Aortic Dissection in Patients with Disorders of Connective Tissue
Over the past decade, the medical community has slowly accepted the idea that patients presenting with aortic aneurysms and dissections are part of a wide spectrum of genetically mediated diseases that present in syndromic as well as non-syndromic forms. Marfan syndrome (MFS) has long been the only seriously considered differential diagnosis in terms of a heritable disorder of connective tissue in patients with aortic aneurysm.
While growing clinical experience in the treatment of patients with connective tissue disorders has improved surgical management and decision-making, the aorta has emerged as a distinct organ with specific susceptibilities and reactions to pathological stimuli. The spectrum of heritable disorders of aortic disease has certainly become broader necessitating a structured approach to this patient population.
KeywordsMarfan Loeys-Dietz Ehlers-Danlos Connective tissue disorder Aortic aneurysm Aortic dissection
- 14.Januzzi JL, Isselbacher EM, Fattori R, Cooper JV, Smith DE, Fang J, et al. International registry of aortic dissection (IRAD). Characterizing the young patient with aortic dissection: results from the international registry of aortic dissection (IRAD). J Am Coll Cardiol. 2004;43:665–9.CrossRefGoogle Scholar
- 20.Tagusari O, Ogino H, Kobayashi J, Bando K, Minatoya K, Sasaki H, Niwaya K, Okita Y, Ando M, Yagihara T, Kitamura S. Should the transverse aortic arch be replaced simultaneously with aortic root replacement for annuloaortic ectasia in Marfan syndrome? J Thorac Cardiovasc Surg. 2004;127:1373–80.CrossRefGoogle Scholar
- 21.Sun L, Li M, Zhu J, Liu Y, Chang Q, Zheng J, Qi R. 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:e85–91.CrossRefGoogle Scholar
- 31.Grabenwöger M, Alfonso F, Bachet J, Bonser R, Czerny M, Eggebrecht H, et al. Thoracic endovascular aortic repair (TEVAR) for the treatment of aortic diseases: a position statement from the European Association for Cardio-Thoracic Surgery (EACTS) and the European Society of Cardiology (ESC), in collaboration with the European Association of Percutaneous Cardiovascular Interventions (EAPCI). Eur J Cardiothorac Surg. 2012;42:17–24.CrossRefGoogle Scholar
- 34.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. Circulation. 2010;121:e266–369.Google Scholar
- 35.Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo RD, Eggebrecht H, et al. ESC Committee for practice guidelines.. 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;35:2873–926.CrossRefGoogle Scholar
- 36.Baumgartner H, Bonhoeffer P, De Groot NM, de Haan F, Deanfield JE, Galie N, et al. Task force on the management of grown-up congenital heart disease of the European Society of Cardiology (ESC); Association for European Paediatric Cardiology (AEPC); ESC Committee for Practice Guidelines (CPG). ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31:2915–57.CrossRefGoogle Scholar
- 38.Schoenhoff FS, Alejo D, Black JH, Crawford TC, Dietz HC, Grimm JC, et al. The Griepp paper - Management of the Aortic Arch in Loeys-Dietz syndrome. AATS aortic symposium 2016, New York City.Google Scholar