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Canadian Journal of Cardiology

Canadian Cardiovascular Society Position Statement on the Management of Thoracic Aortic Disease

Published:March 03, 2014DOI:https://doi.org/10.1016/j.cjca.2014.02.018

      Abstract

      This Canadian Cardiovascular Society position statement aims to provide succinct perspectives on key issues in the management of thoracic aortic disease (TAD). This document is not a comprehensive overview of TAD and important elements of the epidemiology, presentation, diagnosis, and management of acute aortic syndromes are deliberately not discussed; readers are referred to the 2010 guidelines published by the American Heart Association, American College of Cardiology, American Association for Thoracic Surgery, and other stakeholders. Rather, this document is a practical guide for clinicians managing adult patients with TAD. Topics covered include size thresholds for surgical intervention, emerging therapies, imaging modalities, medical and lifestyle management, and genetics of TAD. The primary panel consisted of experts from a variety of disciplines that are essential for comprehensive management of TAD patients. The methodology involved a focused literature review with an emphasis on updates since 2010 and the use of Grading of Recommendations Assessment, Development, and Evaluation methodology to arrive at specific recommendations. The final document then underwent review by a secondary panel. This document aims to provide recommendations for most patients and situations. However, the ultimate judgement regarding the management of any individual patients should be made by their health care team.

      Résumé

      Cet énoncé de position de la Société canadienne de cardiologie a pour but de donner des perspectives succinctes sur les aspects clés de la prise en charge de la maladie de l’aorte thoracique (MAT). Ce document n’est pas un aperçu complet de la MAT, et les éléments importants de l’épidémiologie, du tableau clinique, du diagnostic et de la prise en charge des syndromes aortiques aigus ne sont délibérément pas discutés; les lecteurs sont invités à consulter les lignes directrices de 2010 publiées par l’American Heart Association, l’American College of Cardiology, l’American Association for Thoracic Surgery et les autres parties prenantes. Ce document est plutôt un guide pratique pour les cliniciens prenant en charge des patients adultes atteints d’une MAT. Les thèmes couverts incluent les seuils de l’ampleur de l’intervention chirurgicale, les nouveaux traitements, les modalités d’imagerie, la prise en charge médicale et du mode de vie, et la génétique de la MAT. Le premier panel comprenait des experts de diverses disciplines qui sont essentielles à la prise en charge complète des patients atteints d’une MAT. La méthodologie exigeait une revue de littérature ciblée s’appuyant sur les mises à jour depuis 2010 et l’utilisation de la méthodologie GRACE (Grading of Recommendations Assessment, Development, and Evaluation) pour parvenir à des recommandations précises. Le document final était ensuite passé en revue par un second panel. Ce document a pour but de fournir des recommandations pour la plupart des patients et la plupart des situations. Cependant, la décision définitive concernant la prise en charge de chaque patient devrait être prise par l’équipe soignante.
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      References

        • Masuda Y.
        • Yamada Z.
        • Morooka N.
        • Watanabe S.
        • Inagaki Y.
        Prognosis of patients with medically treated aortic dissections.
        Circulation. 1991; 84: III7-13
        • Rampoldi V.
        • Trimarchi S.
        • Eagle K.A.
        • et al.
        Simple risk models to predict surgical mortality in acute type A aortic dissection: the International Registry of Acute Aortic Dissection score.
        Ann Thorac Surg. 2007; 83: 55-61
        • Pape L.A.
        • Tsai T.T.
        • Isselbacher E.M.
        • et al.
        Aortic diameter > or = 5.5 cm is not a good predictor of type A aortic dissection: observations from the International Registry of Acute Aortic Dissection (IRAD).
        Circulation. 2007; 116: 1120-1127
        • Trimarchi S.
        • Jonker F.H.
        • Hutchison S.
        • et al.
        Descending aortic diameter of 5.5 cm or greater is not an accurate predictor of acute type B aortic dissection.
        J Thorac Cardiovasc Surg. 2011; 142: e101-e107
        • Davies R.R.
        • Goldstein L.J.
        • Coady M.A.
        • et al.
        Yearly rupture or dissection rates for thoracic aortic aneurysms: simple prediction based on size.
        Ann Thorac Surg. 2002; 73 ([discussion 27-18]): 17-27
        • Coady M.A.
        • Rizzo J.A.
        • Hammond G.L.
        • Kopf G.S.
        • Elefteriades J.A.
        Surgical intervention criteria for thoracic aortic aneurysms: a study of growth rates and complications.
        Ann Thorac Surg. 1999; 67 ([discussion: 1953-8]): 1922-1926
        • Michelena H.I.
        • Desjardins V.A.
        • Avierinos J.F.
        • et al.
        Natural history of asymptomatic patients with normally functioning or minimally dysfunctional bicuspid aortic valve in the community.
        Circulation. 2008; 117: 2776-2784
        • Tzemos N.
        • Therrien J.
        • Yip J.
        • et al.
        Outcomes in adults with bicuspid aortic valves.
        JAMA. 2008; 300: 1317-1325
        • Michelena H.I.
        • Khanna A.D.
        • Mahoney D.
        • et al.
        Incidence of aortic complications in patients with bicuspid aortic valves.
        JAMA. 2011; 306: 1104-1112
        • Jondeau G.
        • Detaint D.
        • Tubach F.
        • et al.
        Aortic event rate in the Marfan population: a cohort study.
        Circulation. 2012; 125: 226-232
        • Roman M.J.
        • Rosen S.E.
        • Kramer-Fox R.
        • Devereux R.B.
        Prognostic significance of the pattern of aortic root dilation in the Marfan syndrome.
        J Am Coll Cardiol. 1993; 22: 1470-1476
        • Albornoz G.
        • Coady M.A.
        • Roberts M.
        • et al.
        Familial thoracic aortic aneurysms and dissections–incidence, modes of inheritance, and phenotypic patterns.
        Ann Thorac Surg. 2006; 82: 1400-1405
        • Coady M.A.
        • Davies R.R.
        • Roberts M.
        • et al.
        Familial patterns of thoracic aortic aneurysms.
        Arch Surg. 1999; 134: 361-367
        • Loeys B.L.
        • Schwarze U.
        • Holm T.
        • et al.
        Aneurysm syndromes caused by mutations in the TGF-beta receptor.
        N Engl J Med. 2006; 355: 788-798
        • Trimarchi S.
        • Eagle K.A.
        • Nienaber C.A.
        • et al.
        Importance of refractory pain and hypertension in acute type B aortic dissection: insights from the International Registry of Acute Aortic Dissection (IRAD).
        Circulation. 2010; 122: 1283-1289
        • Lobato A.C.
        • Puech-Leao P.
        Predictive factors for rupture of thoracoabdominal aortic aneurysm.
        J Vasc Surg. 1998; 27: 446-453
        • Svensson L.G.
        • Kim K.H.
        • Blackstone E.H.
        • et al.
        Bicuspid aortic valve surgery with proactive ascending aorta repair.
        J Thorac Cardiovasc Surg. 2011; 142 (629.e621-3): 622-629
        • McKellar S.H.
        • Michelena H.I.
        • Li Z.
        • Schaff H.V.
        • Sundt 3rd, T.M.
        Long-term risk of aortic events following aortic valve replacement in patients with bicuspid aortic valves.
        Am J Cardiol. 2010; 106: 1626-1633
        • Russo C.F.
        • Mazzetti S.
        • Garatti A.
        • et al.
        Aortic complications after bicuspid aortic valve replacement: long-term results.
        Ann Thorac Surg. 2002; 74 ([discussion S1792-9]): S1773-S1776
        • Davies R.R.
        • Gallo A.
        • Coady M.A.
        • et al.
        Novel measurement of relative aortic size predicts rupture of thoracic aortic aneurysms.
        Ann Thorac Surg. 2006; 81: 169-177
        • Svensson L.G.
        • Khitin L.
        Aortic cross-sectional area/height ratio timing of aortic surgery in asymptomatic patients with Marfan syndrome.
        J Thorac Cardiovasc Surg. 2002; 123: 360-361
        • Patel V.I.
        • Mukhopadhyay S.
        • Ergul E.
        • et al.
        Impact of hospital volume and type on outcomes of open and endovascular repair of descending thoracic aneurysms in the United States Medicare population.
        J Vasc Surg. 2013; 58: 346-354
        • Cheng D.
        • Martin J.
        • Shennib H.
        • et al.
        Endovascular aortic repair versus open surgical repair for descending thoracic aortic disease a systematic review and meta-analysis of comparative studies.
        J Am Coll Cardiol. 2010; 55: 986-1001
        • Goodney P.P.
        • Travis L.
        • Lucas F.L.
        • et al.
        Survival after open versus endovascular thoracic aortic aneurysm repair in an observational study of the Medicare population.
        Circulation. 2011; 124: 2661-2669
        • Lu Q.
        • Feng J.
        • Zhou J.
        • et al.
        Endovascular repair of ascending aortic dissection: a novel treatment option for patients judged unfit for direct surgical repair.
        J Am Coll Cardiol. 2013; 61: 1917-1924
        • Metcalfe M.J.
        • Karthikesalingam A.
        • Black S.A.
        • et al.
        The first endovascular repair of an acute type A dissection using an endograft designed for the ascending aorta.
        J Vasc Surg. 2012; 55: 220-222
        • Tsai T.T.
        • Trimarchi S.
        • Nienaber C.A.
        Acute aortic dissection: perspectives from the International Registry of Acute Aortic Dissection (IRAD).
        Eur J Vasc Endovasc Surg. 2009; 37: 149-159
        • Lombardi J.V.
        • Cambria R.P.
        • Nienaber C.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 (e622): 629-640
        • Nienaber C.A.
        • Kische S.
        • Akin I.
        • et al.
        Strategies for subacute/chronic type B aortic dissection: the Investigation Of Stent Grafts in Patients with type B Aortic Dissection (INSTEAD) trial 1-year outcome.
        J Thorac Cardiovasc Surg. 2010; 140 ([discussion: S142-6]): S101-S108
        • Nienaber C.A.
        • Rousseau H.
        • Eggebrecht H.
        • et al.
        Randomized comparison of strategies for type B aortic dissection: the INvestigation of STEnt Grafts in Aortic Dissection (INSTEAD) trial.
        Circulation. 2009; 120: 2519-2528
        • Nienaber C.A.
        • Kische S.
        • Rousseau H.
        • 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 Interv. 2013; 6: 407-416
        • Coselli J.S.
        • LeMaire S.A.
        • Koksoy C.
        • Schmittling Z.C.
        • Curling P.E.
        Cerebrospinal fluid drainage reduces paraplegia after thoracoabdominal aortic aneurysm repair: results of a randomized clinical trial.
        J Vasc Surg. 2002; 35: 631-639
        • Hnath J.C.
        • Mehta M.
        • Taggert J.B.
        • et al.
        Strategies to improve spinal cord ischemia in endovascular thoracic aortic repair: outcomes of a prospective cerebrospinal fluid drainage protocol.
        J Vasc Surg. 2008; 48: 836-840
        • Bley T.A.
        • Duffek C.C.
        • Francois C.J.
        • et al.
        Presurgical localization of the artery of Adamkiewicz with time-resolved 3.0-T MR angiography.
        Radiology. 2010; 255: 873-881
        • Akins C.W.
        • Miller D.C.
        • Turina M.I.
        • et al.
        Guidelines for reporting mortality and morbidity after cardiac valve interventions.
        J Thorac Cardiovasc Surg. 2008; 135: 732-738
        • David T.E.
        • Armstrong S.
        • Manlhiot C.
        • McCrindle B.W.
        • Feindel C.M.
        Long-term results of aortic root repair using the reimplantation technique.
        J Thorac Cardiovasc Surg. 2013; 145: S22-S25
        • Boodhwani M.
        • de Kerchove L.
        • Glineur D.
        • et al.
        Repair-oriented classification of aortic insufficiency: impact on surgical techniques and clinical outcomes.
        J Thorac Cardiovasc Surg. 2009; 137: 286-294
        • Boodhwani M.
        • de Kerchove L.
        • Glineur D.
        • El Khoury G.
        A simple method for the quantification and correction of aortic cusp prolapse by means of free margin plication.
        J Thorac Cardiovasc Surg. 2010; 139: 1075-1077
        • Aicher D.
        • Kunihara T.
        • Abou Issa O.
        • et al.
        Valve configuration determines long-term results after repair of the bicuspid aortic valve.
        Circulation. 2011; 123: 178-185
        • Boodhwani M.
        • de Kerchove L.
        • Glineur D.
        • et al.
        Repair of regurgitant bicuspid aortic valves: a systematic approach.
        J Thorac Cardiovasc Surg. 2010; 140: 276-284
        • Aicher D.
        • Fries R.
        • Rodionycheva S.
        • et al.
        Aortic valve repair leads to a low incidence of valve-related complications.
        Eur J Cardiothorac Surg. 2010; 37: 127-132
        • Price J.
        • De Kerchove L.
        • Glineur D.
        • et al.
        Risk of valve-related events after aortic valve repair.
        Ann Thorac Surg. 2013; 95 ([discussion: 613]): 606-612
        • ICRP
        Recommendations of the International Commission on Radiological Protection.
        Ann ICRP. 1977; 1: 1-53
        • Brenner D.J.
        • Hall E.J.
        Computed tomography–an increasing source of radiation exposure.
        N Engl J Med. 2007; 357: 2277-2284
        • Cascade P.N.
        • Leibel S.A.
        Decision-making in radiotherapy for the cancer patient: the American College of Radiology Appropriateness Criteria Project.
        CA Cancer J Clin. 1998; 48: 146-150
        • McCollough C.H.
        • Bruesewitz M.R.
        • Kofler Jr., J.M.
        CT dose reduction and dose management tools: overview of available options.
        Radiographics. 2006; 26: 503-512
        • Parker M.S.
        • Matheson T.L.
        • Rao A.V.
        • et al.
        Making the transition: the role of helical CT in the evaluation of potentially acute thoracic aortic injuries.
        AJR Am J Roentgenol. 2001; 176: 1267-1272
        • Scheske J.A.
        • O'Brien J.M.
        • Earls J.P.
        • et al.
        Coronary artery imaging with single-source rapid kilovolt peak-switching dual-energy CT.
        Radiology. 2013; 268: 702-709
        • So A.
        • Lee T.Y.
        • Imai Y.
        • et al.
        Quantitative myocardial perfusion imaging using rapid kVp switch dual-energy CT: preliminary experience.
        J Cardiovasc Comput Tomogr. 2011; 5: 430-442
        • Lang R.M.
        • Bierig M.
        • Devereux R.B.
        • et al.
        Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology.
        J Am Soc Echocardiogr. 2005; 18: 1440-1463
        • Picard M.H.
        • Adams D.
        • Bierig S.M.
        • et al.
        American Society of Echocardiography recommendations for quality echocardiography laboratory operations.
        J Am Soc Echocardiogr. 2011; 24: 1-10
        • Lang R.M.
        • Badano L.P.
        • Tsang W.
        • et al.
        EAE/ASE recommendations for image acquisition and display using three-dimensional echocardiography.
        J Am Soc Echocardiogr. 2012; 25: 3-46
        • Hiratzka L.F.
        • Bakris G.L.
        • Beckman J.A.
        • 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: e266-e369
        • Shiga T.
        • Wajima Z.
        • Apfel C.C.
        • Inoue T.
        • Ohe Y.
        Diagnostic accuracy of transesophageal echocardiography, helical computed tomography, and magnetic resonance imaging for suspected thoracic aortic dissection: systematic review and meta-analysis.
        Arch Intern Med. 2006; 166: 1350-1356
        • Elefteriades J.A.
        Does medical therapy for thoracic aortic aneurysms really work? Are beta-blockers truly indicated? PRO.
        Cardiol Clin. 2010; 28: 255-260
        • Boucek R.J.
        • Gunja-Smith Z.
        • Noble N.L.
        • Simpson C.F.
        Modulation by propranolol of the lysyl cross-links in aortic elastin and collagen of the aneurysm-prone turkey.
        Biochem Pharmacol. 1983; 32: 275-280
        • Suzuki T.
        • Mehta R.H.
        • Ince H.
        • et al.
        Clinical profiles and outcomes of acute type B aortic dissection in the current era: lessons from the International Registry of Aortic Dissection (IRAD).
        Circulation. 2003; 108: II312-II317
        • Mehta R.H.
        • O'Gara P.T.
        • Bossone E.
        • et al.
        Acute type A aortic dissection in the elderly: clinical characteristics, management, and outcomes in the current era.
        J Am Coll Cardiol. 2002; 40: 685-692
        • Law M.R.
        • Morris J.K.
        • Wald N.J.
        Use of blood pressure lowering drugs in the prevention of cardiovascular disease: meta-analysis of 147 randomised trials in the context of expectations from prospective epidemiological studies.
        BMJ. 2009; 338: b1665
        • Neal B.
        • MacMahon S.
        • Chapman N.
        Blood Pressure Lowering Treatment Trialists Collaboration. Effects of ACE inhibitors, calcium antagonists, and other blood-pressure-lowering drugs: results of prospectively designed overviews of randomised trials. Blood Pressure Lowering Treatment Trialists' Collaboration.
        Lancet. 2000; 356: 1955-1964
        • Arguedas J.A.
        • Perez M.I.
        • Wright J.M.
        Treatment blood pressure targets for hypertension.
        Cochrane Database Syst Rev. 2009; : CD004349
        • Batterink J.
        • Stabler S.N.
        • Tejani A.M.
        • Fowkes C.T.
        Spironolactone for hypertension.
        Cochrane Database Syst Rev. 2010; : CD008169
        • Chen J.M.
        • Heran B.S.
        • Wright J.M.
        Blood pressure lowering efficacy of diuretics as second-line therapy for primary hypertension.
        Cochrane Database Syst Rev. 2009; : CD007187
        • Chen N.
        • Zhou M.
        • Yang M.
        • et al.
        Calcium channel blockers versus other classes of drugs for hypertension.
        Cochrane Database Syst Rev. 2010; : CD003654
        • Heran B.S.
        • Galm B.P.
        • Wright J.M.
        Blood pressure lowering efficacy of alpha blockers for primary hypertension.
        Cochrane Database Syst Rev. 2009; : CD004643
        • Heran B.S.
        • Wong M.M.
        • Heran I.K.
        • Wright J.M.
        Blood pressure lowering efficacy of angiotensin converting enzyme (ACE) inhibitors for primary hypertension.
        Cochrane Database Syst Rev. 2008; : CD003823
        • Heran B.S.
        • Wong M.M.
        • Heran I.K.
        • Wright J.M.
        Blood pressure lowering efficacy of angiotensin receptor blockers for primary hypertension.
        Cochrane Database Syst Rev. 2008; : CD003822
        • Wiysonge C.S.
        • Bradley H.A.
        • Volmink J.
        • et al.
        Beta-blockers for hypertension.
        Cochrane Database Syst Rev. 2012; : CD002003
        • Wright J.M.
        • Musini V.M.
        First-line drugs for hypertension.
        Cochrane Database Syst Rev. 2009; : CD001841
        • Groenink M.
        • den Hartog A.W.
        • Franken R.
        • et al.
        Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial.
        Eur Heart J. 2013; 34: 3491-3500
        • Genoni M.
        • Paul M.
        • Jenni R.
        • et al.
        Chronic beta-blocker therapy improves outcome and reduces treatment costs in chronic type B aortic dissection.
        Eur J Cardiothorac Surg. 2001; 19: 606-610
        • Suzuki T.
        • Isselbacher E.M.
        • Nienaber C.A.
        • et al.
        Type-selective benefits of medications in treatment of acute aortic dissection (from the International Registry of Acute Aortic Dissection [IRAD]).
        Am J Cardiol. 2012; 109: 122-127
        • Jonker F.H.
        • Trimarchi S.
        • Rampoldi V.
        • et al.
        Aortic expansion after acute type B aortic dissection.
        Ann Thorac Surg. 2012; 94: 1223-1229
        • Takeshita S.
        • Sakamoto S.
        • Kitada S.
        • Akutsu K.
        • Hashimoto H.
        Angiotensin-converting enzyme inhibitors reduce long-term aortic events in patients with acute type B aortic dissection.
        Circ J. 2008; 72: 1758-1761
        • Grajek S.
        • Cieslinski A.
        • Mitkowski P.
        • et al.
        Results of long-term medical treatment of patients with arterial hypertension complicated by aortic dissection.
        J Hum Hypertens. 1995; 9: 987-992
        • Hackam D.G.
        • Quinn R.R.
        • Ravani P.
        • et al.
        The 2013 Canadian Hypertension Education Program recommendations for blood pressure measurement, diagnosis, assessment of risk, prevention, and treatment of hypertension.
        Can J Cardiol. 2013; 29: 528-542
        • Anderson T.J.
        • Gregoire J.
        • Hegele R.A.
        • et al.
        2012 update of the Canadian Cardiovascular Society guidelines for the diagnosis and treatment of dyslipidemia for the prevention of cardiovascular disease in the adult.
        Can J Cardiol. 2013; 29: 151-167
        • Hatzaras I.
        • Tranquilli M.
        • Coady M.
        • et al.
        Weight lifting and aortic dissection: more evidence for a connection.
        Cardiology. 2007; 107: 103-106
        • Williams M.A.
        • Haskell W.L.
        • Ades P.A.
        • et al.
        Resistance exercise in individuals with and without cardiovascular disease: 2007 update: a scientific statement from the American Heart Association Council on Clinical Cardiology and Council on Nutrition, Physical Activity, and Metabolism.
        Circulation. 2007; 116: 572-584
        • Heran B.S.
        • Chen J.M.
        • Ebrahim S.
        • et al.
        Exercise-based cardiac rehabilitation for coronary heart disease.
        Cochrane Database Syst Rev. 2011; : CD001800
        • Corone S.
        • Iliou M.C.
        • Pierre B.
        • et al.
        French registry of cases of type I acute aortic dissection admitted to a cardiac rehabilitation center after surgery.
        Eur J Cardiovasc Prev Rehabil. 2009; 16: 91-95
      1. Canadian Medical Association. CMA Driver's Guide: determining medical fitness to operate motor vehicles. 8th Ed. Toronto: Canadian Medical Association, 2012.

        • Coady M.A.
        • Rizzo J.A.
        • Hammond G.L.
        • et al.
        What is the appropriate size criterion for resection of thoracic aortic aneurysms?.
        J Thorac Cardiovasc Surg. 1997; 113 ([discussion: 489-91]): 476-491
        • Loeys B.L.
        • Dietz H.C.
        • Braverman A.C.
        • et al.
        The revised Ghent nosology for the Marfan syndrome.
        J Med Genet. 2010; 47: 476-485
        • Jondeau G.
        • Boileau C.
        Genetics of thoracic aortic aneurysms.
        Curr Atheroscler Rep. 2012; 14: 219-226
        • Barrett P.
        • Topol E.J.
        The fibrillin-1 gene: unlocking new therapeutic pathways in cardiovascular disease.
        Heart. 2013; 99: 83-90
        • Williams J.A.
        • Loeys B.L.
        • Nwakanma L.U.
        • et al.
        Early surgical experience with Loeys-Dietz: a new syndrome of aggressive thoracic aortic aneurysm disease.
        Ann Thorac Surg. 2007; 83 ([discussion: S785-90]): S757-S763
        • Kalra V.B.
        • Gilbert J.W.
        • Malhotra A.
        Loeys-Dietz syndrome: cardiovascular, neuroradiological and musculoskeletal imaging findings.
        Pediatr Radiol. 2011; 41 ([quiz: 1616]): 1495-1504
        • Biner S.
        • Rafique A.M.
        • Ray I.
        • et al.
        Aortopathy is prevalent in relatives of bicuspid aortic valve patients.
        J Am Coll Cardiol. 2009; 53: 2288-2295
        • Kerstjens-Frederikse W.S.
        • Du Marchie Sarvaas G.J.
        • Ruiter J.S.
        • et al.
        Left ventricular outflow tract obstruction: should cardiac screening be offered to first-degree relatives?.
        Heart. 2011; 97: 1228-1232
        • Cripe L.
        • Andelfinger G.
        • Martin L.J.
        • Shooner K.
        • Benson D.W.
        Bicuspid aortic valve is heritable.
        J Am Coll Cardiol. 2004; 44: 138-143
        • McBride K.L.
        • Pignatelli R.
        • Lewin M.
        • et al.
        Inheritance analysis of congenital left ventricular outflow tract obstruction malformations: segregation, multiplex relative risk, and heritability.
        Am J Med Genet A. 2005; 134: 180-186
        • Huntington K.
        • Hunter A.G.
        • Chan K.L.
        A prospective study to assess the frequency of familial clustering of congenital bicuspid aortic valve.
        J Am Coll Cardiol. 1997; 30: 1809-1812
        • Loscalzo M.L.
        • Goh D.L.
        • Loeys B.
        • et al.
        Familial thoracic aortic dilation and bicommissural aortic valve: a prospective analysis of natural history and inheritance.
        Am J Med Genet A. 2007; 143A: 1960-1967
        • Liotta R.
        • Chughtai A.
        • Agarwal P.P.
        Computed tomography angiography of thoracic aortic aneurysms.
        Semin Ultrasound CT MR. 2012; 33: 235-246
        • Tsai S.F.
        • Trivedi M.
        • Daniels C.J.
        Comparing imaging modalities for screening aortic complications in patients with bicuspid aortic valve.
        Congenit Heart Dis. 2012; 7: 372-377
        • Regalado E.S.
        • Guo D.C.
        • Villamizar C.
        • et al.
        Exome sequencing identifies SMAD3 mutations as a cause of familial thoracic aortic aneurysm and dissection with intracranial and other arterial aneurysms.
        Circ Res. 2011; 109: 680-686
        • van de Laar I.M.
        • Oldenburg R.A.
        • Pals G.
        • et al.
        Mutations in SMAD3 cause a syndromic form of aortic aneurysms and dissections with early-onset osteoarthritis.
        Nat Genet. 2011; 43: 121-126
        • van der Linde D.
        • Verhagen H.J.
        • Moelker A.
        • et al.
        Aneurysm-osteoarthritis syndrome with visceral and iliac artery aneurysms.
        J Vasc Surg. 2013; 57: 96-102
        • De Paepe A.
        • Malfait F.
        The Ehlers-Danlos syndrome, a disorder with many faces.
        Clin Genet. 2012; 82: 1-11
        • Bergqvist D.
        • Bjorck M.
        • Wanhainen A.
        Treatment of vascular Ehlers-Danlos syndrome: a systematic review.
        Ann Surg. 2013; 258: 257-261
        • Gravholt C.H.
        • Landin-Wilhelmsen K.
        • Stochholm K.
        • et al.
        Clinical and epidemiological description of aortic dissection in Turner's syndrome.
        Cardiol Young. 2006; 16: 430-436
        • Carlson M.
        • Airhart N.
        • Lopez L.
        • Silberbach M.
        Moderate aortic enlargement and bicuspid aortic valve are associated with aortic dissection in Turner syndrome: report of the International Turner Syndrome Aortic Dissection Registry.
        Circulation. 2012; 126: 2220-2226