Management Strategies in Aortic Dilation Among Pediatric Cardiology
Patients in a Tertiary Heart Center
Background: Limited information is available to guide therapy for aortic
dilation (AoD) in the absence of genetic syndromes. Our aim was to
identify aortic diameters that prompted intervention. Methods: We
performed a single center retrospective chart review of patients from
birth to 30 years between 2011 and 2017. Advanced imaging [magnetic
resonance (MR)/computed tomography (CT)] and echocardiographic
diameters at the sinuses of Valsalva (SoV) and ascending aorta (AAo)
were reviewed. We identified patients on pharmacotherapy and those who
underwent aortic surgery. Results: Data from 47 patients was analyzed
(74% male) and included bicuspid aortic valve (BAV, 40%), Marfan
syndrome (MFS, 28%), isolated aortic dilation (21%), Turner syndrome
(TS, 4%), and Loeys-Dietz syndrome (LDS, 4%). Family history of AoD,
aortic dissection, or vascular aneurysm was identified in 40%.
Medication was initiated at a median echo diameter of 34 mm at the SoV
and 32 mm at the AAo. Patients with genetic conditions (MFS, LDS, TS)
were started on medication earlier in the course of SoV dilation by
echocardiography. Patients with BAV were started on medication at a
larger AAo diameter compared to patients with genetic diagnoses and
isolated AoD. Five patients underwent aortic surgery at a median age of
20 years (two patients were < 20 years old at surgery).
Conclusion: Patients with genetic conditions were started on medications
at an earlier stage of AoD compared to BAV and those with isolated AoD.
Over a third of patients had a significant family history of AoD, aortic
dissection, or vascular aneurysm.