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Supravalvuläre Aortenstenose: Morphologie der Aortenklappe und deren Bedeutung für die chirurgische Therapie

  • C. Stamm
  • T. Busch
  • T. Felderhoff
  • Jia Li
  • R. H. Anderson
  • Siew Yen Ho
Originalarbeit
  • 27 Downloads

Zusammenfassung

Ein großer Teil der Patienten mit supravalvulärer Aortenstenose zeigt pathologische Veränderungen der Aortenklappe selbst. Eine ausgeprägte Redundanz der Klappensegel, hervorgerufen durch die verengte sinutubuläre Leiste als Aufhängepunkt derselben, kann zu frühzeitigen degenerativen Veränderungen der Klappensegel führen. Die sogenannte “supra”-valvuläre Stenose beeinträchtigt also stets Struktur und Funktion der gesamten Aortenwurzel. Ein korrektiver chirurgischer Eingriff sollte diesem Rechnung tragen, indem die Leiste über allen drei Sinus durchtrennt und adäquat erweitert wird.

Schlüsselwörter

Aortenklappe supravalvuläre Stenose angeborene Herzerkrankungen Morphologie 

Supravalvular aortic stenosis: Morphology and surgical importance of the aortic valve

Summary

Techniques of surgical repair of supravalvar aortic stenosis vary from simple enlargement using a single patch to more extensive procedures. In this review we describe the development of surgical therapy and elucidate the structural basis in the context of anatomical restoration of the aortic root. Thickened and rigid aortic valvar leaflets, as well as partial adhesion of the leaflets to the stenosing sinutubular ridge, were seen frequently, together with markedly abnormal dimensions of the aortic root. A marked redundancy of the free edge of the leaflets relative to the diameter of the sinutubular junction was observed in anatomic specimens. The entire valvar apparatus, therefore, is always affected in hearts with so-called supravalvular stenosis. We recommend that ideal surgical restoration of the aortic root should take account of all the anatomic components by enlarging all three sinuses of Valsalva at the sinutubular junction.

Key words

Aortic valve supravalvular stenosis congenital heart disease morphology 

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Literatur

  1. 1.
    Archer RS. Note on a congenital band stretching across the origin of the aorta. Dublin J Med Sci 1878; 65: 405Google Scholar
  2. 2.
    Beuren AJ, Apitz J, Harmjanz D (1962) Supravalvular aortic stenosis in association with mental retardation and a certain facial appearance. Circulation 26: 1235–40PubMedGoogle Scholar
  3. 3.
    Blieden LC, Lucas RV, Carter JB, Miller K, Edwards JE (1974) A development complex including supravalvular stenosis of the aorta and pulmonary trunk. Circulation 49: 585–90PubMedGoogle Scholar
  4. 4.
    Brewer RJ, Deck D, Capati B, Nolan SP (1976) The dynamic aortic root. Its role in aortic valve function. J Thorac Cardiovasc Surg 72: 413–17PubMedGoogle Scholar
  5. 5.
    Brom AG (1988) Obstruction of the left ventricular outflow tract. In: Khonsari S., ed.: Cardiac Surgery: Safeguards and Pitfalls in Operative Technique. Rockville, Mt: Aspen, 276: 80Google Scholar
  6. 6.
    Chard RB, Cartmill TB (1993) Localized supravalvar aortic stenosis: a new technique for repair. Ann Thorac Surg 55: 782–4PubMedGoogle Scholar
  7. 7.
    Chevers N (1842) Observations on the disease of the orifice and valves of the aorta. Guy's Hosp Rep 7: 387Google Scholar
  8. 8.
    Conway EE, Noonan J, Marion RW, Steeg CN (1990) Myocardial infarction leading to sudden death in the Williams syndrome: Report of three cases. J Pediatr 117: 593–6PubMedCrossRefGoogle Scholar
  9. 9.
    Debich DE, Williams KE, Anderson RH (1989) Congenital atresia of the orifice of the left coronary artery and its main stem. Int J Cardiol 22: 398–404PubMedCrossRefGoogle Scholar
  10. 10.
    Delius RE, Steinberg JB, L'Ecuyer T, Doty DB, Behrendt DM (1995) Longterm follow-up of extended aortoplasty for supravalvular aortic stenosis. J Thorac Cardiovasc Surg 109: 155–63PubMedCrossRefGoogle Scholar
  11. 11.
    Denie JJ, Verheugt AP (1958) Supravalvular aortic stenosis. Circulation 18: 902–8PubMedGoogle Scholar
  12. 12.
    Doty DB, Polansky DB, Jenson CB (1977) Supravalvular aortic stenosis. Repair by extended aortoplasty. J Thorac Cardiovasc Surg 74: 362–71PubMedGoogle Scholar
  13. 13.
    Doty DB (1991) Supravalvular aortic stenosi. Ann Thorac Surg 51: 886–7PubMedGoogle Scholar
  14. 14.
    Edwards JE (1965) Pathology of left ventricular outflow tract obstruction. Circulation 31: 586–99PubMedGoogle Scholar
  15. 15.
    Flaker G, Teske D, Kilman J, Hosier D, Wooley C (1983) Supravalvular aortic stenosis. A 20-year clinical perspective with patch aortoplasty. Am J Cardiol 51: 256–60PubMedCrossRefGoogle Scholar
  16. 16.
    Keane JF, Fellows KE, LaFarge CG, Nadas AS, Bernhard WF (1976) The surgical management of discrete and diffuse supravalvar aortic stenosis. Circulation 54: 112–7PubMedGoogle Scholar
  17. 17.
    Keating MT (1995) Genetic approaches to cardiovascular disease. Supravalvular aortic stenosis, Williams syndrome, and Long-QT syndrome. Circulation 92: 142–7PubMedGoogle Scholar
  18. 18.
    Martin MM, Lemmer JH, Shaffer E, Dick M, Bove EL (1988) Obstruction to left coronary artery blood flow secondary to obliteration of the cornary ostium in supravalvular aortic stenosis. Ann Thorac Surg 45: 16–20PubMedGoogle Scholar
  19. 19.
    Matsuda H, Miyamoto Y, Takahashi T, Kadoba K, Nakano S, Sano T (1991) Extended aortic and left main coronary angioplasty with a single pericardial patch in a patient with Williams syndrome. Ann Thorac Surg 52: 1331–3PubMedGoogle Scholar
  20. 20.
    McDonald AH, Gerlis LM, Somerville J (1969) Familial arteriopathy with associated pulmonary and systemic arterial stenoses. Br Heart J 31: 375–85PubMedCrossRefGoogle Scholar
  21. 21.
    McGoon DC, Mankin HT, Vlad P, Kirklin JW (1961) The surgical treatment of supravalvular aortic stenosis. J Thorac Cardiovasc Surg 41: 125–33Google Scholar
  22. 22.
    Mencarelli L (1930) Stenosi sopravalvolare aortica ad anello. Arch Ital Anat Patol 1: 829–841Google Scholar
  23. 23.
    Morrow AG, Waldhausen JA, Peters RL, Bloodwell RD, Braunwald E (1959) Supravalvular aortic stenosis. Clinical, hemodynamic and pathologic observations. Circulation 20: 1003–10PubMedGoogle Scholar
  24. 24.
    Myers JL, Waldhausen JA, Cyran SE, Gleason MM, Weber HS, Baylen BG (1993) Results of surgical repair of congenital supravalvular aortic stenosis. J Thorac Cardiovasc Surg 105: 281–8PubMedGoogle Scholar
  25. 25.
    Nakanishi T, Iwasaki Y, Momma K, Imai Y (1996) Supravalvular aortic stenosis, Pulmonary artery stenosis, and coronary artery stenosis in twins. Pediatr Cardiol 17: 125–8PubMedCrossRefGoogle Scholar
  26. 26.
    Neufeld HN, Wagenvoort CA, Ongley PA, Edwards JE (1962) Hypoplasia of the ascending aorta. Am J Cardiol 10: 746–51PubMedCrossRefGoogle Scholar
  27. 27.
    O'Connor WN, Davis JB, Geissler R, Cottrill CM, Noonan JA, Todd EP (1985) Supravalvular aortic stenosis. Clinical and pathological observations in six patients. Arch Pathol Lab Med 109: 179–85PubMedGoogle Scholar
  28. 28.
    Perou ML (1961) Congenital supravalvular aortic stenosis. Morphological study with attempt at classification. Arch Pathol Lab Med 71: 453–66Google Scholar
  29. 29.
    Peterson TA, Todd DB, Edwards JE (1965) Supravalvular aortic stenosis. J Thorac Cardiovasc Surg 50: 734–41PubMedGoogle Scholar
  30. 30.
    Rastelli GC, McGoon DC, Ongley PA, Mankin HT, Kirklin JW (1966) Surgical treatment of supravalvular aortic stenosis. J Thorac Cardiovasc Surg 51: 873–82PubMedGoogle Scholar
  31. 31.
    Roberts WC (1973) Valvular, subvalvular, and supravalvular aortic stenosis: Morphologic features. Cardiovasc Clinics 5: 97–125Google Scholar
  32. 32.
    Schmidt RE, Gilbert EF, Amend TC, Chamberlain CR, Lucas RV (1969) Generalized arterial fibromuscular dysplasia and myocardial infarction in familial supravalvular aortic stenosis syndrome. J Pediatr 74: 576–84PubMedCrossRefGoogle Scholar
  33. 33.
    Sharma BK, Fujiwara H, Hallman GL, Ott RA, Reul GJ, Cooley DA (1991) Supravalvar aortic stenosis: a 29-year review of surgical experience. Ann Thorac Surg 51: 1031–39PubMedCrossRefGoogle Scholar
  34. 34.
    Stamm C, Li J, Ho SY, Redington AN, Anderson RH (1997) The aortic root in supravalvular aortic stenosis: The potential surgical relevance of morphologic findings. J Thorac Cardiovasc Surg 114: 16–24PubMedCrossRefGoogle Scholar
  35. 35.
    Starr A, Dotter C, Griswold H (1961) Supravalvular aortic stenosis: Diagnosis and treatment. J Thorac Cardiovasc Surg 41: 134–40Google Scholar
  36. 36.
    Stewart S, Alexon C, Manning J (1988) Extended aortoplasty to relieve supravalvular aortic stenosis. Ann Thorac Surg 46: 427–9CrossRefGoogle Scholar
  37. 37.
    Terhune PE, Buchino JJ, Rees AH (1985) Myocardial infarction associated with supravalvular aortic stenosis. J Pediatr 106: 251–4PubMedCrossRefGoogle Scholar
  38. 38.
    van Son JAM, Danielson GK, Puga FJ, Schaff HV, Rastogi A, Edwards WD, Feldt RH (1994) Supravalvular aortic stenosis. Long-term results of surgical treatment. J Thorac Cardiovasc Surg 107: 103–15PubMedGoogle Scholar
  39. 39.
    van Son JAM, Edwards WD, Danielson GK (1994) Pathology of coronary arteries, myocardium, and great arteries in supravalvular aortic stenosis. J Thorac Cardiovasc Surg 108: 21–8PubMedGoogle Scholar
  40. 40.
    Williams JCP, Barratt-Boyes BG, Lowe JB (1961) Supravalvular Aortic Stenosis. Circulation 24: 1311–18PubMedGoogle Scholar

Copyright information

© Steinkopff Verlag 1997

Authors and Affiliations

  • C. Stamm
    • 1
  • T. Busch
    • 1
  • T. Felderhoff
    • 1
  • Jia Li
    • 2
  • R. H. Anderson
    • 2
  • Siew Yen Ho
    • 2
  1. 1.Klinik für Thorax-, Herz- und GefäßchirurgieGeorg-August Universität GöttingenGöttingen
  2. 2.Paediatrics National Heart & Lung InstituteImperial College School of MedicineLondonUK

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