Skip to main content
PMC home page
Heart logoLink to Heart
. 1997 Jul;78(1):83–87. doi: 10.1136/hrt.78.1.83

Congenital quadricuspid aortic valve anomaly associated with hypertrophic non-obstructive cardiomyopathy: a case report and review of the literature.

U Janssens 1, H G Klues 1, P Hanrath 1
PMCID: PMC484869  PMID: 9290407

Abstract

A case is reported of a 38 year old woman without known cardiac congenital abnormality but a history of well controlled arterial hypertension who was admitted to hospital after successful resuscitation at home following cardiac arrest. There was no evidence of myocardial infarction on 12-lead electrocardiogram but there were signs of left ventricular hypertrophy. Transoesophageal echocardiography revealed a rare quadricuspid aortic valve (QAV) malformation with concomitant mild aortic regurgitation. The left ventricle showed a massive concentric hypertrophy without obstruction. The patient was eventually transferred in a persistent vegetative state to a home care facility. A review of the literature revealed 70 cases of QAV diagnosed by transthoracic or transoesophageal echocardiography (26 cases), at necropsy (25), during surgery (15), and during angiography (4). The present case is the first report of QAV associated with non-obstructive hypertrophic cardiomyopathy.

Full text

PDF
83

Images in this article

84Image
on p.84
87Image
on p.87

Selected References

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

  1. Amioka H., Okamoto M., Matsumoto K., Yamagata T., Yokote Y., Sakura E., Hashimoto M., Tsuchioka Y., Matsuura H., Kajiyama G. [Echo and Doppler cardiographic findings of isolated quadricuspid aortic valve: a case report and a review of the literature]. J Cardiogr. 1986 Dec;16(4):1003–1011. [PubMed] [Google Scholar]
  2. Aoyagi S., Kawara T., Yasunaga H., Kosuga K., Oishi K. Congenital quadricuspid aortic valve associated with aortic regurgitation. Thorac Cardiovasc Surg. 1992 Aug;40(4):225–226. doi: 10.1055/s-2007-1020156. [DOI] [PubMed] [Google Scholar]
  3. Barbosa M. M., Motta M. S. Quadricuspid aortic valve and aortic regurgitation diagnosed by Doppler echocardiography: report of two cases and review of the literature. J Am Soc Echocardiogr. 1991 Jan-Feb;4(1):69–74. doi: 10.1016/s0894-7317(14)80163-6. [DOI] [PubMed] [Google Scholar]
  4. Brouwer M. H., de Graaf J. J., Ebels T. Congenital quadricuspid aortic valve. Int J Cardiol. 1993 Feb;38(2):196–198. doi: 10.1016/0167-5273(93)90182-g. [DOI] [PubMed] [Google Scholar]
  5. Chamsi-Pasha H., Barnes P. C., Beton D. C. Echocardiographic diagnosis of quadricuspid aortic valve. Eur Heart J. 1988 Nov;9(11):1269–1270. doi: 10.1093/oxfordjournals.eurheartj.a062440. [DOI] [PubMed] [Google Scholar]
  6. Chandrasekaran K., Tajik A. J., Edwards W. D., Seward J. B. Two-dimensional echocardiographic diagnosis of quadricuspid aortic valve. Am J Cardiol. 1984 Jun 1;53(11):1732–1733. doi: 10.1016/0002-9149(84)90617-9. [DOI] [PubMed] [Google Scholar]
  7. Coeurderoy A., Biron Y., Laurent M., Almange C. Quadricuspidie aortique congénitale. A propos d'un cas. Revue de la littérature. Arch Mal Coeur Vaiss. 1986 May;79(5):745–748. [PubMed] [Google Scholar]
  8. Costa-Martorell J., Rivas-Puy I., Peraire J. J., Bardají A., Ridao C., Richart C. Válvula aórtica cuadricúspide. Presentación de un caso y revisión de la literatura. Rev Esp Cardiol. 1992 Nov;45(9):608–610. [PubMed] [Google Scholar]
  9. Cruz L. A., Castier M. B., Ferro M. E., Menezes C. C., Albanesi Filho F. M. Insuficiência aórtica por valva aórtica quadricúspide. Arq Bras Cardiol. 1993 Feb;60(2):103–105. [PubMed] [Google Scholar]
  10. Davia J. E., Fenoglio J. J., DeCastro C. M., McAllister H. A., Jr, Cheitlin M. D. Quadricuspid semilunar valves. Chest. 1977 Aug;72(2):186–189. doi: 10.1378/chest.72.2.186. [DOI] [PubMed] [Google Scholar]
  11. Feizi O., Farrer Brown G., Emanuel R. Familial study of hypertrophic cardiomyopathy and congenital aortic valve disease. Am J Cardiol. 1978 May 1;41(5):956–964. doi: 10.1016/0002-9149(78)90740-3. [DOI] [PubMed] [Google Scholar]
  12. Feldman B. J., Khandheria B. K., Warnes C. A., Seward J. B., Taylor C. L., Tajik A. J. Incidence, description and functional assessment of isolated quadricuspid aortic valves. Am J Cardiol. 1990 Apr 1;65(13):937–938. doi: 10.1016/0002-9149(90)91446-d. [DOI] [PubMed] [Google Scholar]
  13. Fernicola D. J., Mann J. M., Roberts W. C. Congenitally quadricuspid aortic valve: analysis of six necropsy patients. Am J Cardiol. 1989 Jan 1;63(1):136–138. doi: 10.1016/0002-9149(89)91102-8. [DOI] [PubMed] [Google Scholar]
  14. Fischler D., Fitzmaurice M., Ratliff N. B., Jr Quadricuspid aortic valve. Am J Cardiovasc Pathol. 1990;3(1):91–94. [PubMed] [Google Scholar]
  15. Hurwitz L. E., Roberts W. C. Quadricuspid semilunar valve. Am J Cardiol. 1973 May;31(5):623–626. doi: 10.1016/0002-9149(73)90332-9. [DOI] [PubMed] [Google Scholar]
  16. Iglesias A., Oliver J., Muñoz J. E., Nuñez L. Quadricuspid aortic valve associated with fibromuscular subaortic stenosis and aortic regurgitation treated by conservative surgery. Chest. 1981 Sep;80(3):327–328. doi: 10.1378/chest.80.3.327. [DOI] [PubMed] [Google Scholar]
  17. Irisawa T., Yoshiya K., Yokosawa T., Iwamatsu T., Arai K., Aoki T. [A case of quadricuspid aortic valve associated with mitral regurgitation]. Kyobu Geka. 1993 Jul;46(7):618–621. [PubMed] [Google Scholar]
  18. Kim H. S., McBride R. A., Titus J. L. Quadricuspid aortic valve and single coronary ostium. Arch Pathol Lab Med. 1988 Aug;112(8):842–844. [PubMed] [Google Scholar]
  19. Koizumi S., Arai S., Kato T. [A case of quadricuspid aortic valve with aortic regurgitation]. Kyobu Geka. 1990 Jun;43(6):505–508. [PubMed] [Google Scholar]
  20. Kosach G. A., Evzhanov A., Serbin V. I., Zubkova G. A., Tadzhibaeva Z. A. Chetyrekhstvorchatyi aortal'nyi klapan. Kardiologiia. 1982 Apr;22(4):113–114. [PubMed] [Google Scholar]
  21. Kurosawa H., Wagenaar S. S., Becker A. E. Sudden death in a youth. A case of quadricuspid aortic valve with isolation of origin of left coronary artery. Br Heart J. 1981 Aug;46(2):211–215. doi: 10.1136/hrt.46.2.211. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Lai C. P., Koyanagi S., Sadoshima J., Takeshita A., Tokunaga K. Transesophageal echocardiographic findings of quadricuspid aortic valve. Jpn Heart J. 1991 Sep;32(5):731–734. doi: 10.1536/ihj.32.731. [DOI] [PubMed] [Google Scholar]
  23. Lai C. P., Koyanagi S., Sadoshima J., Takeshita A., Tokunaga K. Transesophageal echocardiographic findings of quadricuspid aortic valve. Jpn Heart J. 1991 Sep;32(5):731–734. doi: 10.1536/ihj.32.731. [DOI] [PubMed] [Google Scholar]
  24. Lanzillo G., Breccia P. A., Intonti F. Congenital quadricuspid aortic valve with displacement of the right coronary orifice. Scand J Thorac Cardiovasc Surg. 1981;15(2):149–151. doi: 10.3109/14017438109101040. [DOI] [PubMed] [Google Scholar]
  25. Lim Y. T., Choo M. H., Ling L. H. Echocardiographic diagnosis of quadricuspid aortic valve. Ann Acad Med Singapore. 1992 Nov;21(6):841–842. [PubMed] [Google Scholar]
  26. Luisi V. S., Pasqué A., Verunelli F., Aliboni M., Urbano U., Reginato E. Quadricuspid aortic valve. J Cardiovasc Surg (Torino) 1984 May-Jun;25(3):252–254. [PubMed] [Google Scholar]
  27. Mathison M., Furuse A., Kotsuda H., Tanaka O., Chikada M. [Quadricuspid aortic valve: case reports]. Kyobu Geka. 1990 May;43(5):383–388. [PubMed] [Google Scholar]
  28. Matsui K., Kohno H., Kurisu K., Murakami S., Fukuyama T. Quadricuspid aortic valve--report of a case associated with severe aortic regurgitation and review of the literature. Jpn J Surg. 1989 Jan;19(1):93–97. doi: 10.1007/BF02471575. [DOI] [PubMed] [Google Scholar]
  29. Matsukawa T., Yoshii S., Hashimoto R., Muto S., Suzuki S., Ueno A. Quadricuspid aortic valve perforation resulting from bacterial endocarditis--2-D echo- and angiographic diagnosis and its surgical treatment. Jpn Circ J. 1988 May;52(5):437–440. doi: 10.1253/jcj.52.437. [DOI] [PubMed] [Google Scholar]
  30. Matsumoto M., Miki S., Kusuhara K., Ueda Y., Ohkita Y., Tahata T., Komeda M. Quadricuspid aortic valve associated with severe aortic regurgitation. Jpn Circ J. 1985 Feb;49(2):190–191. doi: 10.1253/jcj.49.190. [DOI] [PubMed] [Google Scholar]
  31. McRonald R. E., Dean D. C. Congenital quadricuspid aortic valve. Am J Cardiol. 1966 Nov;18(5):761–763. doi: 10.1016/0002-9149(66)90095-6. [DOI] [PubMed] [Google Scholar]
  32. Nalbantgil I., Cagatay G. Letter: Quadricuspid aortic valve. Chest. 1975 May;67(5):623–624. doi: 10.1378/chest.67.5.623. [DOI] [PubMed] [Google Scholar]
  33. Peretz D. I., Changfoot G. H., Gourlay R. H. Four-cusped aortic valve with significant hemodynamic abnormality. Am J Cardiol. 1969 Feb;23(2):291–293. doi: 10.1016/0002-9149(69)90081-2. [DOI] [PubMed] [Google Scholar]
  34. Roberts W. C. The congenitally bicuspid aortic valve. A study of 85 autopsy cases. Am J Cardiol. 1970 Jul;26(1):72–83. doi: 10.1016/0002-9149(70)90761-7. [DOI] [PubMed] [Google Scholar]
  35. Robicsek F., Sanger P. W., Daugherty H. K., Montgomery C. C. Congenital quadricuspid aortic valve with displacement of the left coronary orifrice. Am J Cardiol. 1969 Feb;23(2):288–290. doi: 10.1016/0002-9149(69)90080-0. [DOI] [PubMed] [Google Scholar]
  36. Shinkai M., Okazaki T., Fujiwara H., Nasu M., Sono J., Okada Y., Miyamoto S., Nishiuchi S., Shomura T. [An operative case of aortic regurgitation due to quadricuspid valve]. Kyobu Geka. 1994 Apr;47(4):311–314. [PubMed] [Google Scholar]
  37. Shioi K., Nagata Y., Kato S., Kato R., Tsuchioka H. [A case of quadricuspid aortic valve associated with coronary arterial legion]. Nihon Kyobu Geka Gakkai Zasshi. 1991 Jul;39(7):1072–1076. [PubMed] [Google Scholar]
  38. Sievers H. H., Regensburger D., Bernhard A. Quadricuspid aortic valve with significant insufficiency. Thorac Cardiovasc Surg. 1982 Feb;30(1):44–45. doi: 10.1055/s-2007-1022206. [DOI] [PubMed] [Google Scholar]
  39. Stassano P., Iorio D., Di Lello F., Tecchia L. B., Spampinato N. Insufficienza aortica da valvola quadricuspide. Minerva Cardioangiol. 1982 Jan-Feb;30(1-2):7–9. [PubMed] [Google Scholar]
  40. Subramanian R., Olson L. J., Edwards W. D. Surgical pathology of combined aortic stenosis and insufficiency: a study of 213 cases. Mayo Clin Proc. 1985 Apr;60(4):247–254. doi: 10.1016/s0025-6196(12)60317-6. [DOI] [PubMed] [Google Scholar]
  41. Suda H., Rikitake K., Furukawa K., Shiraishi R., Kurashige Y., Mori E., Itoh T. [Quadricuspid aortic valve: a case report and the review of the literature]. Kyobu Geka. 1993 Mar;46(3):283–286. [PubMed] [Google Scholar]
  42. Waller B. F., Taliercio C. P., Dickos D. K., Howard J., Adlam J. H., Jolly W. Rare or unusual causes of chronic, isolated, pure aortic regurgitation. Clin Cardiol. 1990 Aug;13(8):577–581. doi: 10.1002/clc.4960130812. [DOI] [PubMed] [Google Scholar]

Articles from Heart are provided here courtesy of BMJ Publishing Group

RESOURCES