An Emergent Nexus between Striae and Thoracic Aortic Dissection.

Larry W Markham,Joel S Corvera,Saila T Pillai,Benjamin J Landis,John W Brown,Courtney E Vujakovich,Phillip J Hess,Jeffrey E Everett,Lawrence S Lee,Lindsey R Elmore

doi:10.3390/genes13010023

Abstract

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.

Highlights

Thoracic aortic aneurysm (TAA) is an aortopathy that predisposes one to life-threatening thoracic aortic dissection (TAD)
Prior work characterized systemic connective tissue abnormalities in a pediatric population with TAA who did not have a genetic syndrome, finding that skin striae were associated with increased rates of aortic root dilation [4]
Eighty patients had a history of TAD (Figure 1), consisting of 61 with a type A dissection and 21 with a type B dissection; two patients had a type A and type B

Summary

Introduction

Thoracic aortic aneurysm (TAA) is an aortopathy that predisposes one to life-threatening thoracic aortic dissection (TAD). Diagnosis of TAA and risk stratification provide an opportunity to intervene medically to prevent progressive dilation or surgically with prophylactic aortic repair before the onset of a TAD. Establishing a genetic diagnosis or familial predisposition may alter clinical management, including modified thresholds for earlier prophylactic aortic repair [3]. Prior work characterized systemic connective tissue abnormalities in a pediatric population with TAA who did not have a genetic syndrome, finding that skin striae were associated with increased rates of aortic root dilation [4]. Neither the prevalence nor potential prognostic utility of abnormal connective tissue findings is well understood in the general adult TAA population

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