Clinical strategy of surgical management for Marfan syndrome in patients with severe left ventricular dysfunction_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

XU Wenliu ¹ ,  FAN Xiaoping ² , HUANG Jingsong ² , ZHANG Mingsheng ³ , PENG Jihai ³ , CAI Shihao ² , HE Jie ² ,  CHEN Qunqing ¹

1. Department of Cardiovascular Surgery, Zhujiang Hospital, Southern Medical University, Guangzhou, 510280, P.R.China;
2. Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, 510080, P.R.China;
3. Department of Rehabilitation, Guangdong General Hospital, Guangdong Academy of Medical Sciences, Guangzhou, 510080, P.R.China;

Corresponding author：

FAN Xiaoping, Email: fukui-hanson@hotmail.com; CHEN Qunqing, Email: chenqqg1985@sina.com

Keywords：

Marfan syndrome; left ventricular dysfunction; aortic dissection; surgical management

DOI：

10.7507/1007-4848.201801005

Video：

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Objective To retrospectively reviewed our experience of the surgical and perioperative treatment of patients suffering from critical Marfan syndrome with severe left ventricular dysfunction and to evaluate its therapeutic effect and prognosis. Methods Between January 2012 and October 2016, 15 patients diagnosed with Marfan syndrome combined with severe left ventricular dysfunction (left ventricular ejection fraction≤40% or left ventricular end diastolic diameter≥75 mm) underwent operations for aortic root aneurysm in Zhujiang Hospital and Guangdong General Hospital. Among them, 11 were males and 4 were females with a mean age of 32.9±8.7 years ranging from 19 to 55 years. Five patients with aortic dissection underwent Bentall procedure and total arch reconstruction with stent graft implantation. Two patients underwent Bentall procedure and hemi-arch replacement, seven patients underwent Bentall procedure and one patient underwent Cabrol procedure. Concomitant procedures included mitral valve repair in 12 patients, mitral valve replacement in 3 patients and tricuspid valve repair in 12 patients. Results There were 11 patients (73.3%) receiving intra-aortic balloon pumping implantation. One (6.7%) in-hospital death occurred. The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 58.3±6.0 mm (P<0.05) and the left ventricular ejection fraction improved from 37.3%±5.2% to 46.3%±4.4% 3 months postoperatively (P<0.05). The left ventricular end diastolic diameter decreased from 80.5±7.4 mm to 53.7±3.6 mm (P<0.05) and the left ventricular ejection fraction improved from 37.3%±5.2% to 57.7%±4.2% after one year (P<0.05). No death and reoperation occurred in the follow-up. Conclusion Although the patients with Marfan syndrome and severe left ventricular dysfunction usually have a high surgical mortality, the key to satisfactory outcomes of severe Marfan syndrome is adequate preoperative preparation, complete correction of all vascular lesions during the operation, application of circulatory auxiliary device and perioperative strict and long-term ICU monitoring.

Citation： XU Wenliu, FAN Xiaoping, HUANG Jingsong, ZHANG Mingsheng, PENG Jihai, CAI Shihao, HE Jie, CHEN Qunqing. Clinical strategy of surgical management for Marfan syndrome in patients with severe left ventricular dysfunction. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2018, 25(6): 507-511. doi: 10.7507/1007-4848.201801005 Copy

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2.	Judge DP, Dietz HC. Marfan's syndrome. Lancet, 2005, 366(9501): 1965-1976.
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.
4.	Fleischer KJ, Nousari HC, Anhalt GJ, et al. Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan's syndrome. Ann Thorac Surg, 1997, 63(4): 1012-1017.
5.	Weyman AE, Scherrer-Crosbie M. Marfan syndrome and mitral valve prolapse. J Clin Invest, 2004, 114(11): 1543-1546.
6.	Westaby S, Katsumata T, Vaccari G. Aortic root replacement with coronary button re-implantation: low risk and predictable outcome. Eur J Cardiothorac Surg, 2000, 17(3): 259-265.
7.	Chowdhury UK, Kumar AS, Airan B, et al. Mitral valve replacement with and without chordal preservation in a rheumatic population: serial echocardiographic assessment of left ventricular size and function. Ann Thorac Surg, 2005, 79(6): 1926-1933.
8.	García-Fuster R, Estevez V, Gil O, et al. Mitral valve replacement in rheumatic patients: effects of chordal preservation. Ann Thorac Surg, 2008, 86(2): 472-481.
9.	Dunkman WB, Leinbach RC, Buckley MJ, et al. Clinical and hemodynamic results of intraaortic balloon pumping and surgery for cardiogenic shock. Circulation, 1972, 46(3): 465-477.
10.	Pennington DG, Swartz M, Codd JE, et al. Intraaortic balloon pumping in cardiac surgical patients: a nine-year experience. Ann Thorac Surg, 1983, 36(2): 125-131.
11.	Baskett RJ, Ghali WA, Maitland A, et al. The intraaortic balloon pump in cardiac surgery. Ann Thorac Surg, 2002, 74(4): 1276-1287.

1. Dietz HC, Cutting GR, Pyeritz RE, et al. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature, 1991, 352(6333): 337-339.
2. Judge DP, Dietz HC. Marfan's syndrome. Lancet, 2005, 366(9501): 1965-1976.
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.
4. Fleischer KJ, Nousari HC, Anhalt GJ, et al. Immunohistochemical abnormalities of fibrillin in cardiovascular tissues in Marfan's syndrome. Ann Thorac Surg, 1997, 63(4): 1012-1017.
5. Weyman AE, Scherrer-Crosbie M. Marfan syndrome and mitral valve prolapse. J Clin Invest, 2004, 114(11): 1543-1546.
6. Westaby S, Katsumata T, Vaccari G. Aortic root replacement with coronary button re-implantation: low risk and predictable outcome. Eur J Cardiothorac Surg, 2000, 17(3): 259-265.
7. Chowdhury UK, Kumar AS, Airan B, et al. Mitral valve replacement with and without chordal preservation in a rheumatic population: serial echocardiographic assessment of left ventricular size and function. Ann Thorac Surg, 2005, 79(6): 1926-1933.
8. García-Fuster R, Estevez V, Gil O, et al. Mitral valve replacement in rheumatic patients: effects of chordal preservation. Ann Thorac Surg, 2008, 86(2): 472-481.
9. Dunkman WB, Leinbach RC, Buckley MJ, et al. Clinical and hemodynamic results of intraaortic balloon pumping and surgery for cardiogenic shock. Circulation, 1972, 46(3): 465-477.
10. Pennington DG, Swartz M, Codd JE, et al. Intraaortic balloon pumping in cardiac surgical patients: a nine-year experience. Ann Thorac Surg, 1983, 36(2): 125-131.
11. Baskett RJ, Ghali WA, Maitland A, et al. The intraaortic balloon pump in cardiac surgery. Ann Thorac Surg, 2002, 74(4): 1276-1287.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Clinical strategy of surgical management for Marfan syndrome in patients with severe left ventricular dysfunction

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