Aortic Dissection in Patients With Genetically Mediated Aneurysms Incidence and Predictors in the GenTAC Registry

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:67

Article

Weinsaft, Jonathan W.¹  Devereux, Richard B.¹  Preiss, Liliana R.²  Feher, Attila¹  Roman, Mary J.¹  Basson, Craig T.¹  Geevarghese, Alexi¹  Ravekes, William³  Dietz, Harry C.⁴  Holmes, Kathryn³  Habashi, Jennifer³  Pyeritz, Reed E.⁵  Bavaria, Joseph⁶  Milewski, Karianna⁶  LeMaire, Scott A.^7,8  Morris, Shaine⁹  Milewicz, Dianna M.¹⁰  Prakash, Siddharth¹⁰  Maslen, Cheryl¹¹  Song, Howard K.¹²  Silberbach, G. Michael¹³  Shohet, Ralph V.¹⁴  McDonnell, Nazli¹⁵  Hendershot, Tabitha²  Eagle, Kim A.¹⁶  Asch, Federico M.¹⁷ 

[1] Weill Cornell Med Coll, 525 East 68th St, New York, NY 10021 USA

[2] RTI Int, Biostat & Epidemiol Div, Rockville, MD USA

[3] Johns Hopkins Univ, Dept Pediat Cardiol, Baltimore, MD USA

[4] Johns Hopkins Univ, Inst Med Genet, Baltimore, MD USA

[5] Univ Penn, Div Med Genet, Philadelphia, PA 19104 USA

[6] Univ Penn, Div Cardiovasc Surg, Philadelphia, PA 19104 USA

[7] Baylor Coll Med, Div Cardiothorac Surg, Houston, TX 77030 USA

[8] Texas Heart Inst, Houston, TX 77025 USA

[9] Baylor Coll Med, Dept Pediat, Houston, TX 77030 USA

[10] Univ Texas Hlth Sci Ctr Houston, Div Med Genet, Houston, TX 77030 USA

[11] Oregon Hlth & Sci Univ, Heart Res Ctr, Portland, OR 97201 USA

[12] Oregon Hlth & Sci Univ, Div Cardiothorac Surg, Portland, OR 97201 USA

[13] Oregon Hlth & Sci Univ, Dept Pediat Cardiol, Portland, OR 97201 USA

[14] Queens Med Ctr, Div Cardiovasc Res, Honolulu, HI USA

[15] NIA, NIH, Baltimore, MD 21224 USA

[16] Univ Michigan Hlth Syst, Dept Cardiol, Ann Arbor, MI USA

[17] Washington Hosp Ctr, MedStar Hlth Res Inst, Cardiovasc Core Lab, Washington, DC 20010 USA

关键词: aortic aneurysm;    aortic dissection;    bicuspid aortic valve;    Marfan syndrome;   

DOI  :  10.1016/j.jacc.2016.03.570

来源: Elsevier

【 摘 要 】

BACKGROUND Aortic dissection (AoD) is a serious complication of thoracic aortic aneurysm (TAA). Relative risk for AoD in relation to TAA etiology, incidence, and pattern after prophylactic TAA surgery are poorly understood. OBJECTIVES This study sought to determine the incidence, pattern, and relative risk for AoD among patients with genetically associated TAA. METHODS The population included adult GenTAC participants without AoD at baseline. Standardized core laboratory tests classified TAA etiology and measured aortic size. Follow-up was performed for AoD. RESULTS Bicuspid aortic valve (BAV) (39%) and Marfan syndrome (MFS) (22%) were the leading diagnoses in the studied GenTAC participants (n = 1,991). AoD occurred in 1.6% over 3.6 +/- 2.0 years; 61% of AoD occurred in patients with MFS. Cumulative AoD incidence was 6-fold higher among patients with MFS (4.5%) versus others (0.7%; p < 0.001). MFS event rates were similarly elevated versus those in patients with BAV (0.3%; p < 0.001). AoD originated in the distal arch or descending aorta in 71%; 52% of affected patients, including 68% with MFS, had previously undergone aortic grafting. In patients with proximal aortic surgery, distal aortic size (descending thoracic, abdominal aorta) was larger among patients with AoD versus those without AoD (both p < 0.05), whereas the ascending aorta size was similar. Conversely, in patients without previous surgery, aortic root size was greater in patients with subsequent AoD (p < 0.05), whereas distal aortic segments were of similar size. MFS (odds ratio: 7.42; 95% confidence interval: 3.43 to 16.82; p < 0.001) and maximal aortic size (1.86 per cm; 95% confidence interval: 1.26 to 2.67; p = 0.001) were independently associated with AoD. Only 4 of 31 (13%) patients with AoD had pre-dissection images that fulfilled size criteria for prophylactic TAA surgery at a subsequent AoD site. CONCLUSIONS Among patients with genetically associated TAA, MFS augments risk for AoD even after TAA grafting. Although increased aortic size is a risk factor for subsequent AoD, events typically occur below established thresholds for prophylactic TAA repair. (C) 2016 by the American College of Cardiology Foundation.

【 授权许可】

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