ArticleJournal of the American College of Cardiology2022
Cardiovascular Outcomes in Aortopathy: GenTAC Registry of Genetically Triggered Aortic Aneurysms and Related Conditions.
Article in Journal of the American College of Cardiology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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17 citing papers in PubMed, 1 synthesis or guideline pooled it, 30 citations in OpenAlex.
- Thoracic Aortic Aneurysm Growth Rates and Predicting Factors: A Systematic Review and Meta-Analysis.Journal of the American Heart Association · 2025Pooled it
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- Weighing the Risks: The Impact of Body Mass Index on Outcomes After Frozen Elephant Trunk Aortic Arch Repair.Medicina (Kaunas, Lithuania) · 2026Article
- Clinically Translatable Mutation-Based Biomarkers in Ascending Aortic Aneurysm: A Bibliometric Study.Human mutation · 2026Article
- Analysis of epidemiological characteristics and prognosis based on 1,343 cases of aortic dissection from a regional single center.Frontiers in cardiovascular medicine · 2026Article
- Outcomes of fenestrated-branched endovascular aortic repair of thoracoabdominal aortic aneurysms in patients with heritable thoracic aortic diseases.JTCVS techniques · 2025Article
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- The Cardiovascular Care of the Pediatric Athlete.Journal of the American College of Cardiology · 2025Review
- Outcome Following Open Repair of Hereditary and Non-Hereditary Thoracoabdominal Aortic Aneurysm in Patients Under 60 Years Old-A Multicenter Study.Journal of clinical medicine · 2025Article
- The proprotein convertase FURIN is a novel aneurysm predisposition gene impairing TGF-β signalling.Cardiovascular research · 2024Article
- Hereditary Thoracic Aortic Diseases.Diagnostics (Basel, Switzerland) · 2024Review
- Current understanding of the genetics of thoracic aortic disease.Vessel plus · 2024Article
- Outcomes of cardiothoracic surgery in women with Turner syndrome.Annals of cardiothoracic surgery · 2023Article
- Genetic Basis, New Diagnostic Approaches, and Updated Therapeutic Strategies of the Syndromic Aortic Diseases: Marfan, Loeys-Dietz, and Vascular Ehlers-Danlos Syndrome.International journal of environmental research and public health · 2023Review
- Association Between Genetic Diagnosis and Clinical Outcomes in Patients With Heritable Thoracic Aortic Disease.Journal of the American Heart Association · 2023Article
- Health disparities in Turner Syndrome: UTHealth Turner Syndrome Research Registry.Rare disease and orphan drugs journal · 2023Article
- Long-term outcomes in heritable thoracic aortic disease.Frontiers in cardiovascular medicine · 2022Article
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21 authors at 10 institutions in 1 country.
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Abstract
backgroundThe GenTAC (Genetically Triggered Thoracic Aortic Aneurysm and Cardiovascular Conditions) Registry enrolled patients with genetic aortopathies between 2007 and 2016.
objectivesThe purpose of this study was to compare age distribution and probability of elective surgery for proximal aortic aneurysm, any dissection surgery, and cardiovascular mortality among aortopathy etiologies.
methodsThe GenTAC study had a retrospective/prospective design. Participants with bicuspid aortic valve (BAV) with aneurysm (n = 879), Marfan syndrome (MFS) (n = 861), nonsyndromic heritable thoracic aortic disease (nsHTAD) (n = 378), Turner syndrome (TS) (n = 298), vascular Ehlers-Danlos syndrome (vEDS) (n = 149), and Loeys-Dietz syndrome (LDS) (n = 121) were analyzed.
resultsThe 25% probability of elective proximal aortic aneurysm surgery was 30 years for LDS (95% CI: 18-37 years), followed by MFS (34 years; 95% CI: 32-36 years), nsHTAD (52 years; 95% CI: 48-56 years), and BAV (55 years; 95% CI: 53-58 years). Any dissection surgery 25% probability was highest in LDS (38 years; 95% CI: 33-53 years) followed by MFS (51 years; 95% CI: 46-57 years) and nsHTAD (54 years; 95% CI: 51-61 years). BAV experienced the largest relative frequency of elective surgery to any dissection surgery (254/33 = 7.7), compared with MFS (273/112 = 2.4), LDS (35/16 = 2.2), or nsHTAD (82/76 = 1.1). With MFS as the reference population, risk of any dissection surgery or cardiovascular mortality was lowest in BAV patients (HR: 0.13; 95% CI: 0.08-0.18; HR: 0.13; 95%: CI: 0.06-0.27, respectively). The greatest risk of mortality was seen in patients with vEDS.
conclusionsMarfan and LDS cohorts demonstrate age and event profiles congruent with the current understanding of syndromic aortopathies. BAV events weigh toward elective replacement with relatively few dissection surgeries. Nonsyndromic HTAD patients experience near equal probability of dissection vs prophylactic surgery, possibly because of failure of early diagnosis.
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