Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1989 Feb;64(2):196–200. doi: 10.1136/adc.64.2.196

Cardiac rhabdomyomata in tuberous sclerosis: their course and diagnostic value.

H C Smith 1, G H Watson 1, R G Patel 1, M Super 1
PMCID: PMC1791849  PMID: 2930225

Abstract

Echocardiography was performed in 60 people with tuberous sclerosis to ascertain the prevalence and course of cardiac rhabdomyomata at different ages. Twenty five (58%) of 43 children had tumours, but only three (18%) of 17 adults. The tumours tended to remain the same size through childhood. Only three infants were included but evidence from this and some published case reports suggest that the tumours tend to regress in early infancy and again in adolescence. The prevalence of tumours in young infants with tuberous sclerosis is likely to be considerably above 50%. As other signs of tuberous sclerosis are usually absent at this age echocardiography may afford the most useful diagnostic test in early infancy.

Full text

PDF
200

Images in this article

197Fig 1
on p.197
197Fig 2
on p.197
198Fig 3
on p.198
198Fig 4
on p.198
198Fig 5
on p.198
198Fig 6
on p.198

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bass J. L., Breningstall G. N., Swaiman K. F. Echocardiographic incidence of cardiac rhabdomyoma in tuberous sclerosis. Am J Cardiol. 1985 May 1;55(11):1379–1382. doi: 10.1016/0002-9149(85)90508-9. [DOI] [PubMed] [Google Scholar]
  2. Casta A. Tuberous sclerosis and Wolff-Parkinson-White syndrome. J Pediatr. 1986 Aug;109(2):399–400. doi: 10.1016/s0022-3476(86)80432-2. [DOI] [PubMed] [Google Scholar]
  3. Fenoglio J. J., Jr, MCAllister H. A., Jr, Ferrans V. J. Cardiac rhabdomyoma: a clinicopathologic and electron microscopic study. Am J Cardiol. 1976 Aug;38(2):241–251. doi: 10.1016/0002-9149(76)90157-0. [DOI] [PubMed] [Google Scholar]
  4. Foster E. D., Spooner E. W., Farina M. A., Shaher R. M., Alley R. D. Cardiac rhabdomyoma in the neonate: surgical management. Ann Thorac Surg. 1984 Mar;37(3):249–253. doi: 10.1016/s0003-4975(10)60334-7. [DOI] [PubMed] [Google Scholar]
  5. Fryer A. E., Chalmers A., Connor J. M., Fraser I., Povey S., Yates A. D., Yates J. R., Osborne J. P. Evidence that the gene for tuberous sclerosis is on chromosome 9. Lancet. 1987 Mar 21;1(8534):659–661. doi: 10.1016/s0140-6736(87)90416-8. [DOI] [PubMed] [Google Scholar]
  6. Gibbs J. L. The heart and tuberous sclerosis. An echocardiographic and electrocardiographic study. Br Heart J. 1985 Dec;54(6):596–599. doi: 10.1136/hrt.54.6.596. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Gillor A., Stock G., Schuster D., Crespo E., Schickendantz S., Mennicken U. Diagnostik und Behandlung der kardialen Rhabdomyome. Monatsschr Kinderheilkd. 1986 Jul;134(7):445–449. [PubMed] [Google Scholar]
  8. Gresser C. D., Shime J., Rakowski H., Smallhorn J. F., Hui A., Berg J. J. Fetal cardiac tumor: a prenatal echocardiographic marker for tuberous sclerosis. Am J Obstet Gynecol. 1987 Mar;156(3):689–690. doi: 10.1016/0002-9378(87)90079-2. [DOI] [PubMed] [Google Scholar]
  9. Journel H., Roussey M., Plais M. H., Milon J., Almange C., Le Marec B. Prenatal diagnosis of familial tuberous sclerosis following detection of cardiac rhabdomyoma by ultrasound. Prenat Diagn. 1986 Jul-Aug;6(4):283–289. doi: 10.1002/pd.1970060408. [DOI] [PubMed] [Google Scholar]
  10. Khattar H., Guerin R., Fouron J. C., Stanley P., Kratz C., Davignon A. Les tumeurs cardiaques chez l'enfant. Rapport de 3 observations avec évolution spontanément favorable. Arch Mal Coeur Vaiss. 1975 Apr;68(4):419–429. [PubMed] [Google Scholar]
  11. Marx G. R., Bierman F. Z., Matthews E., Williams R. Two-dimensional echocardiographic diagnosis of intracardiac masses in infancy. J Am Coll Cardiol. 1984 Mar;3(3):827–832. doi: 10.1016/s0735-1097(84)80260-0. [DOI] [PubMed] [Google Scholar]
  12. Muller L., de Jong G., Falck V., Hewlett R., Hunter J., Shires J. Antenatal ultrasonographic findings in tuberous sclerosis. Report of 2 cases. S Afr Med J. 1986 May 10;69(10):633–638. [PubMed] [Google Scholar]
  13. Pampiglione G., Pugh E. Letter: Infantile spasms and subsequent appearance of tuberous sclerosis syndrome. Lancet. 1975 Nov 22;2(7943):1046–1046. doi: 10.1016/s0140-6736(75)90343-8. [DOI] [PubMed] [Google Scholar]
  14. Thibault J. H., Manuelidis E. E. Tuberous sclerosis in a premature infant. Report of a case and review of the literature. Neurology. 1970 Feb;20(2):139–146. doi: 10.1212/wnl.20.2.139. [DOI] [PubMed] [Google Scholar]
  15. Wilson R. D., Hall J. G., McGillivray B. C. Tuberous sclerosis: case report and investigation of family members. Can Med Assoc J. 1985 Apr 1;132(7):807–809. [PMC free article] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES