Abstract
Introduction: Connective tissue disease patients are at risk for thoracic aortic aneurysm (TAA) and its complications. We sought to clarify risk factors for TAA specific to women. Methods: Using Yale Aortic Institute database, we identified 266 female patients with diagnosis of Marfan’s, Ehlers-Danlos, or Loeys-Dietz Syndromes. We included imaging, clinical and surgical information collected using retrospective chart review. Results: Amongst women with CTD, the prevalence of TAA in our population was 25%. MFS patients constituted 23% of the study population but accounted for 70% of aneurysm patients. EDS patients comprised 74% of the study population and 24% aneurysm patients. LDS comprised a small percentage of both populations. Non-Caucasian ethnicities seemed to have much higher likelihood of developing an aneurysm. CTD patients with aortic aneurysms were more likely to have valvular heart disease (VHD: MVP, AI), HTN, DLP, and DM. Aneurysm rates were slightly higher in ever-pregnant patients, with highest rates in MFS. TAA patients had higher rates of all-cause mortality (15% vs 2%). CV/aortic-related complications and death occurred only in TAA patients. Aortic dissection occurred in 24%; 30/67 required aortic surgery, with 13/67 requiring multiple surgeries. MFS manifested higher rates of aortic dissection (40%), surgery (40%), and mortality (both all cause and CV/Ao). Conclusions: TAA is common amongst women with CTD, with Marfan’s patients being at highest risk for TAA and its complications (aortic dissection, need for surgery, and death). Risk factors for TAA were VHD, HTN, DLP, and DM. Pregnancy appears to be a risk factor for TAA in select CTD subgroups.
Published Version
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