Hostname: page-component-78c5997874-dh8gc Total loading time: 0 Render date: 2024-11-04T21:21:51.309Z Has data issue: false hasContentIssue false
  • English
  • Français

Cardiac rhabdomyoma in childhood tuberous sclerosis

Published online by Cambridge University Press:  19 August 2008

Jung Yun Choi ,
Eun Jung Bae ,
Chung Il Noh ,
Yong-Soo Yoon  and
Yong Seung Hwang
Jung Yun Choi*
Affiliation:
From the Department of Pediatrics, Seoul National University Children's Hospital, Seoul
Eun Jung Bae
Affiliation:
From the Department of Pediatrics, Seoul National University Children's Hospital, Seoul
Chung Il Noh
Affiliation:
From the Department of Pediatrics, Seoul National University Children's Hospital, Seoul
Yong-Soo Yoon
Affiliation:
From the Department of Pediatrics, Seoul National University Children's Hospital, Seoul
Yong Seung Hwang
Affiliation:
From the Department of Pediatrics, Seoul National University Children's Hospital, Seoul
*
Dr. Jung Yun Choi, Department of Pediatrics, Seoul National University Children's Hospital, 28 Yongon-Dong, Chongno-gu, Seoul,110-744, Korea. Tel. 82-2-760-3570; Fax. 82-2-741-0546.
Get access Rights & Permissions [Opens in a new window]

Abstract

We studied 52 children in order to assess the prevalence, natural history, and electrophysiologic effects of cardiac rhabdomyoma in tuberous sclerosis. Their ages ranged from one day to 12 years (median age 1.33 year). The tumors in the heart were found in 22 patients through cross-sectional echocardiography. The overall prevalence of cardiac rhabdomyoma was 42%. The prevalence of cardiac tumors in patients first seen in infancy was 77%, and that in patients over one year of age was 31%. Those with cardiac tumors diagnosed in infancy had more tumors per person than did the patients diagnosed after infancy, and the tumors were smaller in the patients diagnosed over one year of age. Follow-up cross-sectional echocardiography showed that almost all tumors showed spontaneous regression. Complete regression occurred more commonly in younger patients, and in those with smaller tumors. About 70% of patients with cardiac tumors had either normal findings or infrequent premature contractions on 24hour Holter monitorings. The rest of the patients with cardiac tumors showed either frequent premature contractions, atrioventricular block, or atrial tachycardia, but antiarrhythmic medication was rarely required. Cross-sectional echocardiography, therefore, seems to be the best diagnostic tool for screening cardiac rhabdomyomas in patients with tuberous sclerosis. The tumors may be managed more conservatively in view of their high rate of spontaneous regression and rather rare association with symptomatic arrhythmias.

Keywords

Cardiac rhabdomyomatuberous sclerosischildhood
Type
Original Articles
Information
Cardiology in the Young , Volume 5 , Issue 2 , April 1995 , pp. 166 - 171
Copyright
Copyright © Cambridge University Press 1995

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Roach, ES.Neurocutaneous syndromes. Ped Clin North Amer 1992; 39: 591–620.CrossRefGoogle ScholarPubMed
Bass, JL, Breningstall, GN, Swaimam, KF.Echocardiographic incidence of cardiac rhabdomyoma in tuberous sclerosis. Am J Cardiol 1985; 55: 1379–1382.CrossRefGoogle ScholarPubMed
Smith, HC, Watson, GH, Patel, RG, Super, M.Cardiac rhabdomyomata in tuberous sclerosis; their course and diagnostic value. Arch D Child 1989; 64: 196–200.CrossRefGoogle ScholarPubMed
Fischer, DR, Beerman, LB, Park, SC, Bahnson, HT, Fricker, FJ, Mathews, RA.Diagnosis of intracardiac rhabdomyoma by two-dimensional echocardiography. Am J Cardiol 1984; 53: 978–979.CrossRefGoogle ScholarPubMed
Nadas, AS, Ellison, RC.Cardiac tumors in infancy. Am J Cardiol 1968; 21: 363–366.CrossRefGoogle ScholarPubMed
Van der Hauwaert, LG.Cardiac tumors in infancy and childhood. Br Heart J 1971; 33: 125–132.CrossRefGoogle Scholar
Shaher, RM, Mintzer, J, Farina, M, Alley, R, Bishop, M.Clinical presentation of rhabdomyoma of the heart in infancy and childhood. Am J Cardiol 1972; 30: 95–103.CrossRefGoogle ScholarPubMed
Engle, MA, Ebert, PA, Redo, SF.Recurrent ventricular tachycardia due to resectable cardiac tumor, Report of two cases in two-years-old in heart failure. Circulation 1974; 50: 1052–1057.CrossRefGoogle Scholar
Mair, DD, Edwards, WD, Seward, JB. Cardiac manifestations. In: Gomez, MR (ed). Tuberous Sclerosis. Second edition. Raven Press Ltd, New York, 1988, pp 147–158.Google Scholar
Guereta, LG, Burgueros, M, Elorza, MD, Alix, AG, Benito, F, Gamallo, C.Cardiac rhabdomyoma presenting as fetal hydrops. Pediatr Cardiol 1986; 7: 171–74.CrossRefGoogle ScholarPubMed
Webb, DW, Thomas, RD, Osborne, JP.Cardiac rhabdomyomas and their association with tuberous sclerosis. Arch Dis Child 1993; 68: 367–370.CrossRefGoogle ScholarPubMed
Fenoglio, JJ Jr, McAllister, HA, Ferrans, VJ.Cardiac rhabdomyoma, A clinicopathologic and electron microscopicstudy. Am J Cardiol 1976; 38: 241–251.CrossRefGoogle Scholar
Alkalay, AL, Ferry, DA, Lin, B, Fink, BW, Pomerance, JJ.Spontaneous regression of cardiac rhabdomyoma in tuberous sclerosis. Clin Pediatrics 1987; 26: 532–535.CrossRefGoogle ScholarPubMed
Symthe, JF, Dyck, JD, Smallhorn, JF, Freedom, RM. Natural history of cardiac rhabdomyoma in infancy and childhood. Am J Cardiol 1990; 66: 1247–1249.CrossRefGoogle Scholar
Farooki, ZQ, Ross, RD, Paridon, SM, Humes, RA, Karpawich, PP, Pinsky, WW.Spontaneous regression of cardiac rhabdomyoma. Am J Cardiol 1991; 67: 897–899.CrossRefGoogle ScholarPubMed
Taylor, TR.Tuberous sclerosis presenting as cardiac arrhythmia. Br Heart J 1968; 30: 132–134.CrossRefGoogle ScholarPubMed
Gotlieb, Al, Chan, M, Palmer, WH, Huang, S.Ventricular preexcitation syndrome: Accessory left atrioventricular connection and rhabdomyomatous myocardial fibers. Arch Pathol Lab Med 1977; 101:486–489.Google ScholarPubMed
Jayakar, PB, Stanwick, RS, Seshia, SS.Tuberous sclerosis and Wolff-Parkinson-White syndrome. J Pediatr 1986; 108: 259–260.CrossRefGoogle ScholarPubMed
Manetti, A, De Simone, L, Favilli, S, Lini, M, Buti, D, Borgheresi, S, Dolara, A.Echocardiographic study of heart rhabdomyorma in tuberous sclerosis. Giornale Italiano di Cardiologia 1989; 9: 319–323.Google Scholar
Gibbs, JL.The heart and tuberous sclerosis; An echocardio graphic and electrocardiographic study. Br Heart J 1985; 54: 596–599.CrossRefGoogle Scholar
Hagood, CO, Garvin, DD, Lachina, FM, Polsky, WS, Ball, TP, Bobroff, LM.Abdominal aortic aneurysm and renal hamartoma in an infant with tuberous sclerosis. Surgery 1976; 79: 713–715.Google Scholar
Biancaniello, TM, Meyer, RA, Gaum, WE, Kaplan, S.Primary benign intramural ventricular tumors in children. Pre- and postoperative electrocardiographic, echocardiographic, and angiocardiographic evaluation. Am Heart J 1982; 103: 852–857.CrossRefGoogle ScholarPubMed
Marx, GR, Bierman, FZ, Matthews, E, Williams, R.Two- dimensional echocardiographic diagnosis of intracardiac masses in infancy. J Am Coll Cardiol 1984; 3: 827–832.CrossRefGoogle ScholarPubMed
Shepherd, CW, Gomez, MR, Lie, JT, Crowson, CS.Causes of death in patients with tuberous sclerosis. Mayo Clin Proc 1991; 66: 792–796.CrossRefGoogle ScholarPubMed
Matsuoka, Y, Nakati, T, Kawaguchi, K, Hayakawa, K.Disappearance of cardiac rhabdomyoma complicating congenital mitral regurgitation as observed by two-dimensional echocar diography. Pediatr Cardiol 1990; 11: 98–101.CrossRefGoogle Scholar
Bini, RM, Westaby, S, Bargeron, LM, Pacifico, AD, Kirklin, JW.Investigation and management of primary cardiac tumors in infants and children. J Am Coll Cardiol 1983; 2: 351–357.CrossRefGoogle ScholarPubMed
Arciniegas, E, Hakimi, M, Farooki, ZQ, Truccone, NJ, Green, EW.Primary cardiac tumors in children. J Thorac Cardiovasc Surg 1980; 79: 582–591.CrossRefGoogle ScholarPubMed
Bertolini, P, Meisner, H, Paek, SU, Sebening, F.Special consideration on primary cardiac tumors in infancy and childhood. Thorac Cardiovasc Surg 1990; 38(Suppl 2yes): 164–167.CrossRefGoogle ScholarPubMed