Presented During:
Thursday, April 25, 2024: 5:38PM - 7:00PM
Sheraton Times Square
Posted Room Name:
Central Park
Abstract No:
P0302
Submission Type:
Abstract Submission
Authors:
Christina Waldron (1), Afsheen Nasir (2), Alan Chou (3), Roland Assi (3)
Institutions:
(1) Yale School of Medicine, New Haven, CT, (2) Yale New Haven Hospital, New Haven, CT, (3) Yale University School of Medicine, New Haven, CT
Submitting Author:
Christina Waldron
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Yale School of Medicine
Co-Author(s):
Afsheen Nasir
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Yale New Haven Hospital
Alan Chou
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Yale University School of Medicine
Roland Assi
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Yale University School of Medicine
Presenting Author:
Christina Waldron
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Yale School of Medicine
Abstract:
Objective: Diagnosis of Marfan (MFS), Ehlers-Danlos (EDS), and Loeys-Dietz (LDS) syndromes often warrants specialized evaluation for screening and surveillance of aortic disease. This study aims to characterize the incidence of referral to cardiovascular medicine and cardiothoracic surgery between male and female patients within a large healthcare network for patients diagnosed with MFS, EDS, or LDS.
Methods: We conducted a retrospective review of patients with genetic or clinical diagnoses of MFS, EDS, or LDS from the electronic medical record database in a large healthcare delivery network between 2013-2022. We explored the referral pattern to cardiovascular medicine and cardiothoracic surgery based on connective tissue disease and sex.
Results: 995 patients were identified (74% (n=741) female). There were 242 (24%) patients with MFS (41% (n=99) female), 772 (73%) with EDS (87% (n=627) female), and 31 (3%) with LDS (48% (n=15) female). Overall, the referral rates to cardiovascular medicine and cardiothoracic surgery were 69% (n=687) and 14% (n=137), respectively, both with a significantly higher referral for men compared to women (77% (n=196/254) vs 66% (n=491/741), p=0.001 and 33% (n=83/254) vs 7.3% (n=54/741), p<0.001). Among referred patients, 74% (n=507) saw cardiovascular medicine at our institution, with the remaining 26% (n=180) going to outside institutions. Referral rates to cardiovascular medicine for MFS, EDS, and LDS were 90% (n=217), 61% (n=441), and 94% (n=29), respectively. There was no significant sex difference. Referral rates to cardiothoracic surgery for MFS, EDS, and LDS were 38% (n=92), 2.9% (n=21), and 48% (n=15), respectively. Men with EDS were more likely to be referred to cardiothoracic surgery than women (11% (n=10/95) vs 1.8% (n=11/627), p<0.001). Among patients with vascular EDS, 71% (n=25/35) and 11% (n=4) were referred to cardiovascular medicine and cardiothoracic surgery, respectively, with a higher rate for men compared to women (100% (n=10/10) vs 60% (n=15/25), p=0.03; 30% (n=3) vs. 4% (n=1), p=0.061, respectively). Among patients with EDS and aortic pathology (4.4%, n=32), men were more likely to be referred to cardiothoracic surgery than women (62% (n=8) vs. 32% (n=6), p=0.093).
Conclusions: Patients with MFS and LDS had appropriately high referral rates to cardiovascular medicine. Women with connective tissue diseases are less likely to be referred to cardiovascular medicine, particular women with vascular EDS.
Aortic Symposium:
Natural History/Follow-Up
Keywords - Adult
Aorta - Aorta