Introduction
Transcatheter aortic valve replacement (TAVR) has evolved into a well-established minimally invasive option for patients with severe symptomatic aortic stenosis. As the range of patients undergoing TAVR continues to expand, atypical anatomy such as congenital right aortic arch (RAA) may present a potential barrier to achieving consistently excellent outcomes. Here, we illustrate our strategy for TAVR in a patient with RAA.
Procedural Vignette
A 75-year-old woman with obesity (body mass index 39.4 kg/m2), hypertension, and heart failure with preserved ejection fraction (63%) presented with worsening exertional dyspnea. Transthoracic echocardiography revealed a thickened tri-leaflet aortic valve (AV) with stage D3 paradoxical severe low-flow low-gradient aortic stenosis (mean gradient 24 mmHg, maximum aortic velocity 3.2 m/s, indexed AV area 0.58 cm2/m2, stroke volume index 27.5 mL/m2), without regurgitation. Coronary catheterization confirmed no obstructive disease. Computed tomographic angiography scan showed a calcified AV (annulus 419 mm2) and suitable transfemoral access bilaterally but also RAA with a retroesophageal left subclavian artery (Figure 1a-c, Supplemental Videos 1 and 2). The Society of Thoracic Surgeons risk score was 2.3%. After heart team evaluation, the patient consented for TAVR and for the publication of this report.
Figure 1.
Computed tomographic angiography scan with (a) three-dimensional reconstruction of the aorta, illustrating the patient’s right aortic arch in an anterior-posterior view. (b) Axial cross-section further delineates the right aortic arch (left arrow), the left carotid artery as the first branch of the arch (top right arrow), and an aberrant retroesophageal left subclavian artery (bottom right arrow). Scale: 1 division represents 1 cm. (c) Axial cross-section shows the aortic valve (top arrow) and midline descending aorta (bottom arrow). Scale: 1 division represents 1 cm. A, anterior; F, foot; H, head; L, left; P, posterior; R, right. Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, illustrating (d) advancement of the valve delivery system along the descending thoracic aorta and (e) traversing the right aortic arch, followed by (f) deployment of a 23-mm Sapien 3 Ultra valve and (g) completion aortogram after valve deployment.
Abbreviations: CAU, caudal; CRA, cranial; LAO, left anterior oblique; RAO, right anterior oblique.
TAVR was performed under monitored anesthesia care. Through the right common femoral artery and vein, respectively, a pigtail catheter was advanced into the aortic root confirming the 3-cusp view (left anterior oblique 2°, cranial 7°), and a pacing catheter was positioned in the right ventricle. The left common femoral artery was accessed for the delivery system to avoid excess torsion along the RAA, which may interfere with valve positioning and deployment. A 14-Fr Edwards eSheath (Edwards Lifesciences, Irvine, CA) was inserted over a Lunderquist wire (Cook Medical, Bloomington, IN) following systemic heparinization. An AL-1 catheter and Emerald wire (Cordis, Santa Clara, CA) were used to cross the AV. The invasive AV gradient was 46 mmHg. A curled Amplatz Extra-Stiff wire (Cook Medical) was positioned in the left ventricle apex. Initial balloon valvuloplasty was performed using an 18-mm Z-MED balloon (B. Braun Medical, Bethlehem, PA) under rapid ventricular pacing. Finally, the Edwards Commander Delivery System was advanced with orientation rotated 180° to angulate with the RAA (“E” insignia facing posteriorly instead of anteriorly), using an anterior-posterior view (left anterior oblique 1°, cranial 7°) to reach the arch (Figure 1d, Supplemental Video 3), and a right anterior oblique view (right anterior oblique 32°, cranial 7°) to maneuver across the RAA to the root (Figure 1e, Supplemental Video 4). A 23-mm Sapien 3 Ultra valve (Edwards Lifesciences) was deployed under rapid ventricular pacing (Figure 1f and g, Supplemental Videos 5 and 6). The postdeployment AV gradient was 8 mmHg. Immediate transthoracic echocardiography confirmed a well-seated valve without intravalvular or paravalvular regurgitation. The patient was discharged the next day without complications.
Discussion
RAA occurs in 0.05% of the population and most commonly develops with an aberrant retroesophageal left subclavian artery. This anatomy is rarely associated with other congenital heart diseases and is typically incidentally discovered. However, RAA introduces technical challenges for TAVR given that commercial valve systems are designed for the normal left aortic arch anatomy. While advancing the valve, to angulate the device with the contour of the RAA, we rotated the Commander Delivery System 180 degrees such that the Edwards “E” insignia faced downward instead of the usual upward direction.1,2 To avoid excessive torsion, which may cause aortic injury or interfere with optimal valve positioning and deployment, we intentionally chose the left common femoral artery as our access for the delivery system. As patients with RAA often have a midline descending aorta, advancing the valve system through the left femoral artery reduces the effective tortuosity of the device’s trajectory, starting from the left iliofemoral system into the midline descending aorta and finally into the RAA and ascending aorta along a single arc. While a previous report utilized a stiff buddy wire to mitigate aortic tortuosity while approaching from the right femoral artery,1 this maneuver was not necessary in our case. Finally, using 3-dimensional geometric reconstruction of the computed tomography imaging to guide the fluoroscopic angles for the 3-cusp view, and capitalizing on the maneuverability and stability of the delivery system in the RAA via our left femoral artery approach, we encountered no difficulties in achieving optimal valve positioning and successful deployment.
The few reports of TAVR in patients with RAA have frequently described complications including heart block requiring permanent pacemaker placement,1,3 paravalvular leak,2 and cardiac tamponade,3 which may be due in part to challenges with catheter positioning and valve deployment given the atypical anatomy. Using specific strategies to optimize TAVR in the setting of a RAA, our patient had an uneventful course and was discharged the next day per our institution’s fast-track protocol. Overall, we illustrate an important example of how, with experience and specific strategies to navigate a congenital aortic arch anomaly, TAVR can be performed safely and efficaciously in patients with this challenging anatomy.
Consent Statement
Consent was obtained from the patient for publication of this report and any accompanying images.
Funding
The authors have no funding to report.
Disclosure Statement
Dr Sharma is a paid consultant and proctor for Edwards Lifesciences. The other authors had no conflicts to declare.
Footnotes
Supplemental data for this article can be accessed on the publisher’s website.
Supplementary Material
Computed tomographic angiography scan revealing a calcified aortic valve, right aortic arch, and suitable transfemoral access for transcatheter aortic valve replacement. Scale: 1 division represents 1 cm. L, left; P, posterior
Three-dimensional reconstruction of computed tomographic angiography scan, illustrating the patient’s right aortic arch. A, anterior; F, foot; H, head; L, left; P, posterior; R, right
Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, showing advancement of the Edwards Commander Delivery System up to the patient’s right aortic arch. Left anterior oblique 1°, cranial 7°
Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, showing the traversal of the valve delivery system across the patient’s right aortic arch into the aortic root. Right anterior oblique 32°, cranial 7°
Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, illustrating deployment of a 23-mm Sapien 3 Ultra valve. Left anterior oblique 2°, cranial 7°
Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, showing the completion aortogram after deployment of a 23-mm Sapien 3 Ultra valve. Left anterior oblique 2°, caudal 6°
References
- 1.Yashima F., Hayashida K., Munakata M., et al. Aortic stenosis with right-sided aortic arch treated with transfemoral aortic valve implantation. Cardiovasc Interv Ther. 2019;34(1):70–71. doi: 10.1007/s12928-018-0509-x. [DOI] [PubMed] [Google Scholar]
- 2.D'Ascenzi F., Iadanza A., Zacà V., et al. How should I treat severe symptomatic aortic stenosis with transcatheter aortic valve implantation in a patient with right aortic arch? EuroIntervention. 2014;10(1):169–172. doi: 10.4244/EIJV10I1A27. [DOI] [PubMed] [Google Scholar]
- 3.Romaguera R., Roura G., Gómez-Hospital J.A., et al. CoreValve® aortic bioprosthesis implantation in a patient with situs inversus totalis with dextrocardia. Rev Esp Cardiol (Engl Ed) 2013;66(5):409–410. doi: 10.1016/j.rec.2012.10.014. [DOI] [PubMed] [Google Scholar]
Associated Data
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Supplementary Materials
Computed tomographic angiography scan revealing a calcified aortic valve, right aortic arch, and suitable transfemoral access for transcatheter aortic valve replacement. Scale: 1 division represents 1 cm. L, left; P, posterior
Three-dimensional reconstruction of computed tomographic angiography scan, illustrating the patient’s right aortic arch. A, anterior; F, foot; H, head; L, left; P, posterior; R, right
Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, showing advancement of the Edwards Commander Delivery System up to the patient’s right aortic arch. Left anterior oblique 1°, cranial 7°
Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, showing the traversal of the valve delivery system across the patient’s right aortic arch into the aortic root. Right anterior oblique 32°, cranial 7°
Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, illustrating deployment of a 23-mm Sapien 3 Ultra valve. Left anterior oblique 2°, cranial 7°
Fluoroscopic cine images of transcatheter aortic valve replacement via a left common femoral artery approach in the context of a right aortic arch, showing the completion aortogram after deployment of a 23-mm Sapien 3 Ultra valve. Left anterior oblique 2°, caudal 6°