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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.

There is a paucity of data describing the effect of time interval between diagnosis and surgery for Acute Type A Aortic Dissection. We describe our 8-year experience and investigate the impact of time interval between symptom onset, diagnosis and surgery on outcomes. Retrospective single-center study utilizing our Society of Thoracic Surgeons registry and patient records. Subjects were grouped by time interval between radiographic diagnosis and surgical treatment: Group A (0-4 h), Group B (4.1-8 h), Group C (8.1-12 h), and Group D (12.1 + h). Data were analyzed to identify factors associated with mortality and outcomes. 164 patients were included. Overall mortality was 21.3%. Group C had the greatest intervals between symptom onset to diagnosis to surgery, and also the highest mortality (66.7%). Preoperative tamponade, cardiac arrest, malperfusion, elevated creatinine, cardiopulmonary bypass time, and blood transfusions were associated with increased mortality, while distance of referring hospital was not. Time intervals between symptom onset, diagnosis and surgery have a significant effect on mortality. Surgery performed 8-12 h after diagnosis carries the highest mortality, which may be exacerbated by longer interval since symptom onset. Time-dependent effects should be considered when determining optimal strategy especially if inter-facility transfer is necessary.

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. [ABSTRACT FROM AUTHOR]