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J Am Coll Cardiol. 2016 Jun 14;67(23):2744-2754. doi: 10.1016/j.jacc.2016.03.570.

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

Author information

1
Weill Cornell Medical College, New York, New York. Electronic address: jww2001@med.cornell.edu.
2
Weill Cornell Medical College, New York, New York.
3
Biostatistics and Epidemiology Division, RTI International, Rockville, Maryland.
4
Department of Pediatric Cardiology, Johns Hopkins University, Baltimore, Maryland.
5
Institute of Genetic Medicine, Johns Hopkins University, Baltimore, Maryland.
6
Division of Medical Genetics, University of Pennsylvania, Philadelphia, Pennsylvania.
7
Division of Cardiovascular Surgery, University of Pennsylvania, Philadelphia, Pennsylvania.
8
Division of Cardiothoracic Surgery, Baylor College of Medicine and the Texas Heart Institute, Houston, Texas.
9
Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
10
Division of Medical Genetics, University of Texas Health Sciences Center, Houston, Texas.
11
Heart Research Center, Oregon Health & Sciences University, Portland, Oregon.
12
Division of Cardiothoracic Surgery, Oregon Health & Sciences University, Portland, Oregon.
13
Department of Pediatric Cardiology, Oregon Health & Sciences University, Portland, Oregon.
14
Division of Cardiovascular Research, Queens Medical Center, Honolulu, Hawaii.
15
National Institute on Aging, National Institutes of Health, Baltimore, Maryland.
16
Department of Cardiology, University of Michigan Health System, Ann Arbor, Michigan.
17
Cardiovascular Core Laboratory, MedStar Health Research Institute at Washington Hospital Center, Washington, DC.

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:

Marfan syndrome; aortic aneurysm; aortic dissection; bicuspid aortic valve

PMID:
27282895
PMCID:
PMC5040186
DOI:
10.1016/j.jacc.2016.03.570
[Indexed for MEDLINE]
Free PMC Article

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