Marfan's syndrome surgery

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Editors-In-Chief: William James Gibson, C. Michael Gibson, M.S., M.D.; Associate Editor(s)-in-Chief: Cafer Zorkun, M.D., Ph.D. [1] ; Assistant Editor-In-Chief: Cassandra Abueg, M.P.H. [2]


If the dilation of the aorta progresses to a significant diameter aneurysm, causes a dissection or a rupture, or leads to failure of the aortic or other valve, then surgery (possibly a composite aortic valve graft [CAVG] or valve-sparing procedure) becomes necessary.


Patients at high risk of aortic dissection may be counseled to undergo prophylactic aortic root replacement procedure. The 2010 ACC/AHA/AATS recommendations for indications for surgery include external diameter >50mm, rapid dilation (>5mm/year), a family history of aortic dissection at smaller diameters, the presence of aortic regurgitation, or extension of dilation beyond the sinuses of valsalva [1], [2]. The gold standard surgical intervention remains the technique described by Bentall and De Bono in 1968. The aortic root and valve are replaced with a composite Dacron graft and artificial valve. Patients treated with this procedure must take anticoagulants for the remainder of their lives. More recently, two valve-sparing procedures have been practiced: the aortic root remodeling procedure and the aortic valve reimplantation procedure. In the remodeling procedure, a graft is created containing three neosinuses and sutured slightly superior to the native valve. The reimplantation procedure reimplants the native valve into the Dakon graft and is thought to prevent future dilation, but is more technically demanding.

Although aortic graft surgery (or any vascular surgery) is a serious undertaking it is generally successful if undertaken on an elective basis. Surgery in the setting of acute aortic dissection or rupture is considerably more problematic. Elective aortic valve/graft surgery is usually considered when aortic root diameter reaches 50 millimetres, but each case needs to be specifically evaluated by a qualified cardiologist. New valve-sparing surgical techniques are becoming more common.[3] As Marfan patients live longer, other vascular repairs are becoming more common, e.g. repairs of descending thoractic aortic aneurysms and aneurysms of vessels other than the aorta.

The skeletal and ocular manifestations of Marfan syndrome can also be serious, although not life-threatening. These symptoms are usually treated in the typical manner for the appropriate condition. This can also affect height, arm length, and life span. The Nuss procedure is now being offered to people with Marfan syndrome to correct 'sunken chest' or (pectus excavatum).[4] Because Marfan may cause spinal abnormalities that are asymptomatic, any spinal surgery contemplated on a Marfan patient should only follow detailed imaging and careful surgical planning, regardless of the indication for surgery.

2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guideline Recommendations: Evaluation and Management of Acute Thoracic Aortic Disease (DO NOT EDIT)[2]

Genetic Syndromes (DO NOT EDIT)[2]

Class IIa
"1. If the maximal cross-sectional area in square centimeters of the ascending aorta or root divided by the patient's height in meters exceeds a ratio of 10, surgical repair is reasonable because shorter patients have dissection at a smaller size and 15% of patients with Marfan syndrome have dissection at a size smaller than 5.0 cm[5][6][7]. (Level of Evidence:C)"
"2. For women with Marfan syndrome contemplating pregnancy, it is reasonable to prophylactically replace the aortic root and ascending aorta if the diameter exceeds 4.0 cm[8]. (Level of Evidence:C)"


  1. Cañadas V, Vilacosta I, Bruna I, Fuster V (2010). "Marfan syndrome. Part 2: treatment and management of patients". Nature Reviews. Cardiology. 7 (5): 266–76. doi:10.1038/nrcardio.2010.31. PMID 20351702. Retrieved 2010-12-22. Unknown parameter |month= ignored (help)
  2. 2.0 2.1 2.2 Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE, Eagle KA, Hermann LK, Isselbacher EM, Kazerooni EA, Kouchoukos NT, Lytle BW, Milewicz DM, Reich DL, Sen S, Shinn JA, Svensson LG, Williams DM (2010). "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. 121 (13): e266–369. doi:10.1161/CIR.0b013e3181d4739e. PMID 20233780. Retrieved 2010-12-22. Unknown parameter |month= ignored (help)
  3. "Heart Surgery for Marfan Syndrome". Mayo Clinic.
  4. "Overview of the Nuss Procedure for Pectus Excavatum". Children's Hospital of The King's Daughters.
  5. Svensson LG, Blackstone EH, Feng J; et al. (2007). "Are Marfan syndrome and marfanoid patients distinguishable on long-term follow-up?". Ann. Thorac. Surg. 83 (3): 1067–74. doi:10.1016/j.athoracsur.2006.10.062. PMID 17307461. Unknown parameter |month= ignored (help)
  6. Gott VL, Greene PS, Alejo DE; et al. (1999). "Replacement of the aortic root in patients with Marfan's syndrome". N. Engl. J. Med. 340 (17): 1307–13. doi:10.1056/NEJM199904293401702. PMID 10219065. Unknown parameter |month= ignored (help)
  7. Svensson LG, Khitin L (2002). "Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic patients with Marfan syndrome". J. Thorac. Cardiovasc. Surg. 123 (2): 360–1. PMID 11828302. Unknown parameter |month= ignored (help)
  8. Pearson GD, Devereux R, Loeys B; et al. (2008). "Report of the National Heart, Lung, and Blood Institute and National Marfan Foundation Working Group on research in Marfan syndrome and related disorders". Circulation. 118 (7): 785–91. doi:10.1161/CIRCULATIONAHA.108.783753. PMC 2909440. PMID 18695204. Unknown parameter |month= ignored (help)

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