Imaging for Transcatheter Edge-to-Edge Repair for Tricuspid Regurgitation in Ebstein Anomaly.

Abstract

HomeCirculation: Cardiovascular ImagingVol. 14, No. 11Imaging for Transcatheter Edge-to-Edge Repair for Tricuspid Regurgitation in Ebstein Anomaly Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplementary MaterialsFree AccessCase ReportPDF/EPUBImaging for Transcatheter Edge-to-Edge Repair for Tricuspid Regurgitation in Ebstein Anomaly Yiting Fan, PhD, Kevin Kam, MBChB, Kent So, MBChB, Yat-yin Lam, MBChB and Alex Pui-Wai Lee, MBChB, MD Yiting FanYiting Fan Department of Cardiology, Shanghai Chest Hospital, Shanghai Jiao Tong University, Shanghai, China (Y.F.). Search for more papers by this author , Kevin KamKevin Kam Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong SAR, China (K.K., K.S., A.P.-W.L.). Search for more papers by this author , Kent SoKent So Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong SAR, China (K.K., K.S., A.P.-W.L.). Search for more papers by this author , Yat-yin LamYat-yin Lam Hong Kong Asia Heart Center, Hong Kong SAR, China (Y.L). Search for more papers by this author and Alex Pui-Wai LeeAlex Pui-Wai Lee Correspondence to: Alex Pui-Wai Lee, MBChB, MD, Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Rm 114037, 9/F, LCW Clinical Sciences Bldg, Prince of Wales Hospital, Hong Kong, China. Email E-mail Address: [email protected] https://orcid.org/0000-0002-4120-155X Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Hong Kong SAR, China (K.K., K.S., A.P.-W.L.). Search for more papers by this author Originally published11 Nov 2021https://doi.org/10.1161/CIRCIMAGING.121.013327Circulation: Cardiovascular Imaging. 2021;14:e013327Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: November 11, 2021: Ahead of Print An 85-year-old male retired metal worker was referred to our institution for echocardiography with a 12-month history of progressive bilateral lower limb swelling. The patient had a history of alpha thalassemia but had no prior cardiac symptoms. On examination, the patient showed no sign of cyanosis. He was slightly underweight, with a body weight of 41 kg and a body mass index of 18. There was severe bilateral lower extremity edema spreading up to his knees and moderate ascites. Echocardiography showed apical displacement (11.6 mm/m2) of the tricuspid valve (TV) septal and posterior leaflets, consistent with Ebstein anomaly (EA; Figure 1A and 1B, Movie I in the Supplemental Material). There was a wide gap between anterior and the displaced septal leaflets with torrential tricuspid regurgitation (TR; Figure 1C). The functional right ventricle was dilated (Figures 1A and 2A). Three-dimensional transesophageal echocardiography showed the TV had 4 leaflets (2 posterior), corresponding to the type IIIB morphology1 (Figure 1D). There was no atrial sepal defect. Computed tomography demonstrated anterior and superior rotation of the functional TV orifice relative to the chest (Figure 1E and 1F). Electrocardiogram indicated sinus rhythm with no preexcitation. His symptoms became refractory to diuretics at maximally tolerated dose (furosemide 40 mg daily, spironolactone 12.5 mg daily, and metolazone 5 mg weekly). Because of the advanced age and comorbidities, the patient was deemed inoperable by the heart team. Transcatheter edge-to-edge repair was offered on compassionate ground after approval by the central cardiac ethics committee of the Hospital Authority.Download figureDownload PowerPointFigure 1. Transcatheter edge-to-edge repair (TEER) for tricuspid valve regurgitation in Ebstein anomaly. Preprocedural echocardiography (A–D) and computed tomography (CT; E and F). A, Four-chamber view showing apical displacement (yellow arrow) of the septal (S) and posterior leaflet (P) with leaflet tethering (white arrows) to underlying right ventricle (RV) myocardium. B, RV inflow view showing spiral displacement (curved arrow) of the functional tricuspid valve (TV) orifice and delamination/thinning of the RV inlet myocardium (red arrow heads). C, Wide leaflet gap with torrential tricuspid regurgitation (TR). D, Three-dimensional (3D) echocardiography showing 4-leaflet morphology (type IIIB) of the TV without leaflet fenestrations. E and F, CT showing anterior and superior rotation of the functional tricuspid orifice. G through J, Intraprocedural imaging. Live 3D transesophageal echocardiography (TEE) in the low esophageal TV-focused (G) and RV inflow-outflow views (H) showing, respectively, outward rotation of the guide and superior steering of the clip delivery system (CDS; pink arrows) to align with the rotated TV plane. I, Failed initial attempts to grasp both leaflets with anterior leaflet losing capture. Sternal compression (red arrow) did not reduce TV dimension. J, Right parasternal chest compression (green arrow) facilitated leaflet grasping. K, Relations between compressive directions and TV plane orientation on CT. Oblique right parasternal compression (palm with green arrow) would markedly reduce functional tricuspid orifice dimension (dashed line). Small insert: operator’s hand position (dashed outline) on right anterior oblique fluoroscopic view during right parasternal compression. L, Postprocedural 3D echocardiography showing one clip implanted in the anteroseptal commissure with TV bicuspidization. M, TR reduction with mild-to-moderate residual regurgitation at 4 mo. A indicates anterior leaflet; aRV, atrialized RV; asterisk, leaflet coaptation gap; C, MitraClip; dashed line, functional tricuspid orifice; dotted line, anatomic atrioventricular groove; fRV, functional RV; pm, anterior papillary muscle; RA, right atrium; SP, spine; and ST, sternum.Download figureDownload PowerPointFigure 2. Right ventricular (RV) structure and function.A, Baseline. B, 4-month follow-up. The functional RV end-diastolic volume decreased post-transcatheter edge-to-edge repair (TEER), with preserved RV ejection fraction (EF) and stroke volume (SV). ED indicates end diastole; EDV, end-diastolic volume; EDVi, end-diastolic volume index; ES, end systole; ESV, end-systolic volume; and ESVi, end-systolic volume index.The procedure was performed under general anesthesia with transesophageal echocardiography and fluoroscopic guidance. We used the MitraClip G4 system (Abbott, Santa Clara, CA; TriClip is unavailable in our region) using the mis-keyed technique. Outward torque of the guide catheter and careful adjustment of the A-knob under transesophageal echocardiography guidance aligned the clip delivery system with a trajectory perpendicular to the rotated TV plane (Figure 1G and 1H, Movie II in the Supplemental Material). Implantation of a clip in an anteroseptal position was attempted but multiple attempts to grasp both leaflets failed due to the wide leaflet gap (Movies III and IV in the Supplemental Material). In an attempt to reduce TV annular dimensions and improve leaflet grasping, pressure was applied on the anterior chest at the distal sternum. However, sternal compression had little effect in reducing the TV dimension because of the misalignment of the direction of compressive force with the rotated TV plane (Figure 1I). Using a modified approach of chest compression, we applied oblique pressure over the right parasternal chest towards the left axilla, creating a coaxial force to the TV. This maneuver markedly reduced TV dimension in the septolateral direction and effectively facilitated leaflet grasping (Figure 1J and 1K, Movie V in the Supplemental Material). Implantation of one clip (XTW) in the anteroseptal commissure reduced TR from torrential to mild-to-moderate (Figure 1L, Movies VI through VIII in the Supplemental Material). At 4-month follow-up, symptomatic improvement and TR reduction were significant (Figure 1M, Movie IX in the Supplemental Material). There was mild ankle edema and no ascites. Diuretics use was reduced to spironolactone 12.5 mg daily, while regular weekly metolazone and daily furosemide were no longer required. The functional right ventricle decreased in size (Figure 2).EA is a rare congenital malformation characterized by apical displacement of hinge points of the septal and posterior TV leaflets; the functional tricuspid orifice is displaced from the anatomic atrioventricular junction and rotated anteriorly and superiorly along the inner curvature of the right ventricle.2 The clinical presentation varied with age at diagnosis. Patients without associated intracardiac shunt or other defects may present with severe TR and heart failure in the seventh/eighth decades of life, at which time the risk of surgery is high. Transcatheter edge-to-edge repair using the TriClip device has recently been reported to be safe and effective for treatment of moderate or greater TR.3 However, patients with TR due to EA were excluded. There is currently no established interventional therapy for TR associated with EA. We demonstrated for the first time that transcatheter edge-to-edge repair for EA is feasible. Careful imaging planning and modified interventional techniques are keys to overcoming the anatomic challenge and achieving procedural success.Article InformationSources of FundingThis work is supported by the Hong Kong HMRF (05160976).DisclosuresDr Lee is a speaker and consultant for Abbott Structural and Philips Healthcare. The other authors report no conflicts.Supplemental MaterialsSupplemental Videos I–IXFootnotesThe Supplemental Material is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCIMAGING.121.013327.For Sources of Funding and Disclosures, see page 1087.Correspondence to: Alex Pui-Wai Lee, MBChB, MD, Li Ka Shing Institute of Health Science, The Chinese University of Hong Kong, Rm 114037, 9/F, LCW Clinical Sciences Bldg, Prince of Wales Hospital, Hong Kong, China. Email [email protected]edu.hk