Recurrent Aortic Dissection: Observations From the International Registry of Aortic Dissection.
Eric M. Isselbacher,Marc P. Bonaca,Marco Di Eusanio,James B. Froehlich,Eduardo Bassone,Udo Sechtem,Reed E. Pyeritz,Himanshu J. Patel,Ali Khoynezhad,Hans-Henning Eckstein,Guillaume Jondeau,Fabio Ramponi,Mohammad Abbasi,Daniel G. Montgomery,Christoph A. Nienaber,Kim A. Eagle,Mark E. Lindsay +16 more
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TLDR
In this article, the authors used the International Registry of Acute Aortic Dissection (IRAD) database to examine the clinical profiles and outcomes of patients with recurrent AD.Abstract:
Background: Improved medical care after initial aortic dissection (AD) has led to increased survivorship and a population of individuals at risk for further cardiovascular events, including recurrent AD. Reports describing recurrent ADs have been restricted to small numbers of patients from single institutions. We used the IRAD (International Registry of Acute Aortic Dissection) database to examine the clinical profiles and outcomes of patients with recurrent AD.
Methods: We identified 204 patients enrolled in IRAD with recurrent AD. For the primary analysis, patient characteristics, interventions, and outcomes were analyzed and compared with 3624 patients with initial AD. Iterative logistic modeling was performed to investigate variables associated with recurrent AD. Cox regression analyses were used to determine variables associated with 5-year survival. A subset of recurrent AD patients was analyzed for anatomic and demographic details of initial and recurrent ADs.
Results: Patients with recurrent AD were more likely to have Marfan syndrome (21.5% versus 3.1%; P <0.001) but not bicuspid aortic valve (3.6% versus 3.2%; P =0.77). Descending aortic dimensions were greater in patients with recurrent AD than in patients with initial AD independently of sentinel dissection type (type A: 4.3 cm [3.5–5.6 cm] versus 3.3 cm [2.9–3.7 cm], P <0.001; type B: 5.0 cm [3.9–6.0 cm] versus 4.0 cm [3.5–4.8 cm], P <0.001), and this observation was accentuated among patients with Marfan syndrome. In multivariate analysis, the diagnosis of Marfan syndrome independently predicted recurrent AD (hazard ratio, 8.6; 95% confidence interval, 5.8–12.8; P <0.001). Patients with recurrent AD who presented with proximal followed by distal AD were younger than patients who experienced distal followed by proximal dissection AD (42.1±16.1 versus 54.3±14.8 years; P =0.004).
Conclusions: Among those suffering acute aortic dissection, 5% have a history of a prior aortic dissection. Recurrent AD is strongly associated with Marfan syndrome.
# Clinical Perspective {#article-title-29}read more
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The International Registry of Acute Aortic Dissection (IRAD): new insights into an old disease.
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The revised Ghent nosology for the Marfan syndrome
Bart Loeys,Harry C. Dietz,Alan C. Braverman,Bert Callewaert,Julie De Backer,Richard B. Devereux,Yvonne Hilhorst-Hofstee,Guillaume Jondeau,Laurence Faivre,Dianna M. Milewicz,Reed E. Pyeritz,Paul D. Sponseller,Paul Wordsworth,Anne De Paepe +13 more
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A syndrome of altered cardiovascular, craniofacial, neurocognitive and skeletal development caused by mutations in TGFBR1 or TGFBR2
Bart Loeys,Junji Chen,Enid Neptune,Daniel P. Judge,Megan Podowski,Tammy M. Holm,Jennifer Meyers,Carmen C. Leitch,Nicholas Katsanis,Neda A. Sharifi,F Lauren Xu,Loretha Myers,Philip J. Spevak,Duke E. Cameron,Julie De Backer,Jan Hellemans,Yan Chen,Elaine C. Davis,Catherine L. Webb,Wolfram Kress,Paul Coucke,Daniel B. Rifkin,Anne De Paepe,Harry C. Dietz +23 more
TL;DR: These data definitively implicate perturbation of TGFβ signaling in many common human phenotypes, including craniosynostosis, cleft palate, arterial aneurysms, congenital heart disease and mental retardation, and suggest that comprehensive mechanistic insight will require consideration of both primary and compensatory events.
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