Abstract
The goal of elective surgical repair of ascending aortic aneurysms is to prevent catastrophic aortic dissection and rupture. The timing of elective surgical repair for ascending aortic aneurysms is based on measurements of aortic diameter. The general threshold of 5.5cm is based on the observation that the incidence of aortic dissection and rupture rises significantly as aortic diameter approaches 6.0 cm or more. A variety of syndromic and non-syndromic connective tissue conditions predispose to aortic dissection and rupture at a smaller diameter. Identification of patients with these disorders is important as the aortic diameter threshold for recommending surgical repair must be lower than 5.5 cm. For an individual patient we must balance the risk of surgical repair, which is relatively low in experienced centers, against the risks of catastrophic dissection and rupture.
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References
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;121:266–369.
Wolak A, Gransar H, Thomson LJ, et al. Aortic size assessment by noncontrast cardiac computed tomography: normal limits by age, gender, and body surface area. J Am Coll Cardiol Imaging. 2008;1:200–9.
Hannuksela M, Lundqvist S, Carlberg B. Thoracic aorta: dilated or not? Scand Cardiovasc J. 2006;40:175–8.
Rogers IS, Massaro JM, Truong QA, Mahabadi AA, Kriegel MF, Fox CS, et al. Distribution, determinants, and normal reference values of thoracic and abdominal aortic diameters by computed tomography (from the Framingham Heart Study). Am J Cardiol. 2013;111:1510–6.
Daviess RR, Gallo A, Coady MA, Tellides G, Botta DM, Burke B, et al. Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms. Ann Thorac Surg. 2006;81:169–77.
Svensson LG, Khitin L. Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic patients with Marfan syndrome. J Thorac Cardiovasc Surg. 2002;123:360–1.
Svensson LG, Kim KH, Lytle BW, et al. Relationship of aortic cross-sectional area to height ratio and the risk of aortic dissection in patients with bicuspid aortic valves. J Thorac Cardiovasc Surg. 2003;126:892–3.
Elefteriades JA. Natural history of thoracic aortic aneurysms: indications for surgery, and surgical versus nonsurgical risks. Ann Thorac Surg. 2002;74:S1877–80.
Deitz HC, Pyeritz HE. Mutations in the human gene for fibrillin-1 (FBN1) in the Marfan syndrome and related disorders. Hum Mol Genet. 1995;4:S1799–809.
van Karnebeek CD, Naeff MS, Mulder BJ, et al. Natural history of cardiovascular manifestations in Marfan syndrome. Arch Dis Child. 2001;84:129–37.
Roman MJ, Rosen SE, Kramer-Fox R, et al. Prognostic significance of the pattern of aortic root dilation in the Marfan syndrome. J Am Coll Cardiol. 1993;22:1470–6.
Shores J, Berger KR, Murphy EA, et al. Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan’s syndrome. N Engl J Med. 1994;330:1335–41.
Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome. Circulation. 2005;111:150–7.
Rossiter JP, Repke JT, Morales AJ, et al. A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. Am J Obstet Gynecol. 1995;173:1599–606.
Sakaguchi M, Kitahara H, Watanabe T, et al. Successful surgical treatment for acute aortic dissection in pregnancy with Marfan’s syndrome. Jpn J Thorac Cardiovasc Surg. 2005;53:220–2.
Singh KK, Rommel K, Mishra A, et al. TGFBR1 and TGFBR2 mutations in patients with features of Marfan syndrome and Loeys-Dietz syndrome. Hum Mutat. 2006;27:770–7.
Loeys BL, Chen J, Neptune ER, et al. A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2. Nat Genet. 2005;37:275–81.
Loeys BL, Schwarze U, Holm T, et al. Aneurysm syndromes caused by mutations in the TGF-beta receptor. N Engl J Med. 2006;355:788–98.
Pepin M, Schwarze U, Superti-Furga A, et al. Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342:673–80.
Ascione R, Gomes WJ, Bates M, et al. Emergency repair of type A aortic dissection in type IV Ehlers-Danlos syndrome. Cardiovasc Surg. 2000;8:75–8.
Sybert VP. Cardiovascular malformations and complications in Turner syndrome. Pediatrics. 1998;101:11.
Gotzsche CO, Krag-Olsen B, Nielsen J, et al. Prevalence of cardiovascular malformations and association with karyotypes in Turner’s syndrome. Arch Dis Child. 1994;71:433–6.
Ostberg JE, Brookes JA, McCarthy C, et al. A comparison of echocardiography and magnetic resonance imaging in cardiovascular screening of adults with Turner syndrome. J Clin Endocrinol Metab. 2004;89:5966–71.
Lin AE, Silberbach M. Focus on the heart and aorta in Turner syndrome. J Pediatr. 2007;150:572–4.
Carlson M, Airhart N, Lopez L, Silberbach M. Moderate aortic enlargement and bicuspid aortic valve are associate with aortic dissection in Turner syndrome. Circulation. 2012;126:2220–6.
Matura LA, Ho VB, Rosing DR, Bondy CA. Aortic dilatation and dissection in Turner syndrome. Circulation. 2007;116:1663–70.
Bondy CA. Aortic dissection in Turner syndrome. Curr Opin Cardiol. 2008;23:519–26.
Svensson LG, Blackstone EH, Feng J, et al. Are Marfan syndrome and marfanoid patients distinguishable on long-term follow-up? Ann Thorac Surg. 2007;83:1067–74.
Wenstrup RJ, Meyer RA, Lyle JS, et al. Prevalence of aortic root dilation in the Ehlers-Danlos syndrome. Genet Med. 2002;4:112–7.
Gupta PA, Putnam EA, Carmical SG, et al. Ten novel FBN2 mutations in congenital contractural arachnodactyly: delineation of the molecular pathogenesis and clinical phenotype. Hum Mutat. 2002;19:39–48.
Gupta PA, Wallis DD, Chin TO, et al. FBN2 mutation associated with manifestations of Marfan syndrome and congenital contractural arachnodactyly. J Med Genet. 2004;41:56.
Adeola T, Adeleye O, Potts JL, et al. Thoracic aortic dissection in a patient with autosomal dominant polycystic kidney disease. J Natl Med Assoc. 2001;93:282–7.
Lee CC, Chang WT, Fang CC, et al. Sudden death caused by dissecting thoracic aortic aneurysm in a patient with autosomal dominant polycystic kidney disease. Resuscitation. 2004;63:93–6.
Purnell R, Williams I, Von Oppell U, et al. Giant aneurysms of the sinuses of Valsalva and aortic regurgitation in a patient with Noonan’s syndrome. Eur J Cardiothorac Surg. 2005;28:346–8.
Morgan JM, Coupe MO, Honey M, et al. Aneurysms of the sinuses of Valsalva in Noonan’s syndrome. Eur Heart J. 1989;10:190–3.
Shachter N, Perloff JK, Mulder DG. Aortic dissection in Noonan’s syndrome (46 XY turner). Am J Cardiol. 1984;54:464–5.
McElhinney DB, Krantz ID, Bason L, et al. Analysis of cardiovascular phenotype and genotype-phenotype correlation in individuals with a JAG1 mutation and/or Alagille syndrome. Circulation. 2002;106:2567–74.
Coady MA, Davies RR, Roberts M, et al. Familial patterns of thoracic aortic aneurysms. Arch Surg. 1999;134:361–7.
Biddinger A, Rocklin M, Coselli J, et al. Familial thoracic aortic dilatations and dissections: a case control study. J Vasc Surg. 1997;25:506–11.
Milewicz DM, Chen H, Park ES, et al. Reduced penetrance and variable expressivity of familial thoracic aortic aneurysms/dissections. Am J Cardiol. 1998;82:474–9.
Guo D, Pannu H, Tran-Fadula V, et al. Mutations in smooth muscle alpha-actin (ACTA2). Nat Genet. 2007;39:1488–93.
Zhu L, Vranckx R, Khau Van Kien P, et al. Mutations in myosin heavy chain 11 cause a syndrome associating thoracic aortic aneurysm/aortic dissection and patent ductus arteriosus. Nat Genet. 2006;38:343–9.
Guo DC, Papke CL, Tran-Fadulu V, et al. Mutations in smooth muscle alpha-actin (ACTA2) cause coronary artery disease, stroke, and moyamoya disease, along with thoracic aortic disease. Am J Hum Genet. 2009;84:617–27.
Guo D, Hasham S, Kuang SQ, et al. Familial thoracic aortic aneurysms and dissections: genetic heterogeneity with a major locus mapping to 5q13-14. Circulation. 2001;103:2461–8.
Hasham SN, Willing MC, Guo DC, et al. Mapping a locus for familial thoracic aortic aneurysms and dissections (TAAD2) to 3p24 –25. Circulation. 2003;107:3184–90.
Vaughan CJ, Casey M, He J, et al. Identification of a chromosome 11q23.2-q24 locus for familial aortic aneurysm disease, a genetically heterogeneous disorder. Circulation. 2001;103:2469–75.
Braverman AC, Guven H, Beardslee MA, et al. The bicuspid aortic valve. Curr Probl Cardiol. 2005;30:470–522.
Huntington K, Hunter AG, Chan KL. A prospective study to assess the frequency of familial clustering of congenital bicuspid aortic valve. J Am Coll Cardiol. 1997;30:1809–12.
Larson EW, Edwards WD. Risk factors for aortic dissection: a necropsy study of 161 cases. Am J Cardiol. 1984;53:849–55.
Michelena HI, Khanna AD, Mahoney D, et al. Incidence of aortic complications in patients with bicuspid aortic valves. JAMA. 2011;306:1104–13.
Svensson LG, Kim KH, Blackstone EH, et al. Bicuspid aortic valve surgery with proactive ascending aorta repair. J Thorac Cardiovasc Surg. 2011;142:622–9.
Svensson LG, Adams DH, Bonow RO, et al. Aortic valve and ascending aorta guidelines for management and quality measures: Executive summary. Ann Thorac Surg. 2013;95:1491–505.
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Hiratzka, L.F. (2019). Recommendations on Thresholds for Elective Surgery for Thoracic Ascending Aortic Aneurysms. In: Stanger, O., Pepper, J., Svensson, L. (eds) Surgical Management of Aortic Pathology. Springer, Vienna. https://doi.org/10.1007/978-3-7091-4874-7_59
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