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

on behalf of the GENTAC Registry Investigators

Research output: Contribution to journalArticle

24 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)2744-2754
Number of pages11
JournalJournal of the American College of Cardiology
Volume67
Issue number23
DOIs
StatePublished - 2016

Fingerprint

Aneurysm
Registries
Dissection
Thoracic Aortic Aneurysm
Incidence
Marfan Syndrome
Thoracic Aorta
Confidence Intervals
Abdominal Aorta
Aorta

Keywords

  • aortic aneurysm
  • aortic dissection
  • bicuspid aortic valve
  • Marfan syndrome

ASJC Scopus subject areas

  • Medicine(all)
  • Cardiology and Cardiovascular Medicine

Cite this

Aortic Dissection in Patients With Genetically Mediated Aneurysms : Incidence and Predictors in the GenTAC Registry. / on behalf of the GENTAC Registry Investigators.

In: Journal of the American College of Cardiology, Vol. 67, No. 23, 2016, p. 2744-2754.

Research output: Contribution to journalArticle

@article{de9d5b266c084922986a52d76b4573e4,
title = "Aortic Dissection in Patients With Genetically Mediated Aneurysms: Incidence and Predictors in the GenTAC Registry",
abstract = "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.",
keywords = "aortic aneurysm, aortic dissection, bicuspid aortic valve, Marfan syndrome",
author = "{on behalf of the GENTAC Registry Investigators} and Weinsaft, {Jonathan W.} and Devereux, {Richard B.} and Preiss, {Liliana R.} and Attila Feher and Roman, {Mary J.} and Basson, {Craig T.} and Alexi Geevarghese and William Ravekes and Dietz, {Harry C.} and Kathryn Holmes and Jennifer Habashi and Pyeritz, {Reed E.} and Joseph Bavaria and Karianna Milewski and LeMaire, {Scott A.} and Shaine Morris and Milewicz, {Dianna M.} and Siddharth Prakash and Cheryl Maslen and Howard Song and Silberbach, {Gary (Michael)} and Shohet, {Ralph V.} and Nazli McDonnell and Tabitha Hendershot and Eagle, {Kim A.} and Asch, {Federico M.}",
year = "2016",
doi = "10.1016/j.jacc.2016.03.570",
language = "English (US)",
volume = "67",
pages = "2744--2754",
journal = "Journal of the American College of Cardiology",
issn = "0735-1097",
publisher = "Elsevier USA",
number = "23",

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TY - JOUR

T1 - Aortic Dissection in Patients With Genetically Mediated Aneurysms

T2 - Incidence and Predictors in the GenTAC Registry

AU - on behalf of the GENTAC Registry Investigators

AU - Weinsaft, Jonathan W.

AU - Devereux, Richard B.

AU - Preiss, Liliana R.

AU - Feher, Attila

AU - Roman, Mary J.

AU - Basson, Craig T.

AU - Geevarghese, Alexi

AU - Ravekes, William

AU - Dietz, Harry C.

AU - Holmes, Kathryn

AU - Habashi, Jennifer

AU - Pyeritz, Reed E.

AU - Bavaria, Joseph

AU - Milewski, Karianna

AU - LeMaire, Scott A.

AU - Morris, Shaine

AU - Milewicz, Dianna M.

AU - Prakash, Siddharth

AU - Maslen, Cheryl

AU - Song, Howard

AU - Silberbach, Gary (Michael)

AU - Shohet, Ralph V.

AU - McDonnell, Nazli

AU - Hendershot, Tabitha

AU - Eagle, Kim A.

AU - Asch, Federico M.

PY - 2016

Y1 - 2016

N2 - 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.

AB - 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.

KW - aortic aneurysm

KW - aortic dissection

KW - bicuspid aortic valve

KW - Marfan syndrome

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DO - 10.1016/j.jacc.2016.03.570

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