Journal
GENES
Volume 13, Issue 1, Pages -Publisher
MDPI
DOI: 10.3390/genes13010023
Keywords
thoracic aortic aneurysm; thoracic aortic dissection; connective tissues; striae; Marfan syndrome
Categories
Funding
- National Institutes of Health [K12 HD068371, K23 HL141667]
- Marfan Foundation Early Investigator Grant
- American Heart Association [19 CDA34660278]
- IUSM Strategic Research Initiative
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This study analyzed the association between connective tissue abnormalities and clinical endpoints in adults with aortopathy. The results showed that skin striae were significantly associated with the occurrence of aortic dissection and were more common in younger patients. Skin striae were also more frequently observed in patients with aortic dissection compared to age- and sex-matched controls.
Current approaches to stratify the risk for disease progression in thoracic aortic aneurysm (TAA) lack precision, which hinders clinical decision making. Connective tissue phenotyping of children with TAA previously identified the association between skin striae and increased rate of aortic dilation. The objective of this study was to analyze associations between connective tissue abnormalities and clinical endpoints in adults with aortopathy. Participants with TAA or aortic dissection (TAD) and trileaflet aortic valve were enrolled from 2016 to 2019 in the setting of cardiothoracic surgical care. Data were ascertained by structured interviews with participants. The mean age among 241 cases was 61 +/- 13 years. Eighty (33%) had history of TAD. While most participants lacked a formal syndromic diagnosis clinically, connective tissue abnormalities were identified in 113 (47%). This included 20% with abdominal hernia and 13% with skin striae in atypical location. In multivariate analysis, striae and hypertension were significantly associated with TAD. Striae were associated with younger age of TAD or prophylactic aortic surgery. Striae were more frequent in TAD cases than age- and sex-matched controls. Thus, systemic features of connective tissue dysfunction were prevalent in adults with aortopathy. The emerging nexus between striae and aortopathy severity creates opportunities for clinical stratification and basic research.
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