Clinical Stratification of Pediatric Patients with Idiopathic Thoracic Aortic Aneurysm. J Pediatr 2015 Jul;167(1):131-7.e1-5
Date
03/31/2015Pubmed ID
25812776DOI
10.1016/j.jpeds.2015.02.042Scopus ID
2-s2.0-84937524866 (requires institutional sign-in at Scopus site) 11 CitationsAbstract
OBJECTIVES: To describe the global phenotypes of pediatric patients with thoracic aortic aneurysm (TAA) who do not have a clinical diagnosis of Marfan syndrome (MFS) or related connective tissue disorders. We hypothesized that the presence of noncardiovascular abnormalities correlate with TAA severity and that medical therapy reduces TAA progression.
STUDY DESIGN: This is a retrospective case series of patients with TAA age ≤ 21 years evaluated in a cardiovascular genetics clinic. Patients meeting clinical criteria for MFS or related disorders were excluded. Repeated measures analyses of longitudinal echocardiographic measurements of the aorta were used to test associations between TAA severity and noncardiovascular phenotype and to assess the impact of medical therapy.
RESULTS: Sixty-nine patients with TAA at mean age 12.5 ± 5.3 years were included. Noncardiovascular abnormalities, including skeletal (65%) or craniofacial (54%) findings, were frequently observed. Increased rate of aortic root enlargement was associated with ocular (P = .002) and cutaneous (P = .003) abnormalities, and increased rate of ascending aorta enlargement was associated with craniofacial (P < .001) abnormalities. Beta blocker or angiotensin receptor blocker therapy (n = 41) was associated with reduction in the rate of aortic root growth (P = .018).
CONCLUSIONS: Children with TAA not satisfying diagnostic criteria for MFS or related disorders frequently have noncardiovascular findings, some of which are associated with TAA progression. Because therapy initiation may reduce risk of progression and long-term complications, comprehensive assessment of noncardiovascular findings may facilitate early risk stratification and improve outcomes.
Author List
Landis BJ, Ware SM, James J, Shikany AR, Martin LJ, Hinton RBMESH terms used to index this publication - Major topics in bold
Abnormalities, MultipleAdrenergic beta-Antagonists
Angiotensin Receptor Antagonists
Aorta
Aortic Aneurysm, Thoracic
Child
Cohort Studies
Disease Progression
Female
Humans
Male
Retrospective Studies
Severity of Illness Index
Ultrasonography