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Electrocardiographic changes in patients with cardiac rhabdomyomas associated with tuberous sclerosis

Published online by Cambridge University Press:  24 May 2005

Junko Shiono ,
Hitoshi Horigome ,
Seiyo Yasui ,
Tomoyuki Miyamoto ,
Miho Takahashi-Igari ,
Nobuaki Iwasaki  and
Akira Matsui
Junko Shiono
Affiliation:
Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan
Hitoshi Horigome
Affiliation:
Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan
Seiyo Yasui
Affiliation:
Department of Cardiology, Kanagawa Children's Medical Center, Mutsukawa, Yokohama, Japan
Tomoyuki Miyamoto
Affiliation:
Department of Cardiology, Kanagawa Children's Medical Center, Mutsukawa, Yokohama, Japan
Miho Takahashi-Igari
Affiliation:
Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan
Nobuaki Iwasaki
Affiliation:
Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan
Akira Matsui
Affiliation:
Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan
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Abstract

Background: Cardiac rhabdomyomas associated with tuberous sclerosis induce various abnormalities in the electrocardiogram. Electrocardiographic evidence of ventricular hypertrophy may appear if the tumour is electrically active. To our knowledge, electrocardiographic evidence of ventricular hypertrophy has been reported only in association with congestive heart failure. Follow-up studies of changes in electrocardiographic findings are also lacking. Methods: We studied 21 consecutive patients with cardiac rhabdomyoma associated with tuberous sclerosis, 10 males and 11 females, aged from the date of birth to 9 years at diagnosis. The mean period of follow-up was 53 months. None of the patients developed congestive heart failure. We evaluated the electrocardiographic changes during the follow-up, and their association with echocardiographic findings. Results: Of the 21 patients, 12 showed one or more abnormalities on the electrocardiogram at presentation, with five demonstrating right or left ventricular hypertrophy. In all of these five cases, the tumours were mainly located in the respective ventricular cavity. In one patient with a giant tumour expanding exteriorly, there was marked left ventricular hypertrophy on the electrocardiogram. Followup studies showed spontaneous regression of the tumours in 12 of 19 patients, with abnormalities still present in only 7 patients. A gradual disappearance of left ventricular hypertrophy as seen on the electrocardiogram was noted in the patient with marked left ventricular hypertrophy at presentation in parallel with regression of the tumour. Conclusions: The presence of cardiac rhabdomyomas in patients with tuberous sclerosis might explain the ventricular hypertrophy seen on the electrocardiogram through its electrically active tissue without ventricular pressure overload or ventricular enlargement, although pre-excitation might affect the amplitude of the QRS complex. Even in cases with large tumours, nonetheless, the electric potential might not alter the surface electrocardiogram if the direction of growth of the tumour is towards the ventricular cavity. In many cases, electrocardiographic abnormalities tend to disappear, concomitant with regression of the tumours.

Keywords

Ventricular hypertrophytumoursventricular pre-excitation
Type
Original Article
Information
Cardiology in the Young , Volume 13 , Issue 3 , June 2003 , pp. 258 - 263
Copyright
© 2003 Cambridge University Press

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References

Longino LA, Meeker IA Jr. Primary cardiac tumors in infancy. J Pediatr 1953; 43: 724–731.Google Scholar
Nadas AS, Ellison RC. Cardiac tumors in infancy. Am J Cardiol 1968; 21: 363–366.Google Scholar
Freedom RM, Lee KJ, MacDonald C, Taylor G. Selected aspects of cardiac tumors in infancy and childhood. Pediatr Cardiol 2000; 21: 299–316.Google Scholar
Becker AE. Primary heart tumors in the pediatric age group: a review of salient pathologic features relevant for clinicians. Pediatr Cardiol 2000; 21: 317–323.Google Scholar
Fenoglio JJ Jr, McAllister HA Jr, Ferrans VJ. Cardiac rhabdomyoma: a clinicopathologic and electron microscopic study. Am J Cardiol 1976; 38: 241–251.Google Scholar
Bass JL, Breningstall GN, Swaiman KF. Echocardiographic incidence of cardiac rhabdomyoma in tuberous sclerosis. Am J Cardiol 1985; 55: 1379–1382.Google Scholar
Smith HC, Watson GH, Patel RG, Super M. Cardiac rhabdomyomata in tuberous sclerosis: their course and diagnostic value. Arch Dis Child 1989; 64: 196–200.Google Scholar
Bosi G, Lintermans JP, Pellegrino PA, Svaluto-Moreolo G, Vliers A. The natural history of cardiac rhabdomyoma with and without tuberous sclerosis. Acta Paediatr 1996; 85: 928–931.Google Scholar
Shepherd CW, Gomez MR, Lie JT, Crowson CS. Causes of death in patients with tuberous sclerosis. Mayo Clin Proc 1991; 66: 792–796.Google Scholar
Smythe JF, Dyck JD, Smallhorn JF, Freedom RM. Natural history of cardiac rhabdomyoma in infancy and childhood. Am J Cardiol 1990; 66: 1247–1249.Google 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.Google Scholar
Mühler EG, Turniski-Harder V, Engelhardt W, von Bernuth G. Cardiac involvement in tuberous sclerosis. Br Heart J 1994; 72: 584–590.Google Scholar
Cosnett JE, Gibb BH. Tuberous sclerosis and cardiac arrhythmia in three Zulu patients. Br Med J 1969; 2: 672–673.Google Scholar
Taylor TR. Tuberous sclerosis presenting as cardiac arrhythmia. Br Heart J 1968; 30: 132–134.Google Scholar
Lessick J, Schwartz Y, Lorber A. Neonatal advanced heart block due to cardiac tumor. Pediatr Cardiol 1998; 19: 263–265.Google Scholar
Cowley CG, Tani LY, Judd VE, Shaddy RE. Sinus node dysfunction in tuberous sclerosis. Pediatr Cardiol 1996; 17: 51–52.Google Scholar
O'Callaghan FJK, Clarke AC, Joffe H, et al. Tuberous sclerosis complex and Wolff-Parkinson-White syndrome. Arch Dis Child 1998; 78: 159–162.Google Scholar
Mehta AV. Rhabdomyoma and ventricular preexcitation syndrome. A report of two cases and review of literature. AJDC 1993; 147: 669–671.Google Scholar
Gomez MR. Criteria for diagnosis. In: Gomez MR (ed.). Tuberous Sclerosis, 2nd edn. Raven Press, New York, 1988, pp 89–109.
Massumi RA, Adkins PC, Reichelderfer TR, Fraga JR, Sampson R. Congenital rhabdomyoma of the heart. Presentation of two cases. J Thorac Cardiovasc Surg 1968; 55: 711–718.Google Scholar
Kuehl KS, Perry LW, Chandra R, Scott LP. Left ventricular rhabdomyoma: a rare cause of subaortic stenosis in the newborn infant. Pediatrics 1970; 46: 464–468.Google Scholar
Van der Hauwaert LG. Cardiac tumours in infancy and childhood. Br Heart J 1971; 33: 125–132.Google Scholar
Simcha A, Wells BG, Tynan MJ, Waterston DJ. Primary cardiac tumours in childhood. Arch Dis Child 1971; 46: 508–514.Google 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.Google Scholar
Shaher RM, Farina M, Alley R, Hansen P, Bishop M. Congenital subaortic stenosis in infancy caused by rhabdomyoma of the left ventricle. J Thorac Cardiovasc Surg 1972; 63: 157–163.Google Scholar
Tsakraklides V, Burke B, Mastri A, Runge W, Roe E, Anderson R. Rhabdomyomas of heart. Am J Dis Child 1974; 128: 639–646.Google Scholar
Goodwin JF. The spectrum of cardiac tumors. Am J Cardiol 1968; 21: 307–313.Google Scholar
Elliott GB, McGeachy WG. The monster Purkinje-cell nature of so-called “congenital rhabdomyoma of heart”: a forme fruste of tuberous sclerosis. Am Heart J 1962; 63: 636–643.Google Scholar