Tổng quan về các chiến lược phẫu thuật hiện tại đối với bệnh lý động mạch chủ ở bệnh nhân mắc hội chứng Marfan

The Japanese journal of surgery - Tập 46 - Trang 1006-1018 - 2015
Shunsuke Miyahara1, Yutaka Okita1
1Division of Cardiovascular Surgery, Department Surgery, Kobe University Graduate School of Medicine, Kobe, Japan

Tóm tắt

Hội chứng Marfan là một rối loạn di truyền, toàn thân của mô liên kết với mức độ thẩm thấu cao, được đặt tên theo bác sĩ Antoine Marfan. Các biểu hiện lâm sàng quan trọng nhất của hội chứng này là các bệnh lý tim mạch gây ra những sự kiện đe dọa tính mạng, chẳng hạn như phẫu đoạn động mạch chủ cấp tính, vỡ động mạch chủ và hồi lưu van động mạch chủ hoặc các van nhĩ thất khác, dẫn đến suy tim. Những sự kiện này đóng vai trò quan trọng trong tuổi thọ của những bệnh nhân mắc rối loạn này, đặc biệt là trước khi phát triển các phương pháp phẫu thuật hiệu quả cho bệnh lý động mạch chủ lên. Để ngăn ngừa các sự kiện động mạch chủ thảm khốc như vậy, một ngưỡng thấp hơn đã được khuyến nghị cho các can thiệp phòng ngừa trên gốc động mạch chủ. Sau khi thay thế gốc động mạch chủ phòng ngừa, bệnh lý trong động mạch chủ ngoài gốc và xa nhánh vẫn là một vấn đề quan ngại. Nhiều cuộc phẫu thuật là cần thiết trong suốt cuộc đời của bệnh nhân, điều này có thể gặp vấn đề do tổn thương xa phức tạp bởi sự phẫu đoạn. Nhiều tranh cãi về các chiến lược phẫu thuật vẫn còn tồn tại, chẳng hạn như sửa chữa nội mạch, để quản lý các trường hợp phức tạp như vậy. Bài tổng quan này xem xét các xu hướng trong các chiến lược phẫu thuật để điều trị bệnh lý tim mạch ở bệnh nhân mắc hội chứng Marfan, và các quan điểm hiện tại trong lĩnh vực này.

Từ khóa

#hội chứng Marfan #bệnh lý động mạch chủ #can thiệp phòng ngừa #phẫu thuật tim mạch

Tài liệu tham khảo

Marfan ABJ. Un cas de déformation congénitale des quatre membres, plus prononcée aux extrémités, caractérisée par l’allongement des os avec un certain degré d’amincissement. Impr Maretheux; 1896. Van de Velde S, Fillman R, Yandow S. Protrusio acetabuli in Marfan syndrome. History, diagnosis, and treatment. J Bone Joint Surg Am Vol. 2006;88(3):639–46. Dietz HC, Cutting GR, Pyeritz RE, Maslen CL, Sakai LY, Corson GM, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature. 1991;352(6333):337–9. Mc KV. The cardiovascular aspects of Marfan’s syndrome: a heritable disorder of connective tissue. Circulation. 1955;11(3):321–42. Silverman DI, Burton KJ, Gray J, Bosner MS, Kouchoukos NT, Roman MJ, et al. Life expectancy in the Marfan syndrome. Am J Cardiol. 1995;75(2):157–60. Murdoch JL, Walker BA, Halpern BL, Kuzma JW, McKusick VA. Life expectancy and causes of death in the Marfan syndrome. N Engl J Med. 1972;286(15):804–8. Okita Y. Surgery for thoracic aortic disease in Japan: evolving strategies toward the growing enemies. Gen Thorac Cardiovasc Surg. 2015;63(4):185–96. Judge DP, Dietz HC. Marfan’s syndrome. Lancet. 2005;366(9501):1965–76. Lu S, Sun X, Wang C, Hong T, Xu D, Zhao W, et al. Surgical correction of giant extracardiac unruptured aneurysm of the right coronary sinus of Valsalva: case report and review of the literature. Gen Thorac Cardiovasc Surg. 2013;61(3):143–6. Nakagiri K, Kadowaki T, Morimoto N, Murakami H, Yoshida M, Mukohara N. Aortic root reimplantation for isolated sinus of valsalva aneurysm in the patient with Marfan’s syndrome. Ann Thorac Surg. 2012;93(3):e49–51. Roman MJ, Devereux RB, Kramer-Fox R, O’Loughlin J. Two-dimensional echocardiographic aortic root dimensions in normal children and adults. Am J Cardiol. 1989;64(8):507–12. Nienaber CA, Eagle KA. Aortic dissection: new frontiers in diagnosis and management: part I: from etiology to diagnostic strategies. Circulation. 2003;108(5):628–35. de Kerchove L, Glineur D, Poncelet A, Rubay J, Astarci P, Verhelst R, et al. Repair-oriented classification of aortic insufficiency: impact on surgical techniques and clinical outcomes. J Thorac Cardiovasc Surg. 2009;137(2):286–94. Gott VL, Greene PS, Alejo DE, Cameron DE, Naftel DC, Miller DC, et al. Replacement of the aortic root in patients with Marfan’s syndrome. N Engl J Med. 1999;340(17):1307–13. Elefteriades JA. Natural history of thoracic aortic aneurysms: indications for surgery, and surgical versus nonsurgical risks. Ann Thorac Surg. 2002;74(5):S1877–80 (discussion S92–8). Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome. Circulation. 2005;111(11):e150–7. Bentall H, De Bono A. A technique for complete replacement of the ascending aorta. Thorax. 1968;23(4):338–9. Etz CD, Homann TM, Rane N, Bodian CA, Di Luozzo G, Plestis KA, et al. Aortic root reconstruction with a bioprosthetic valved conduit: a consecutive series of 275 procedures. J Thorac Cardiovasc Surg. 2007;133(6):1455–63. Pearson GD, Devereux R, Loeys B, Maslen C, Milewicz D, Pyeritz R, et al. Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on research in Marfan syndrome and related disorders. Circulation. 2008;118(7):785–91. Gott VL, Pyeritz RE, Magovern GJ Jr, Cameron DE, McKusick VA. Surgical treatment of aneurysms of the ascending aorta in the Marfan syndrome. Results of composite-graft repair in 50 patients. N Engl J Med. 1986;314(17):1070–4. Sato S, Ogino H, Matsuda H, Sasaki H, Tanaka H, Iba Y. Annuloaortic ectasia treated successfully in a pregnant woman with Marfan syndrome: report of a case. Surg Today [Case Reports]. 2012;42(3):285–7. Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE Jr, et al. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for the diagnosis and management of patients with thoracic aortic disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, and Society for Vascular Medicine. Circulation. 2010;121(13):e266–369. Nishida T, Sonoda H, Oishi Y, Ushijima T, Tanoue Y, Nakashima A, et al. More than 20-year experience of Bentall operation with mechanical prostheses for chronic aortic root aneurysm. Gen Thorac Cardiovasc Surg. 2014:1–8. Fukunaga N, Koyama T, Konishi Y, Murashita T, Kanemitsu H, Okada Y. Clinical outcome of redo operation on aortic root. Gen Thorac Cardiovasc Surg. 2014;62(4):215–20. Cameron DE, Alejo DE, Patel ND, Nwakanma LU, Weiss ES, Vricella LA, et al. Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years. Ann Thorac Surg. 2009;87(5):1344-9 (discussion 9–50). 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. David TE, Feindel CM. An aortic-valve sparing operation for patients with aortic incompetence and aneurysm of the ascending aorta. J Thorac Cardiovasc Surg. 1992;103(4):617–22. Sarsam MA, Yacoub M. Remodeling of the aortic valve anulus. J Thorac Cardiovasc Surg. 1993;105(3):435–8. 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(4):1012–7. David TE. The aortic valve-sparing operation. J Thorac Cardiovasc Surg. 2011;141(3):613–5. David TE, Armstrong S, Maganti M, Colman J, Bradley TJ. Long-term results of aortic valve-sparing operations in patients with Marfan syndrome. J Thorac Cardiovasc Surg 2009;138(4):859–64 (discussion 63–4). Kallenbach K, Karck M, Pak D, Salcher R, Khaladj N, Leyh RG, et al. A decade of aortic valve-sparing reimplantation: are we pushing the limits too far? Circulation. 2004;110(17):671. Karck M, Kallenbach K, Hagl C, Rhein C, Leyh R, Haverich A. Aortic root surgery in Marfan syndrome: comparison of aortic valve-sparing reimplantation versus composite grafting. J Thorac Cardiov Surg. 2004;127(2):391–8. Kvitting JPE, Kari FA, Fischbein MP, Liang DH, Beraud AS, Stephens EH, et al. David valve-sparing aortic root replacement: Equivalent mid-term outcome for different valve types with or without connective tissue disorder. J Thorac Cardiovasc Surg. 2013;145(1):117. Coselli JS, Volguina IV, LeMaire SA, Sundt TM, Connolly HM, Stephens EH, et al. Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: a prospective, multicenter, comparative study. J Thorac Cardiovasc Surg 2014;147(6):1758–67 e4. David TE, Feindel CM, David CM, Manlhiot C. A quarter of a century of experience with aortic valve-sparing operations. J Thorac Cardiovasc Surg 2014 May 15. Birks EJ, Webb C, Child A, Radley-Smith R, Yacoub MH. Early and long-term results of a valve-sparing operation for Marfan syndrome. Circulation. 1999;100(19 Suppl):II29–35. Tanaka H, Ogino H, Matsuda H, Minatoya K, Sasaki H, Iba Y. Midterm outcome of valve-sparing aortic root replacement in inherited connective tissue disorders. Ann Thorac Surg. 2011;92(5):1646–9; (discussion 9–50). Hanke T, Charitos EI, Stierle U, Robinson D, Gorski A, Sievers HH, et al. Factors associated with the development of aortic valve regurgitation over time after two different techniques of valve-sparing aortic root surgery. J Thorac Cardiovasc Surg. 2009;137(2):314–9. Kallenbach K, Baraki H, Khaladj N, Kamiya H, Hagl C, Haverich A, et al. Aortic Valve-Sparing Operation in Marfan Syndrome: what Do We Know After a Decade? Ann Thorac Surg. 2007;83(2):S764–8. Patel ND, Weiss ES, Alejo DE, Nwakanma LU, Williams JA, Dietz HC, et al. Aortic root operations for Marfan syndrome: a comparison of the Bentall and valve-sparing procedures. Ann Thorac Surg. 2008;85(6):2003–10 (discussion 10–1). Erasmi AW, Sievers HH, Bechtel JF, Hanke T, Stierle U, Misfeld M. Remodeling or reimplantation for valve-sparing aortic root surgery? Ann Thorac Surg. 2007;83(2):S752–6 (discussion S85–90). 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(3):767–72; (discussion 72). Kunihara T, Aicher D, Rodionycheva S, Asano M, Tochii M, Sata F, et al. Outcomes after valve-preserving root surgery for patients with Marfan syndrome. J Heart Valve Dis. 2012;21(5):615–22. Leyh RG, Schmidtke C, Sievers HH, Yacoub MH. Opening and closing characteristics of the aortic valve after different types of valve-preserving surgery. Circulation. 1999;100(21):2153–60. Kunihara T, Aicher D, Rodionycheva S, Groesdonk HV, Langer F, Sata F, et al. Preoperative aortic root geometry and postoperative cusp configuration primarily determine long-term outcome after valve-preserving aortic root repair. J Thorac Cardiovasc Surg. 2011; Aug 18. Fries R, Graeter T, Aicher D, Reul H, Schmitz C, Bohm M, et al. In vitro comparison of aortic valve movement after valve-preserving aortic replacement. J Thorac Cardiovasc Surg. 2006;132(1):32–7. De Paulis R, De Matteis GM, Nardi P, Scaffa R, Colella DF, Bassano C, et al. One-year appraisal of a new aortic root conduit with sinuses of Valsalva. J Thorac Cardiovasc Surg. 2002;123(1):33–9. Matsumori M, Tanaka H, Kawanishi Y, Onishi T, Nakagiri K, Yamashita T, et al. Comparison of distensibility of the aortic root and cusp motion after aortic root replacement with two reimplantation techniques: valsalva graft versus tube graft. Interact CardioVasc Thorac Surg. 2007;6(2):177–81. Lansac E, Di Centa I, Sleilaty G, Crozat EA, Bouchot O, Hacini R, et al. An aortic ring: from physiologic reconstruction of the root to a standardized approach for aortic valve repair. J Thorac Cardiovasc Surg 2010;140(6 Suppl):S28–35 (discussion S45–51). Kazui T, Tsuboi J, Izumoto H, Nakajima T, Ishihara K, Kawazoe K. Aortic root remodeling with aortic annuloplasty: mid-term results. Circ J: Off J Jpn Circ Soc. 2007;71(2):207–10. Aicher D, Schneider U, Schmied W, Kunihara T, Tochii M, Schafers HJ. Early results with annular support in reconstruction of the bicuspid aortic valve. J Thorac Cardiovasc Surg. 2013;145(3 Suppl):S30–4. Tochii M, Hattori K, Ishikawa H, Ishida M, Higuchi Y, Amano K, et al. Mid-term outcomes of cardiovascular surgery for patients with Marfan syndrome. Gen Thorac Cardiovasc Surg. 2015;63(5):267–72. Niinami H, Aomi S, Tagusari O, Hashimoto A, Koyanagi H. Extensive aortic reconstruction for aortic aneurysms in Marfan syndrome. Ann Thorac Surg 1999;67(6):1864–7 (discussion 8–70). Nollen GJ, Groenink M, Tijssen JG, Van Der Wall EE, Mulder BJ. Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome. Eur Heart J. 2004;25(13):1146–52. den Hartog AW, Franken R, Zwinderman AH, Timmermans J, Scholte AJ, van den Berg MP, et al. The risk for type B aortic dissection in marfan syndrome. J Am Coll Cardiol. 2015;65(3):246–54. Scharfschwerdt M, Sievers HH, Greggersen J, Hanke T, Misfeld M. Prosthetic replacement of the ascending aorta increases wall tension in the residual aorta. Ann Thorac Surg. 2007;83(3):954–7. Nollen GJ, Meijboom LJ, Groenink M, Timmermans J, Barentsz JO, Merchant N, et al. Comparison of aortic elasticity in patients with the Marfan syndrome with and without aortic root replacement. Am J Cardiol. 2003;91(5):637–40. Schoenhoff FS, Jungi S, Czerny M, Roost E, Reineke D, Matyas G, et al. Acute Aortic dissection determines the fate of initially untreated aortic segments in Marfan syndrome. Circulation. 2013; Mar 14. Girdauskas E, Kuntze T, Borger MA, Falk V, Mohr FW. Distal aortic reinterventions after root surgery in Marfan patients. Ann Thorac Surg. 2008;86(6):1815–9. Carrel T, Beyeler L, Schnyder A, Zurmuhle P, Berdat P, Schmidli J, et al. Reoperations and late adverse outcome in Marfan patients following cardiovascular surgery. Eur J Cardio-Thorac Surg: Off J Eur Assoc Cardio-thorac Surg. 2004;25(5):671–5. Pyeritz RE. Marfan syndrome: 30 years of research equals 30 years of additional life expectancy. Heart. 2009;95(3):173–5. Kari FA, Beyersdorf F, Stephens EH, Peter P, Rylski B, Russe M, et al. Results after thoracic aortic reoperations in marfan syndrome. Ann Thorac Surg. 2014;97(4):1275–80. Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo RD, Eggebrecht H, et al. 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC). Eur Heart J. 2014;35(41):2873–926. Achneck HE, Rizzo JA, Tranquilli M, Elefteriades JA. Safety of thoracic aortic surgery in the present era. Ann Thorac Surg. 2007;84(4):1180–5 (discussion 5). Okita Y. Fighting spinal cord complication during surgery for thoracoabdominal aortic disease. Gen Thorac Cardiovasc Surg. 2011;59(2):79–90. Svensson LG, Hess KR, D’Agostino RS, Entrup MH, Hreib K, Kimmel WA, et al. Reduction of neurologic injury after high-risk thoracoabdominal aortic operation. Ann Thorac Surg. 1998;66(1):132–8. Miyamoto K, Ueno A, Wada T, Kimoto S. A new and simple method of preventing spinal cord damage following temporary occlusion of the thoracic aorta by draining the cerebrospinal fluid. J Cardiovasc Surg. 1960;1:188–97. Coselli JS, LeMaire SA, Koksoy C, Schmittling ZC, Curling PE. Cerebrospinal fluid drainage reduces paraplegia after thoracoabdominal aortic aneurysm repair: results of a randomized clinical trial. J Vasc Surg. 2002;35(4):631–9. Crawford ES, Svensson LG, Hess KR, Shenaq SS, Coselli JS, Safi HJ, et al. A prospective randomized study of cerebrospinal fluid drainage to prevent paraplegia after high-risk surgery on the thoracoabdominal aorta. J Vasc Surg. 13(1):36–45 (discussion 6). Kouchoukos NT, Rokkas CK. Hypothermic cardiopulmonary bypass for spinal cord protection: rationale and clinical results. Ann Thorac Surg. 1999;67(6):1940–2 (discussion 53–8). Bischoff MS, Scheumann J, Brenner RM, Ladage D, Bodian CA, Kleinman G, et al. Staged approach prevents spinal cord injury in hybrid surgical-endovascular thoracoabdominal aortic aneurysm repair: an experimental model. Ann Thorac Surg. 2011;92(1):138–46 (discussion 46). Etz CD, Zoli S, Mueller CS, Bodian CA, Di Luozzo G, Lazala R, et al. Staged repair significantly reduces paraplegia rate after extensive thoracoabdominal aortic aneurysm repair. J Thorac Cardiovasc Surg. 2010;139(6):1464–72. Omura A, Tanaka A, Miyahara S, Sakamoto T, Nomura Y, Inoue T, et al. Early and late results of graft replacement for dissecting aneurysm of thoracoabdominal aorta in patients with Marfan syndrome. Ann Thorac Surg. 2012;94(3):759–65. LeMaire SA, Carter SA, Volguina IV, Laux AT, Milewicz DM, Borsato GW, et al. Spectrum of aortic operations in 300 patients with confirmed or suspected Marfan syndrome. Ann Thorac Surg. 2006;81(6):2063–78 (discussion 78). Kalkat MS, Rahman I, Kotidis K, Davies B, Bonser RS. Presentation and outcome of Marfan’s syndrome patients with dissection and thoraco-abdominal aortic aneurysm. Eur J Cardiothorac Surg. 2007;32(2):250–4. Mommertz G, Sigala F, Langer S, Koeppel TA, Mess WH, Schurink GW, et al. Thoracoabdominal aortic aneurysm repair in patients with marfan syndrome. Eur J Vasc Endovasc Surg. 2008;35(2):181–6. Svensson LG, Crawford ES, Coselli JS, Safi HJ, Hess KR. Impact of cardiovascular operation on survival in the Marfan patient. Circulation. 1989;80(3 Pt 1):I233–42. Schoenhoff FS, Kadner A, Czerny M, Jungi S, Meszaros K, Schmidli J, et al. Should aortic arch replacement be performed during initial surgery for aortic root aneurysm in patients with Marfan syndrome? Eur J Cardio-Thorac. 2013;44(2):346–51. Castrovinci S, Murana G, de Maat GE, Smith T, Schepens MA, Heijmen RH, et al. The classic Elephant Trunk Technique for staged thoracic and thoracoabdominal aortic repair: long-term results. J Thorac Cardiovasc Surg. 2014. Tagusari O, Ogino H, Kobayashi J, Bando K, Minatoya K, Sasaki H, et al. Should the transverse aortic arch be replaced simultaneously with aortic root replacement for annuloaortic ectasia in Marfan syndrome? J Thorac Cardiov Surg. 2004;127(5):1373–80. Bachet J, Larrazet F, Goudot B, Dreyfus G, Folliguet T, Laborde F, et al. When should the aortic arch be replaced in Marfan patients? Ann Thorac Surg. 2007;83(2):S774–9. Borst HG, Walterbusch G, Schaps D. Extensive aortic replacement using elephant trunk prosthesis. Thorac Cardiovas Surg. 1983;31(1):37–40. Safi HJ, Miller CC, 3rd, Estrera AL, Villa MA, Goodrick JS, Porat E, et al. Optimization of aortic arch replacement: two-stage approach. Ann Thorac Surg. 2007;83(2):S815–8 (discussion S24–31). Wong DR, Parenti JL, Green SY, Chowdhary V, Liao JM, Zarda S, et al. Open repair of thoracoabdominal aortic aneurysm in the modern surgical era: contemporary outcomes in 509 patients. J Am Coll Surg. 2011;212(4):569–79 (discussion 79–81). Hino Y, Okada K, Oka T, Inoue T, Tanaka A, Omura A, et al. Extended replacement of the thoracic aorta. Eur J Cardio-Thorac Surg: Off J Eur Assoc Cardio-Thorac Surg. 2013;43(1):176–81 (discussion 81). Sun XG, Zhang L, Yu CT, Qian XY, Chang Q. One-stage repair of extensive aortic aneurysms: mid-term results with total or subtotal aortic replacement. Interact Cardiovasc Thorac Surg. 2014;18(3):278–82. Corvera JS, Fehrenbacher JW. Total arch and descending thoracic aortic replacement by left thoracotomy. Ann Thorac Surg. 2012;93(5):1510–5 (discussion 5–6). Masuda M, Kuwano H, Okumura M, Amano J, Arai H, Endo S, et al. Thoracic and cardiovascular surgery in Japan during 2012: annual report by The Japanese Association for Thoracic Surgery. Gen Thorac Cardiovasc Surg. 2014;62(12):734–64. Kari FA, Russe MF, Peter P, Blanke P, Rylski B, Euringer W, et al. Late complications and distal growth rates of Marfan aortas after proximal aortic repair. Eur J Cardio-Thorac Surg: Off J Eur Assoc Cardio-Thorac Surg. 2013;44(1):163–71. Rylski B, Bavaria JE, Beyersdorf F, Branchetti E, Desai ND, Milewski RK, et al. Type A aortic dissection in Marfan syndrome: extent of initial surgery determines long-term outcome. Circulation. 2014;129(13):1381–6. Minami H, Miyahara S, Okada K, Matsumori M, Kano H, Inoue T, et al. Clinical outcomes of combined aortic root reimplantation technique and total arch replacement. Eur J Cardio-Thorac Surg: Off J Eur Assoc Cardio-Thorac Surg. 2014; Oct 29. van Karnebeek CD, Naeff MS, Mulder BJ, Hennekam RC, Offringa M. Natural history of cardiovascular manifestations in Marfan syndrome. Arch Dis Child. 2001;84(2):129–37. Sisk HE, Zahka KG, Pyeritz RE. The Marfan syndrome in early childhood: analysis of 15 patients diagnosed at less than 4 years of age. Am J Cardiol. 1983;52(3):353–8. Everitt MD, Pinto N, Hawkins JA, Mitchell MB, Kouretas PC, Yetman AT. Cardiovascular surgery in children with Marfan syndrome or Loeys-Dietz syndrome. J Thorac Cardiovasc Surg. 2009;137(6):1327–32 (discussion 32–3). Pyeritz RE, Wappel MA. Mitral valve dysfunction in the Marfan syndrome. Clinical and echocardiographic study of prevalence and natural history. Am J Med. 1983;74(5):797–807. Fuzellier JF, Chauvaud SM, Fornes P, Berrebi AJ, Lajos PS, Bruneval P, et al. Surgical management of mitral regurgitation associated with Marfan’s syndrome. Ann Thorac Surg. 1998;66(1):68–72. Carpentier A. Cardiac valve surgery–the “French correction”. J Thorac Cardiovasc Surg. 1983;86(3):323–37. Carpentier A, Chauvaud S, Fabiani JN, Deloche A, Relland J, Lessana A, et al. Reconstructive surgery of mitral valve incompetence: ten-year appraisal. J Thorac Cardiovasc Surg. 1980;79(3):338–48. Anyanwu AC, Adams DH. Etiologic classification of degenerative mitral valve disease: Barlow’s disease and fibroelastic deficiency. Semin Thorac Cardiovasc Surg. 2007;19(2):90–6. Read RC, Thal AP, Wendt VE. Symptomatic valvular myxomatous transformation (the floppy valve syndrome). A possible forme fruste of the Marfan syndrome. Circulation. 1965;32(6):897–910. Bhudia SK, Troughton R, Lam BK, Rajeswaran J, Mills WR, Gillinov AM, et al. Mitral valve surgery in the adult Marfan syndrome patient. Ann Thorac Surg. 2006;81(3):843–8. Gillinov AM, Hulyalkar A, Cameron DE, Cho PW, Greene PS, Reitz BA, et al. Mitral valve operation in patients with the Marfan syndrome. J Thorac Cardiovasc Surg. 1994;107(3):724–31. Keane MG, Pyeritz RE. Medical management of Marfan syndrome. Circulation. 2008;117(21):2802–13. Roberts WC, Honig HS. The spectrum of cardiovascular disease in the Marfan syndrome: a clinico-morphologic study of 18 necropsy patients and comparison to 151 previously reported necropsy patients. Am Heart J. 1982;104(1):115–35. Sliwa K, Hilfiker-Kleiner D, Petrie MC, Mebazaa A, Pieske B, Buchmann E, et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy. Eur J Heart Fail. 2010;12(8):767–78. Nienaber CA, Fattori R, Lund G, Dieckmann C, Wolf W, von Kodolitsch Y, et al. Nonsurgical reconstruction of thoracic aortic dissection by stent-graft placement. N Engl J Med. 1999;340(20):1539–45. Nienaber CA, Kische S, Rousseau H, Eggebrecht H, Rehders TC, Kundt G, et al. Endovascular repair of type B aortic dissection: long-term results of the randomized investigation of stent grafts in aortic dissection trial. Circ Cardiovasc Interven. 2013;6(4):407–16. Lin PH, Huynh TT, Kougias P, Huh J, LeMaire SA, Coselli JS. Descending thoracic aortic dissection: evaluation and management in the era of endovascular technology. Vasc Endovasc Surg. 2009;43(1):5–24. Marcheix B, Rousseau H, Bongard V, Heijmen RH, Nienaber CA, Ehrlich M, et al. Stent grafting of dissected descending aorta in patients with Marfan’s syndrome: mid-term results. JACC Cardiovascular interventions. 2008;1(6):673–80. Botta L, Russo V, La Palombara C, Rosati M, Di Bartolomeo R, Fattori R. Stent graft repair of descending aortic dissection in patients with Marfan syndrome: an effective alternative to open reoperation? J Thorac Cardiovasc Surg. 2009;138(5):1108–14. Tanaka K, Yoshitaka H, Chikazawa G, Sakaguchi T, Totsugawa T, Tamura K. Investigation of the surgical complications during thoracic endovascular aortic repair: experiences with 148 consecutive cases treated at a single institution in Japan. Surg Today. 2015;45(1):22–8. Miyahara S, Nomura Y, Shirasaka T, Taketoshi H, Yamanaka K, Omura A, et al. Early and midterm outcomes of open surgical correction after thoracic endovascular aortic repair. Ann Thorac Surg. 2013; Apr 5. Roselli EE, Abdel-Halim M, Johnston DR, Soltesz EG, Greenberg RK, Svensson LG, et al. Open aortic repair after prior thoracic endovascular aortic repair. Ann Thorac Surg. 2014;97(3):750–6. Nordon IM, Hinchliffe RJ, Holt PJ, Morgan R, Jahangiri M, Loftus IM, et al. Endovascular management of chronic aortic dissection in patients with Marfan syndrome. J Vasc Surg. 2009;50(5):987–91. Geisbusch P, Kotelis D, von Tengg-Kobligk H, Hyhlik-Durr A, Allenberg JR, Bockler D. Thoracic aortic endografting in patients with connective tissue diseases. J Endovasc Therapy: Off J Int Soc Endovasc Specialists. 2008;15(2):144–9. Thrumurthy SG, Karthikesalingam A, Patterson BO, Holt PJ, Hinchliffe RJ, Loftus IM, et al. A systematic review of mid-term outcomes of thoracic endovascular repair (TEVAR) of chronic type B aortic dissection. Eur J Vasc Endovasc Surg: Off J Eur Soc Vasc Surg. 2011;42(5):632–47. Pacini D, Parolari A, Berretta P, Di Bartolomeo R, Alamanni F, Bavaria J. Endovascular treatment for type B dissection in Marfan syndrome: is it worthwhile? Ann Thorac Surg. 2013;95(2):737–49. Xu SD, Huang FJ, Yang JF, Li ZZ, Yang S, Du JH, et al. Early and midterm results of thoracic endovascular aortic repair of chronic type B aortic dissection. J Thorac Cardiovasc Surg. 2010;139(6):1548–53. Czerny M, Roedler S, Fakhimi S, Sodeck G, Funovics M, Dumfarth J, et al. Midterm results of thoracic endovascular aortic repair in patients with aneurysms involving the descending aorta originating from chronic type B dissections. Ann Thorac Surg. 2010;90(1):90–4. Guangqi C, Xiaoxi L, Wei C, Songqi L, Chen Y, Zilun L, et al. Endovascular repair of Stanford type B aortic dissection: early and mid-term outcomes of 121 cases. Eur J Vasc Endovasc Surg: Off J Eur Soc Vasc Surg. 2009;38(4):422–6. Taurino M, Ficarelli R, Rizzo L, Stella N, Persiani F, Capuano F. Hybrid treatment for thoracoabdominal aortic aneurysms in patients with marfan syndrome. Ann Vasc Surg. 2015;29(3):595 e5–9. Gelpi G, Mazzaccaro D, Romagnoni C, Contino M, Antona C. Hybrid endovascular treatment of an aortic root and thoracoabdominal aneurysm in a high-risk patient with Marfan syndrome. Vasc Endovasc Surg. 2013;47(4):300–3. Sun LZ, Li M, Zhu JM, Liu YM, Chang Q, Zheng J, et al. Surgery for patients with Marfan syndrome with type A dissection involving the aortic arch using total arch replacement combined with stented elephant trunk implantation: the acute versus the chronic. J Thorac Cardiovasc Surg. 2011;142(3):E85–91. Uchida N. Open stent grafting for complex diseases of the thoracic aorta: clinical utility. Gen Thorac Cardiovasc Surg. 2013;61(3):118–26. Lombardi JV, Cambria RP, Nienaber CA, Chiesa R, Teebken O, Lee A, et al. Prospective multicenter clinical trial (STABLE) on the endovascular treatment of complicated type B aortic dissection using a composite device design. J Vasc Surg. 2012;55(3):629–40 e2. Fattori R, Montgomery D, Lovato L, Kische S, Di Eusanio M, Ince H, et al. Survival after endovascular therapy in patients with type B aortic dissection: a report from the International Registry of Acute Aortic Dissection (IRAD). JACC Cardiovasc Interventions. 2013;6(8):876–82.