Valdés DO, Naffe AM, Villar IA, Chao GN, Chil DR, Aldama PL

Language: Spanish
References: 33
Page: 182-188
PDF size: 369.62 Kb.

ABSTRACT

Introduction and Objective: Marfan syndrome is a genetic disorder that causes destruction of elastic fibers in tissues that contain them. The cardiovascular system is one of the most commonly affected, where a progressive dilatation of the aorta (often asymptomatic) occurs, and if undiagnosed it can lead to the patient's death at an early age in life. The objective was to describe the results of the progress of patients with Marfan syndrome and ascending aortic surgery.
Método: A descriptive study was conducted between January 2007 and December 2010, where six consecutive patients with Marfan syndrome underwent surgery in the Service of Cardiovascular Surgery of Hermanos Ameijeiras Hospital. The variables related to the diagnosis and surgical procedures and postoperative progress at one year were analyzed.
Resultados: Aortic disorders in Marfan syndrome occurred at a relatively young age (40.33 ± 8.33). Aortic dissection was the most common disease (66.6%), and was related to the presence of pain and hypertension. Radical techniques predominated (66.6%) over the conservative ones. Surgical mortality was 16.6%, with one death; the remaining patients had a survival at one year of 100%.
Conclusiones: Male patients with aortic dissection and normal ejection fraction were predominant. The most used surgical technique was that of Bental De Bono. Only one patient died in the hospital, the rest had an excellent evolution at one year.

REFERENCES

1. Ho NC, Tran J, Bektas A. Marfan´s syndrome. Lan-cet. 2005;366(9501):1978-81.

2. Nollen GJ, Mulder BJ. What is new in the Marfan syndrome? Int J Cardiol. 2004;97 Suppl 1:103-8.

3. Oliva P, Regina Moreno R, Toledo I, Montecinos A, Molina J. Síndrome de Marfán. Rev Méd Chile. 2006;134(11):1455-64.

4. Geva T, Sanders SP, Diogenes MS, Rockenmacher S, Van Praagh R. Two-dimensional and Doppler echocardiographic and pathologic characteristics of the infantile Marfán Syndrome. Am J Cardiol. 1990;65(18):1230-7.

5. Yalcin F, Thomas JD, Homa D, Flachskampf FA. Transesophageal echocardiography: first-line ima-ging for aortic diseases. Cleve Clin J Med. 2000; 67(6):417-21.

6. Montíes J R. Aneurismas de la aorta torácica. Patel J, Blondeau H, Leger L, Blondeau P, Garbay G. En: Tratado de técnica quirúrgica. Vol. 4. Paris: Toray-Masson; 1975. p. 1091.

7. Robicsek F. A New method to treat fusiform aneu-rysms of the ascending aorta associated with aor-tic valve disease: an alternative to radical resec-tion. Ann Thorac Surg. 1982;34(1):32-4.

8. Cooley DA. Early development of surgical treat-ment for aortic aneurysms: personal recollections. Tex Heart Inst J. 2001;28(3):197-9.

9. Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax. 1968; 23(4):338-9.

10. Fagan A, Pillai R, Radley-Smiths R, Yacoub MH. Re-sults of new valve conserving operation for treat-ment of aneurysms or acute dissection of aortic root. Br Heart J. 1983;49(3):302 [Resumen].

11. Yacoub MH, Gehle P, Chandrasekaran V, Birks EJ, Child A, Radley-Smith R. Late results of a valve-preserving operation in patients with aneurysms of the ascending aorta and root. J Thorac Cardio-vasc. 1998;115(5):1080-90.

12. Fries R, Graeter T, Aicher D, Reul H, Schmitz C, Böhm M, et al. In vitro comparison of aortic valve movement after valve-preserving aortic replace-ment. J Thorac Cardiovasc Surg. 2006;132(1):32-7.

13. Schäfers HJ, Bierbach B, Aicher D. A new approach to the assessment of aortic cusp geometry. J Thorac Cardiovasc Surg. 2006;132(2):436-8.

14. David TE, Feindel CM. An aortic valve-sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thoracic and Cardiovasc Surg. 1992;103(4):617-21.

15. David TE, Feindel CM, Webb GD, Colman JM, Armstrong S, Maganti M. Long-term results of aortic valve-sparing operations for aortic root aneurysm. J Thorac Cardiovasc Surg. 2006;132(2): 347-54.

16. Simon P, Mortiz A, Moidl R, Kupilik N, Graben-woeger M, Ehrlich M, et al. Aortic valve resus-pension in ascending aortic aneurysm repair with aortic insufficiency. Ann Thorac Surg. l995;60(l): 176-80.

17. Hess PJ, Klodell CT, Beaver TM, Martin TD. The Florida sleeve: A new technique for aortic root remodeling with preservation of the aortic valve and sinuses. Ann Thorac Surg. 2005;80(2):748-50.

18. Van Karnebeek CD, Naeff MS, Mulder BJ, Henne-kam RC, Offringa M. Natural history of cardiovas-cular manifestations in Marfan syndrome. Arch Dis Child. 2001;84(2):129-37.

19. Segura AM, Luna RE, Horiba K, Stetler WG, McAllis-ter HA, Willerson JT, et al. Inmunohistochemistry of matrix metalloproteinases and their inhibitors in thoracic aortic aneurysm and aortic valves of patients with Marfan’s syndrome. Circulation. 1998;98(19 Suppl):II331-7.

20. Hagl C, Strauch JT, Spielvogel D, Galla JD, Lansman SL, Squitieri R, et al. Is the Bentall procedure for ascending aorta or aortic valve replacement the best approach for long-term event-free survival? Ann Thorac Surg. 2003;76(3):698-703.

21. Gott VL, Cameron DE, Alejo DE, Greene PS, Shake JG, Caparrelli DJ, et al. Aortic root replacement in 271 Marfan patients: a 24 year experience. Ann Thorac Surg. 2002;73 (2):438-43.

22. Cameron DE, Gott VL. Surgical management of the Marfan patient at the Johns Hopkins Hospital. En: Robinson PN, Godfrey M, eds. Marfan Syndrome: A Primer for Clinicians and Scientists. Georgetown: Landes Bioscience, 2004; p, 70-80.

23. David TE, Ivanov J, Amstrong S, Feindel CM, Webb GD. Aortic valve sparing operations in patients with aneurysms of the aortic root or ascending aorta. Ann Thorac Surg. 2002;74:S1758-61.

24. Leyh RG, Kallenbach K, Karck M, Hagl C, Fischer S, Haverich A. Impact of preoperative aortic root diameter on long-term aortic valve function after valve sparing aortic root reimplantation. Circula-tion. 2003;108 Suppl 1:II285-90.

25. Griepp RB, Lytle BW, David TE, Schafers HJ, Kallenbach K, Galla JD, et al. Aortic Surgery Sympo-sium VIII. Discussion: Session 1 – Ascending Aorta. Ann Thorac Surg. 2002;74:S1792-9.

26. Montesinos Mosqueira E, Vásquez Kobashigawa J C, Rojas Peña L, Peralta Rodríguez J. Tratamiento quirúrgico de disección de aorta torácica ascen-dente en síndrome de Marfan. Rev Med Hered. 2007;18(2):110-4.

27. Rampoldi V, Trimarchi S, Eagle KA, Nienaber CA, Oh JK, Bossone E, et al. Simple risk models to predict surgical mortality in acute type A aortic dissection: The International Registry of Acute Aortic Dissection Score. Ann Thorac Surg. 2007; 83(1):55-61.

28. Evangelista A, Padilla F, López-Ayerbe J, Calvo F, López-Pérez JM, Sánchez V, et al. Registro español del síndrome aórtico agudo (RESA). La mejora en el diagnóstico no se refleja en la reducción de la mortalidad. Rev Esp Cardiol. 2009;62(3):255-62.

29. Tamura N, Komiya T, Sakaguchi G, Kobayashi T. ‘Turn-up’ anastomotic technique for acute aortic

30. dissection. Eur J Cardiothorac Surg 2007;31(3): 548-9.

31. Ruvolo G, Fattouch K. Aortic valve-sparing root replacement from inside the aorta using three Da-cron skirts preserving the native Valsalva sinuses geometry and stabilizing the annulus. Interact CardioVasc Thorac Surg. 2009;8(2):179-81.

32. Forteza A, Cortina JM, Sánchez V, Centeno J, López MJ, Pérez de la Sota E, et al. Experiencia inicial con la preservación de la válvula aórtica en el sín-drome de Marfan. Rev Esp Cardiol. 2007;60(5): 471-5.

33. Favaloro RR, Casabé JH, Segura M, Abud J, Casas J, López C, et al. Tratamiento quirúrgico de las complicaciones de la aorta ascendente en el síndrome de Marfan. Resultados inmediatos y a largo plazo. Rev Esp Cardiol. 2008;61(8):884-7.