Abstract
Transcatheter aortic valve replacement is a validated therapeutic option for severe symptomatic aortic stenosis. Dextrocardia with total situs inversus is a rare heart condition (1 in 12,000). We present an interesting case of transcatheter aortic valve replacement with a CoreValve Evolut R (Medtronic, Minneapolis, Minnesota) through a transfemoral approach in a patient with dextrocardia and situs inversus. (Level of Difficulty: Intermediate.)
Key Words: aortic stenosis, dextrocardia with situs inversus, transcatheter aortic valve replacement (TAVR)
Abbreviations and Acronyms: CTA, computed tomography angiography; TAVR, transcatheter aortic valve replacement; TEE, transesophageal echocardiography
Graphical abstract
Transcatheter aortic valve replacement is a validated therapeutic option for severe symptomatic aortic stenosis. Dextrocardia with total situs…
Case Description
An 81-year-old woman presented with severe aortic valve stenosis. On arrival, her blood pressure was 117/40 mm Hg and heart rate 64 beats/min. She also had coronary artery disease, chronic systolic heart failure, bilateral carotid artery stenosis (right internal carotid artery occlusion and 60% to 70% left internal carotid artery stenosis), oxygen-dependent chronic obstructive pulmonary disease, diabetes mellitus, hyperlipidemia, and atrial fibrillation. Echocardiography showed an ejection fraction of 40% to 45%, severe aortic valve stenosis with mean gradient of 46 mm Hg, an aortic valve area 0.76 cm2, and mild aortic insufficiency. She was receiving appropriate medical therapy.
Her Society of Thoracic Surgeons score was 10.6; she was a high risk for surgery and a good candidate for transcatheter aortic valve replacement (TAVR). Computed tomography angiography (CTA) of chest, abdomen, and pelvis confirmed dextrocardia and total situs inversus (Figure 1A), which is a rare heart condition (1 in 12,000) (1). Her annulus perimeter measured 69.1 mm, the minimum annulus diameter was 19.2 mm, and the average diameter was 21.7 mm. Coronary heights were adequate. The aortic root angle was 49°. The coplanar view by CTA was right anterior oblique 25 and caudal 1. Femoral access was limited by heavy calcification and size (minimum diameter of 3.7 mm in the left external iliac artery and the right common femoral artery measuring 5.1 mm with heavy anterior calcification). Alternative access options were considered; but because of the reversed bovine arch with bilateral carotid artery disease and the aortic root angle >30° (left subclavian access not recommended), the patient’s frailty, and her poor lung function, the decision was made to proceed with the right femoral approach through a surgical cut-down.
Figure 1.
TAVR in Dextrocardia and Situs Inversus
(A) Computed tomography angiography: 3-dimensional rendering. (B) Transesophageal echocardiography pre–transcatheter aortic valve replacement (TAVR). (C) Coplanar view in “prone mode.” (D) Valve deployment. (E) Valve deployment in “prone mode.” (F) Transesophageal echocardiography post–transcatheter aortic valve replacement.
The procedure was performed with the patient under general anesthesia; blood pressure was 117/40 mm Hg, and heart rate was 64 beats/min. Transesophageal echocardiography (TEE) was used for intraoperative guidance (Figure 1B). The TAVR procedure was completed in standard fashion with an important modification. A 6-F pigtail catheter in the noncoronary cusp was used for aortography in the coplanar view on CTA (Figure 1C). The image was then reversed using a “prone position” acquisition mode that allowed the procedure to continue in standard fashion (Video 1). The valve was crossed using an Amplatz left (AL-1) catheter and a 0.038 straight wire. A 26-mm CoreValve Evolut R (Medtronic, Minneapolis, Minnesota) was advanced over the Confida guidewire (Medtronic). Parallax of the valve was adjusted by rotating the image-intensifier to a more right anterior oblique with slightly caudal angulation (contrary to the usual left anterior oblique and caudal angulation). Deployment was started with the CoreValve at 1 mm below the annulus. Using ascending aortography and TEE guidance, the 26-mm CoreValve Evolut R was then positioned at a depth of 4 mm below the annulus (Figures 1D and 1E, Video 2). Left-sided heart catheterization confirmed resolution of the gradient across the CoreValve Evolut R bioprosthesis. TEE showed a mean gradient of 5 mm Hg and a mild paravalvular leak (Figure 1F).
Online Video 1.
Coplanar “Prone” View
Online Video 2.
Post-implant Angiogram
“Prone position” imaging helps to normalize the TAVR procedure in a patient with dextrocardia. TEE images are, mirrored, however, they can be normalized by starting the initial multiplane angle at 180°.
Footnotes
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Case Reportsauthor instructions page.
Appendix
For supplemental videos, please see the online version of this paper.
Reference
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