Introduction
Aortic dilation (AoD) at the aortic root and ascending aorta have been
identified in isolation and with genetic anomalies, particularly
connective tissue disorders (CTD) [1, 2]. Despite the advances in
imaging technology and aortic disease management, there is a paucity of
literature regarding imaging choice, medical therapy, and appropriate
timing for surgical intervention in patients with isolated AoD.
Guidelines do exist to aid clinicians in pharmacotherapy management and
surgical intervention in patients with CTD [3]. Additionally, CTD
patients are likely to have aortic root repair or replacement [4]
and early initiation of pharmacotherapy. Identifying patients early with
aortopathy and appropriately intervening with medical therapy to slow
the progression of AoD or surgery to prevent aortic dissection is the
goal.
Establishing defined ranges of aortic dimensions for which intervention
is necessary would be beneficial to clinicians for guiding imaging
choices, medication initiation, and patient counseling. A variety of
imaging modalities have been utilized for measurements of the aorta
including two-dimensional echocardiography, computed tomography (CT),
and magnetic resonance (MR) imaging [5]. Currently CT is the gold
standard for aortic visualization and ruling out significant aortic
pathology [6]. Clinicians have utilized two-dimensional
echocardiography as a screening tool for aortic pathology, such as AoD,
however the literature has shown low sensitivity when attempting to
detect AoD [7].
Information is limited regarding pharmacotherapy initiation or surgical
intervention in patients with isolated AoD, especially when there is
discrepancy between echocardiographic and advanced imaging modality
measurements. Studies have compared aortic root size by advanced imaging
and echocardiography and have demonstrated underestimation of the aortic
root size by echocardiography and a general trend for the difference to
become greater as the aortic root size increases [8]. Family history
of AoD or aortic dissection may prompt clinicians to monitor patients
more closely, obtain advanced imaging as an adjunct to echocardiography,
and when appropriate establish a follow-up program for them [9]. Set
diameter cutoffs have been established for various conditions with
respect to when to consider intervention in the setting of AoD [3].
When patients experience progressive AoD despite pharmacologic therapy
or in the presence of significant family history of aortic dissection,
surgical intervention is typically necessary. From a surgical
standpoint, aortic root replacement has been established as a method to
prevent aortic dissection and in a majority of cases be performed in a
fashion that preserves the aortic valve (David procedure) [4, 10].
In regards to patients with CTD (e.g. Ehlers-Danlos syndrome [EDS],
Loeys-Dietz syndrome [LDS], or Marfan syndrome [MFS]), they are
more likely to require aortic root replacement surgery compared to
patients with other clinical conditions associated with AoD [11].
The purpose of this study was to describe a single center experience in
identification of aortic dimensions, by multiple modalities, which
prompted medical or surgical intervention. We also describe
relationships between each modality at various points of measure of the
aortic root.