İlker MATARACI, Berk ÖZKAYNAK, Adil POLAT, Kaan KIRALİ, Rahmi ZEYBEK, Bülent MERT, Ebru POLAT BAL, Vedat ERENTUĞ, Cevat YAKUT

Cardiovascular interventions in Marfan syndrome: the early and late results

Amaç: Bu çalışmada kliniğimizde ameliyat edilen Marfan sendromlu hastaların erken ve geç dönem sonuçları için risk faktörleri analiz edildi. Çalışma planı: Şubat 1987 - Ekim 2008 tarihleri arasında kliniğimizde Marfan sendromlu 62 hasta (45 erkek 17 kadın; ort. yaş 31.5±9.9 yıl; dağılım 11-56 yıl) ameliyat edildi. Bu hastalara toplam 90 cerrahi işlem uygulandı. Cerrahinin sağkalım üzerindeki etkisini değerlendirmek amacıyla, bu risk faktörlerinin tek değişkenli ve çok değişkenli analizleri yapıldı. Bulgular: Ortalama takip süresi 7.9±4.2 (dağılım 0.2- 17.4) yıl olup, toplam 471.2 hasta/yıl idi. İki hastada (%3.2) erken mortalite bildirildi. Bir, altı ve 15 yıllık sağkalım oranları sırasıyla, %96.8±2.2, %94.4±3.2 ve %87.1±5.8 idi. Hiçbir risk faktörü erken mortalite ile anlamlı düzeyde ilişkili bulunmadı. Geç mortalite için yapılan tek değişkenli analizde, acil ameliyat ve tekrar ameliyat mortalite ile ilişkili bulunurken, çok değişkenli analizde yalnızca acil ameliyat mortalite için anlamlı bir risk faktörüydü. Yeni anevrizma ve diseksiyon gelişimi ile mitral yetmezlik olmadan 15. yılda sağkalım oranı, sırasıyla %73.5±9.7, %93.2±4.9 ve %87.3±8.7 idi. Sonuç: Çalışma sonuçlarımız, cerrahi tedavi ile sağkalımda iyileşme olduğunu göstermekle birlikte, acil ameliyata alınan hastaların yaşam beklentisinde düşüş olduğunu göstermiştir. Bu nedenle, Marfan sendromlu hastalar kardiyovasküler tutulum açısından dikkatle izlenmelidir.

Marfan sendromunda kardiyovasküler girişimler: erken ve geç dönem sonuçları

Background: In this study, we analyzed the risk factors for early and late results in the patients with Marfan syndrome who underwent surgery in our clinic. Methods: Between February 1987 and October 2008, 62 patients with Marfan syndrome (45 males, 17 females; mean age 31.5±9.9 years; range 11 to 56 years) underwent surgery. A total of 90 surgical interventions were performed on these patients. Univariate and multivariate analyses of the risk factors were performed to evaluate the effect of these interventions on survival. Results: Mean follow-up was 7.9±4.2 (range 0.2 to 17.4) years, giving a total follow-up of 471.2 patient/years. Early mortality was reported in two of the patients (3.2%). Survival rates at one, six and 15 years were 96.8±2.2%, 94.4±3.2% and 87.1±5.8%, respectively. None of the risk factors were significantly associated with the early mortality. Univariate analysis of risk factors showed that emergency surgery and re-surgery were significantly associated with late mortality (p

PDF

___

1. Galla JD, Griepp RB. Surgical management of aortic and cardiac manifestations of Marfan syndrome. In: Franco KL, Verrier ED, editors. Advanced therapy in cardiac surgery. 1st ed. London: B.C. Decker; 1999. p 270-79.
2. Erentuğ V, Polat A, Kirali K, Akinci E, Yakut C. Cardiovascular manifestations and treatment in Marfan syndrome. [Article in Turkish] Anadolu Kardiyol Derg 2005;5:46-52.
3. Bonow RO, Carabello BA, Chatterjee K, de Leon AC Jr, Faxon DP, Freed MD, et al. 2008 focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to revise the 1998 guidelines for the management of patients with valvular heart disease). Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 2008;52:e1-142.
4. Erentug V, Polat A, Bozbuga NU, Polat E, Erdogan HB, Kirali K, et al. Cardiovascular reoperations in Marfan syndrome. J Card Surg 2006;21:455-7.
5. Kirali K, Erentuğ V, Rabuş MB, Izgi A, Bozbuğa NU, Erkanli K, et al. Extensive aortic surgery in Marfan syndrome: 16-year experience. Asian Cardiovasc Thorac Ann 2003;11:337-41.
6. Yakut C. A new modified Bentall procedure: the flanged technique. Ann Thorac Surg 2001;71:2050-2.
7. Doty JR, Cameron DE. Surgery for Marfan syndrome. In: Franco KL, Verrier ED, editors. Advanced therapy in cardiac surgery. 2nd ed. London: B.C. Decker; 2003. p. 304-11.
8. Baumgartner WA, Cameron DE, Redmond JM, Greene PS, Gott VL. Operative management of Marfan syndrome: The Johns Hopkins experience. Ann Thorac Surg 1999;67:1859-60.
9. Bonser RB, Quinn DW, Harrington DK. The prediction of thoracic aortic expansion. In: Liotta D, del Rio M, Cooley DA, Cabrol C, Griepp RB, Bonser RS, et al, editors. Diseases of the aorta. Buenos Aires: Domingo Liotta Foundation Medical; 2001. p. 141-58.
10. Ergin MA, Spielvogel D, Apaydin A, Lansman SL, McCullough JN, Galla JD, et al. Surgical treatment of the dilated ascending aorta: when and how? Ann Thorac Surg 1999;67:1834-9.
11. Umaña JP, Lai DT, Mitchell RS, Moore KA, Rodriguez F, Robbins RC, et al. Is medical therapy still the optimal treatment strategy for patients with acute type B aortic dissections? J Thorac Cardiovasc Surg 2002;124:896-910.
12. Kirali K, Mansuroğlu D, Omeroğlu SN, Erentuğ V, Mataraci I, Ipek G, et al. Five-year experience in aortic root replacement with the flanged composite graft. Ann Thorac Surg 2002;73:1130-7.
13. Treasure T. Cardiovascular surgery for Marfan syndrome. Heart 2000;84:674-8.
14. Codecasa R, Mariani MA, D’Alfonso A, Nardi C, Grandjean JG. Current indications for elective surgical treatment of dilated ascending aorta: a new formula. J Thorac Cardiovasc Surg 2003;125:1528-30.
15. Bozbuga N, Erentug V, Kirali K, Akinci E, Yakut C. Surgical management of mitral regurgitation in patients with Marfan syndrome. J Heart Valve Dis 2003;12:717-21.
16. Mataraci I, Polat A, Kiran B, Calişkan A, Tuncer A, Erentug V, et al. Long-term results of aortic root replacement: 15 years' experience. Ann Thorac Surg 2009;87:1783-8.
17. Bethea BT, Fitton TP, Alejo DE, Barreiro CJ, Cattaneo SM, Dietz HC, et al. Results of aortic valve-sparing operations: experience with remodeling and reimplantation procedures in 65 patients. Ann Thorac Surg 2004;78:767-72.
18. Zehr KJ, Orszulak TA, Mullany CJ, Matloobi A, Daly RC, Dearani JA, et al. Surgery for aneurysms of the aortic root: a 30-year experience. Circulation 2004;110:1364-71.
19. Fleischer KJ, Nousari HC, Anhalt GJ, Stone CD, Laschinger JC. Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan’s syndrome. Ann Thorac Surg 1997;63:1012-7.