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.