Author + information
- aDepartment of Cardiovascular Medicine, Mayo Clinic, Rochester, Minnesota
- bDepartment of Radiology, Mayo Clinic, Rochester, Minnesota
- ↵∗Address for correspondence:
Dr. Li-Tan Yang, Department of Cardiovascular Diseases, Mayo Clinic, 200 First Street SW, Rochester Minnesota 55905.
Unicuspid aortic valve (UAV) presented as predominant severe aortic regurgitation (AR) is rare (<1%) in pure AR cohort. We present a case with UAV that took more than 1 decade to achieve accurate diagnosis. Multimodality imaging showcased precise anatomy of UAV and mechanisms of AR, resembling exactly the surgical findings. (Level of Difficulty: Intermediate.)
A 54-year-old man was referred for evaluation of severe aortic regurgitation (AR) and aortic aneurysm. Previous transthoracic echocardiogram (TTE) reported trileaflet, bicuspid aortic valve (BAV), possible unicuspid aortic valve (UAV), or indeterminate cusp number since 2007. The current TTE showed possible UAV with severe left ventricular enlargement and severe AR with mild stenosis. Electrocardiography-gated computed tomography (CT) angiography showed a 54-mm root, a mid-ascending aorta of 56 mm (Figure 1A), and a severely thickened UAV with fusion of right-to-left and right noncoronary cusps, and mild calcification (Figure 1B, Video 1). CT volume-rendered short-axis shows the systolic “tear drop”-like eccentric orifice (Figure 1C, Video 2). Pre-bypass intraoperative 3-dimensional (3D) transesophageal echocardiogram (TEE) confirmed a UAV (Figure 1D, Video 3) with systolic visualization of 2 raphes (Figure 1D, asterisks). Torrential AR in 3D TEE diastolic short-axis (Figure 1E) and 2D TEE long-axis (Figure 1F, Video 4) views were also noted. 3D TEE enabled visualization of the large/elongated diastolic regurgitant orifice (Figure 1G, Video 3) and raphes (Figure 1G, asterisks), which resembled the exact anatomy of the UAV as visualized by the surgeon with stay sutures at the commissures providing tension to mimic a pressurized aortic root in diastole (Figure 1H). The excised UAV demonstrates the severe thickening of the “tear drop”-shaped orifice that had become “uncoaptable” (Figure 1I). The UAV was replaced with a mechanical valved conduit and the ascending aorta with a graft. The usual presentation age of UAV is 30 to early 40 years. UAV mistaken for BAV by 2D TTE is not uncommon, particularly in the setting of stenosis, because both could present with aortopathy, and 67% UAV diagnosed by 2D TTE were found to be BAV pathologically. However, even for a UAV with predominant AR (although rare; represents <1% of cases in pure AR cohorts), accurate diagnosis took more than 1 decade in the present case, highlighting the importance of advanced computed tomography imaging and 3D TEE in defining aortic valve anatomy, critical aortic measurements, and mechanisms of dysfunction.
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
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- Abbreviations and Acronyms
- aortic regurgitation
- bicuspid aortic valve
- transesophageal echocardiogram
- transthoracic echocardiogram
- unicuspid aortic valve
- Received April 16, 2020.
- Accepted April 24, 2020.
- 2020 The Authors