhomogeneous echogenicity and a torsional–dyskinetic movement during systole (Figures 5 and 6 and Movie V). Tumor dimensions were only slightly larger than those measured by the 2-dimensional approach (craniocaudal: 4.6 cm; transverse: 1.8 cm; anteroposterior: 3.1 cm; volume: 13.9 cm3). Download figureDownload PowerPointFigure 5. Three-dimensional echocardiographic image of the septal rhabdomyoma. The mass was characterized by an irregular multilobed architecture (white arrows) with inhomogeneous echogenicity. LV indicates left ventricle; RV, right ventricle.Download figureDownload PowerPointFigure 6. Three-dimensional echocardiographic image showing the tumor as seen from the apex of the left ventricle. The mass showed 2 main lobes in the posterior portion of the septum (white arrows). IVS indicates interventricular septum; MAL, mitral anterior leaflet; MPL, mitral posterior leaflet; TAL, tricuspid anterior leaflet; TPL, tricuspid posterior leaflet; and TSL, tricuspid septal leaflet.Congenital cardiac tumors are rare occurrences, having a prevalence of 0.14%.2 Rhabdomyomas are the most common tumors, accounting for about 50% of all cases in children.3 A strong association with tuberous sclerosis has been reported. Among subjects with cardiac rhabdomyoma, 51% to 86% have tuberous sclerosis, whereas more than half of patients with tuberous sclerosis show cardiac rhabdomyomas.1 These rhabdomyomas are usually characterized by multiple masses, with a peduncolated or rounding intramural shape, and they most commonly involve the ventricular myocardium. Cardiac rhabdomyomas may present at birth with a wide spectrum of clinical features, ranging from occasional echocardiographic detection in asymptomatic newborns to presentations with cardiac rhythm disturbances, Wolff-Parkinson-White syndrome, congestive heart failure caused by flow obstruction or valve regurgitation, and/or sudden death. Cardiac symptoms represent a common finding in the first years of life, but spontaneous regression of the tumor occurs in the majority of cases.4These images illustrate the echocardiographic history of an isolated congenital cardiac rhabdomyoma in an asymptomatic female patient. The evolution of ultrasound techniques—particularly the possibility of performing real-time 3-dimensional imaging—has allowed considerably higher definition of tumor characteristics throughout years. The following unusual aspects should also be pointed out: 1) The tumor was not associated with tuberous sclerosis; 2) the rhabdomyoma was characterized by a single, multilobed mass; 3) the patient did not develop any cardiac symptom; and 4) the dimensions of the mass did not change significantly during a 14-year follow-up.The online-only Data Supplement, which contains 5 movies, can be found at http://circ.ahajournals.org/cgi/content/full/114/21/e591/DC1.AcknowledgmentsThe authors thank Massimiliano Faberi, MCSE, for his important support in the digital acquisition and processing of the original echocardiographic video, stored on a VHS videotape in 1991.DisclosuresNone.FootnotesCorrespondence to Dr Piercarlo Ballo, U.O. Cardiologia, Ospedale "S. Andrea," Via Veneto 197, 19100 La Spezia, Italy. E-mail [email protected]References1 Harding CO, Pagon RA. Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma. Am J Med Genet. 1990; 37: 443–446.CrossrefMedlineGoogle Scholar2 Holley DG, Martin GR, Brenner JI, Fyfe DA, Huhta JC, Kleinman CS, Ritter SB, Silverman NH. Diagnosis and management of fetal cardiac tumors: a multicenter experience and review of published reports. J Am Coll Cardiol. 1995; 26: 516–520.CrossrefMedlineGoogle Scholar3 Piazza N, Chughtai T, Toledano K, Sampalis J, Liao C, Morin JF. Primary cardiac tumours: eighteen years of surgical experience on 21 patients. Can J Cardiol. 2004; 20: 1443–1448.MedlineGoogle Scholar4 Beghetti M, Gow RM, Haney I, Mawson J, Williams WG, Freedom RM. Pediatric primary benign cardiac tumors: a 15-year review. Am Heart J. 1997; 134: 1107–1114.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Padalino M, Reffo E, Cerutti A, Favero V, Biffanti R, Vida V, Stellin G and Milanesi O (2013) Medical and surgical management of primary cardiac tumours in infants and children, Cardiology in the Young, 10.1017/S104795111300022X, 24:2, (268-274), Online publication date: 1-Apr-2014. Plicht B (2011) Cardiac tumors and sources of embolism Three-dimensional Echocardiography, 10.1007/978-3-642-11179-2_12, (223-240), . Chamsi-Pasha M, Anwar A, Al-Nasser I, Nosir Y, Ajam A and Chamsi-Pasha H (2009) Imaging of Ventricular Septal Tumor in an Asymptomatic Adolescent using Multiple Modalities, Echocardiography, 10.1111/j.1540-8175.2008.00850.x, 26:5, (581-585), Online publication date: 1-May-2009. November 21, 2006Vol 114, Issue 21 Advertisement Article InformationMetrics https://doi.org/10.1161/CIRCULATIONAHA.106.637116PMID: 17116773 Originally publishedNovember 21, 2006 PDF download Advertisement SubjectsCongenital Heart DiseaseEchocardiography
Referência(s)