MARC details
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| fixed length control field |
04042nam a22004937a 4500 |
| 008 - FIXED-LENGTH DATA ELEMENTS--GENERAL INFORMATION |
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| fixed length control field |
232233s20232023 xxu||||| |||| 00| 0 eng d |
| 022 ## - INTERNATIONAL STANDARD SERIAL NUMBER |
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| International Standard Serial Number |
2047-9980 |
| 024 ## - OTHER STANDARD IDENTIFIER |
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| Standard number or code |
10.1161/JAHA.123.029518 [doi] |
| 040 ## - CATALOGING SOURCE |
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| Original cataloging agency |
Ovid MEDLINE(R) |
| 099 ## - LOCAL FREE-TEXT CALL NUMBER (OCLC) |
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| PMID |
37776192 |
| 245 ## - TITLE STATEMENT |
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| Title |
Vertebral Tortuosity Is Associated With Increased Rate of Cardiovascular Events in Vascular Ehlers-Danlos Syndrome. |
| 251 ## - Source |
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| Source |
Journal of the American Heart Association. 12(19):e029518, 2023 10 03. |
| 252 ## - Abbreviated Source |
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| Abbreviated source |
J Am Heart Assoc. 12(19):e029518, 2023 10 03. |
| 253 ## - Journal Name |
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| Journal name |
Journal of the American Heart Association |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. |
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| Year |
2023 |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. |
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| Manufacturer |
FY2024 |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. |
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| Publication date |
2023 10 03 |
| 265 ## - SOURCE FOR ACQUISITION/SUBSCRIPTION ADDRESS [OBSOLETE] |
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| Publication status |
ppublish |
| 265 ## - SOURCE FOR ACQUISITION/SUBSCRIPTION ADDRESS [OBSOLETE] |
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| Medline status |
MEDLINE |
| 266 ## - Date added to catalog |
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| Date added to catalog |
2023-11-22 |
| 520 ## - SUMMARY, ETC. |
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| Abstract |
Background Arterial tortuosity is associated with adverse events in Marfan and Loeys-Dietz syndromes but remains understudied in Vascular Ehlers-Danlos syndrome. Methods and Results Subjects with a pathogenic COL3A1 variant diagnosed at age <50 years were included from 2 institutions and the GenTAC Registry (National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions). Height-adjusted vertebral artery tortuosity index (VTI-h) using magnetic resonance or computed tomography angiography was calculated. Associations between VTI-h and outcomes of (1) cardiovascular events (arterial dissection/rupture, aneurysm requiring intervention, stroke), or (2) hollow organ collapse/rupture at age <50 years were evaluated using receiver operator curve analysis (using outcome by age 30 years) and mixed-effects Poisson regression for incidence rate ratios. Of 65 subjects (54% male), median VTI-h was 12 (interquartile range, 8-16). Variants were missense in 46%, splice site in 31%, and null/gene deletion in 14%. Thirty-two subjects (49%) had 59 events, including 28 dissections, 5 arterial ruptures, 4 aneurysms requiring intervention, 4 strokes, 11 hollow organ ruptures, and 7 pneumothoraces. Receiver operator curve analysis suggested optimal discrimination at VTI-h >=15.5 for cardiovascular events (sensitivity 70%, specificity 76%) and no association with noncardiovascular events (area under the curve, 0.49 [95% CI, 0.22-0.78]). By multivariable analysis, older age was associated with increased cardiovascular event rate while VTI-h >=15.5 was not (incidence rate ratios, 1.79 [95% CI, 0.76-4.24], P=0.185). However, VTI-h >=15.5 was associated with events among those with high-risk variants <40 years (incidence rate ratios, 4.14 [95% CI, 1.13-15.10], P=0.032), suggesting effect modification by genotype and age. Conclusions Increased arterial tortuosity is associated with a higher incidence rate of cardiovascular events in Vascular Ehlers-Danlos syndrome. Vertebral tortuosity index may be a useful biomarker for prognosis when evaluated in conjunction with genotype and age. |
| 546 ## - LANGUAGE NOTE |
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| Language note |
English |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
*Aortic Dissection |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
*Ehlers-Danlos Syndrome, Type IV |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
*Loeys-Dietz Syndrome |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Adult |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Arteries |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Female |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Humans |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Male |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Topical term or geographic name entry element |
Middle Aged |
| 650 ## - SUBJECT ADDED ENTRY--TOPICAL TERM |
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| Indexing |
Automated |
| 651 ## - SUBJECT ADDED ENTRY--GEOGRAPHIC NAME |
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| Institution |
MedStar Health Research Institute |
| 651 ## - SUBJECT ADDED ENTRY--GEOGRAPHIC NAME |
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| Institution |
MedStar Heart & Vascular Institute |
| 657 ## - INDEX TERM--FUNCTION |
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| Medline publication type |
Journal Article |
| 657 ## - INDEX TERM--FUNCTION |
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| Medline publication type |
Research Support, N.I.H., Extramural |
| 657 ## - INDEX TERM--FUNCTION |
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| Medline publication type |
Research Support, Non-U.S. Gov't |
| 700 ## - ADDED ENTRY--PERSONAL NAME |
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| Local Authors |
Asch, Federico M |
| Institution Code |
MHRI |
| 700 ## - ADDED ENTRY--PERSONAL NAME |
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| Local Authors |
Kancherla, Kalyan |
| Institution Code |
MHVI |
| 790 ## - Authors |
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| All authors |
Stephens SB, Shalhub S, Dodd N, Li J, Huang M, Oda S, Kancherla K, Doan TT, Prakash SK, Weigand JD, Asch FM, Beecroft T, Cecchi A, Shittu T, Preiss L, LeMaire SA, Devereux RB, Pyeritz RE, Holmes KW, Roman MJ, Lacro RV, Shohet RV, Krishnamurthy R, Eagle K, Byers P, Milewicz DM, Morris SA |
| 856 ## - ELECTRONIC LOCATION AND ACCESS |
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| DOI |
<a href="https://dx.doi.org/10.1161/JAHA.123.029518">https://dx.doi.org/10.1161/JAHA.123.029518</a> |
| Public note |
https://dx.doi.org/10.1161/JAHA.123.029518 |
| 858 ## - ORCID |
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| ORCID text |
Asch, Federico M |
| Orcid |
<a href="https://orcid.org/0000-0002-1744-5271">https://orcid.org/0000-0002-1744-5271</a> |
| Name |
https://orcid.org/0000-0002-1744-5271 |
| 942 ## - ADDED ENTRY ELEMENTS (KOHA) |
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| Koha item type |
Journal Article |
| -- |
Article |
