Zeitschrift für Kardiologie

, Volume 90, Supplement 6, pp 105–111 | Cite as

Marfan-Syndrom und Herzklappenerkrankungen

Article

Zusammenfassung

Das Marfan-Syndrom ist eine erbliche Bindegewebserkrankung, die mit einer erhöhten Inzidenz von kardiovaskulären Komplikationen verbunden ist. Wir beschreiben unsere Erfahrungen bei der chirurgischen Behandlung von 243 Patienten mit Marfan-Syndrom. Bezüglich der Behandlung der anuloaortalen Ektasie zeigen wir unsere Ergebnisse bei der Verwendung der klassischen Operationsmethoden nach BentallDeBono und Cabrol. Neure Operationsmethoden wie die Verfahren nach David und Yacoub werden kritisch gewertet und eine eigene Modifikation zum Klappen erhaltenden Ersatz der Aorta ascendens wird vorgestellt.

Bei einer großen Zahl von Patienten waren Operationen wegen der Manifestetion des Marfan-Syndroms im Bereich der distal der Aorta ascendens gelegenen Aortensegmente, der Mitralklappe und der Trikuspidalklappe erforderlich. Die Indikation zur Rekonstruktion bzw. zum Ersatz der Mitralklappe wird weitgehend entsprechend den klassischen Kriterien gestellt. Die Langzeitresultate des Erhalts der nativen Aortenklappe sowie der Mitralklappenrekonstruktion sind noch nicht vorhanden.

Schlüsselwörter

Marfan-Syndrom kardiovaskuläre Manifestationen chirurgische Behandlung 

Marfan syndrome and valvular disease

Summary

Marfan syndrome is a hereditary disease of the connective tissue with increased mortality mostly due to changes of the cardiovascular system. We describe our experience with the surgical treatment of 243 patients with Marfan syndrome and cardiovascular complications. We report the results of treatment of annulo-aortal ectasia using the classical surgical methods of Bentall DeBono and Cabrol and the method for preservation of the native aortic valve and concomitant surgery of the mitral valve. Reconstruction or replacement of the mitral valve is mainly based on the classical indications. Preservation of the native aortic valve and reconstruction of the mitral valve in patients with Marfan syndrome is possible but the long-term results are still unknown.

Key words

Marfan syndrome cardiovascular manifestations surgical treatment 

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Literatur

  1. 1.
    Beighton P, de Paepe A, Danks D et al. (1988) International nosology of heritable disorders of connective tissue, Berlin, 1986. Am J med Genet 29: 581–594PubMedCrossRefGoogle Scholar
  2. 2.
    Bentall H, DeBono A (1968) A technique for complete replacement of the ascending aorta. Thorax 23: 338–339PubMedCrossRefGoogle Scholar
  3. 3.
    Cabrol C Pavie A, Mesnildrey P et al. (1986) Long-term results with total replacement of the ascending aorta and reimplantation of the coronary arteries. J Thorac Cardiovasc surg 91: 17–25PubMedGoogle Scholar
  4. 4.
    Cosby IK, Ashcroft WC, Reed WA (1973) Surgery of proximal aorta in Marfan’s syndrome. J Thorac Cardiovasc Surg 66: 75–81Google Scholar
  5. 5.
    Crawford ES (1983) Marfan’s syndrome. Broad spectral surgical treatment cardiovascular manifestations. Ann Surg 198: 487–505PubMedCrossRefGoogle Scholar
  6. 6.
    David TE (1993) When, why, and how should the native aortic valve be preserved in patients with annuloaortic ectasia or Marfan syndrome? Sem Thorac Cardiovasc Surg 5: 93–96Google Scholar
  7. 7.
    David TE, Feindel CM, Bos J (1995) Repair of the aortic valve in patients with aortic insufficiency and aortic root aneurysm. J Thorac Cardiovasc Surg 109: 345–352PubMedCrossRefGoogle Scholar
  8. 8.
    De Paepe A, Devereux RB, Dietz HC, Hennekam RCM, Pyeritz RE (1996) Revised diagnostic criteria for the Marfan syndrome. Am J med Genet 62: 417–426PubMedCrossRefGoogle Scholar
  9. 9.
    Habbal MHE (1992) Cardiovascular manifestations of Marfan’s syndrome in the young. Am Heart J 23: 253–257Google Scholar
  10. 10.
    Hirata K, Triposkiadis F, Sparks E et al. (1992) The Marfan sindrome: abnormal aortic elastic properties. J Am Coll Cardiol 18: 57–63CrossRefGoogle Scholar
  11. 11.
    Kainulainen K, Pulkinen L, Savolainen et al. (1990) Location on chromosome 15 of the gene defect causing Marfan syndrome. N Engl J med 323: 935–939PubMedCrossRefGoogle Scholar
  12. 12.
    Kouchoukos NT (1991) Composite graft replacement of the ascending aorta and aortic valve with the inclusion-wrap and open techniques. Semin Thorac Cardiovasc Surg 3: 171–176PubMedGoogle Scholar
  13. 13.
    Murdoch JL, Walker BA, Halpern BA, Kuzma JW, McKussick VA (1972) Life expectancy and causes of deth in the Marfan syndrome. N Engl J Med 286: 804–808PubMedCrossRefGoogle Scholar
  14. 14.
    Pasic M, von Segesser L, Carrel T et al. (1992) Surgical treatment of cardiovascular complications in Marfan syndrome. Eur J Cardiothorac surg 6: 149–155PubMedCrossRefGoogle Scholar
  15. 15.
    Pyeritz RE (1990) Editorial: Marfan syndrome. N Engl J Med 323: 987–989PubMedCrossRefGoogle Scholar
  16. 16.
    Pyeritz RE, Wappel MA (1983) Mitral valve dysfunction in the Marfan syndrome. Am J Med 74: 797–807PubMedCrossRefGoogle Scholar
  17. 17.
    Pyeritz RE, McKusick VA (1979) The Marfan syndrome: diagnosis and management. N Engl J Med 300: 772–777PubMedCrossRefGoogle Scholar
  18. 18.
    Sarsam MA, Yacoub M (1993) Remodeling of the aortic valve anulus. J Thorac Cardiovasc Surg 105: 435–438PubMedGoogle Scholar
  19. 19.
    Svensson LG (1992) Approach to the insertion of composite valve graft. Ann Thorac Surg 54: 376–378PubMedCrossRefGoogle Scholar
  20. 20.
    Svensson LG, Crawford ES, Coselli JS, Safi HJ, Hess KR (1989) Impact of cardiovascular operation on survival in the Marfan patient. Circulation 80: 233–242Google Scholar
  21. 21.
    Svensson LG, Crawford ES, Hess KR et al. (1992) Composite valve graft replacement of the proximal aorta: comparison of techniques in 348 patients. Ann Thorac Surg 54: 427–439PubMedCrossRefGoogle Scholar
  22. 22.
    Symbas PN, Raizner AE, Tyros DH et al. (1971) Aneurysms of all sinuses of Valsalva in patients with Marfan’syndrome. An unusual late complication following replacement of aortic valve and ascending aorta for aortic regurgitation and fusiform aneurysm of ascending aorta Ann Surg 174: 902–907PubMedCrossRefGoogle Scholar
  23. 23.
    Trotter SE, Olsen EG (1991) Marfan’s disease and Erdheim’s cystic medionecrosis: a study of their pathology. Eur heart J 12: 83–87PubMedCrossRefGoogle Scholar

Copyright information

© Steinkopff Verlag 2001

Authors and Affiliations

  1. 1.Deutsches Herzzentrum BerlinKlinik für Herz-, Thorax- und GefäßchirurgieBerlinGermany

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