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Cardiovascular Outcomes in Aortopathy: GenTAC Registry
Quick Takes
- In a large, multicenter registry of patients with a heritable aortic aneurysm, the age associated with risk of elective proximal aortic aneurysm surgery or any dissection surgery was lowest (and therefore the relative risk was highest) for Loeys-Dietz syndrome and Marfan syndrome.
- Compared to patients with other types of heritable aortic aneurysm, patients with BAV had a greater frequency of elective aortic surgery rather than surgery in the setting of dissection, and the lowest relative risk of dissection surgery or cardiovascular mortality.
Study Questions:
What are the age distributions and the probabilities of elective surgery for proximal aortic aneurysm and for any dissection surgery, and for cardiovascular mortality, among various aortopathy etiologies?
Methods:
The GenTAC (Genetically Triggered Thoracic Aortic Aneurysm and Cardiovascular Conditions) Registry is a longitudinal observational cohort study of individuals with heritable thoracic aortic aneurysm at a total of eight centers; enrollment began in 2007, and follow-up was closed in 2016. Clinical endpoints included proximal aortic aneurysm surgeries including valve-sparing and valve replacement procedures; and categorized as elective aneurysm surgery, emergent proximal dissection surgery, or any aortic dissection surgery. Dissections managed without surgery were included in the analysis of cardiovascular mortality but not in the surgical dissection analysis. Data were analyzed to estimate the age at which there was a 25% risk of elective aortic aneurysm surgery or any aortic surgery, and a 5% risk of emergent proximal dissection surgery and cardiovascular mortality.
Results:
Analysis included 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). The age at which there was a 25% probability of elective proximal aortic aneurysm surgery was lowest for LDS at 30 years (95% confidence interval [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). The age at which there was a 25% probability for any dissection surgery was lowest 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). Participants with 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, the risk of any dissection surgery or cardiovascular mortality was lowest in BAV patients (hazard ratio [HR], 0.13; 95% CI, 0.08-0.18; and HR, 0.13; 95% CI, 0.06-0.27, respectively). The greatest risk of mortality was seen in patients with vEDS.
Conclusions:
The authors concluded that MFS 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, and nsHTAD patients experience near equal probability of dissection and prophylactic surgery, possibly because of failure of early diagnosis.
Perspective:
This large, multicenter registry-based study found that the age at which patients with a heritable aortic aneurysm are at risk of elective proximal aortic aneurysm surgery or any dissection surgery was lowest for LDS and MFS, confirming that these heritable conditions carry higher risks compared to nsHTAD or BAV. These data help support recommendations for elective intervention at lower thresholds for patients with LDS and MFS. Patients with BAV had a greater frequency of elective aortic surgery rather than surgery in the setting of dissection, possibly related to concomitant aortic surgery at the time of aortic valve replacement.
Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Invasive Cardiovascular Angiography and Intervention, Prevention, Valvular Heart Disease, Vascular Medicine, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, Interventions and Structural Heart Disease, Interventions and Vascular Medicine
Keywords: Age Distribution, Aneurysm, Dissecting, Aortic Aneurysm, Thoracic, Bicuspid Aortic Valve Disease, Cardiac Surgical Procedures, Dissection, Ehlers-Danlos Syndrome, Heart Defects, Congenital, Heart Valve Diseases, Loeys-Dietz Syndrome, Marfan Syndrome, Secondary Prevention, Thoracic Surgery, Turner Syndrome, Vascular Diseases
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