Transcatheter Edge-to-Edge Repair for Left Atrioventricular Valve Cleft After Previously Repaired Complete Atrioventricular Canal Defect in Down Syndrome.

Transcatheter Edge-to-Edge Repair for Left Atrioventricular Valve Cleft After Previously Repaired Complete Atrioventricular Canal Defect in Down Syndrome Rohit Mital, MD, Satvik Ramakrishna, MD, Gregory L. Judson, MD, Clifton Watt, MD, Kirsten Tolstrup, MD, and Vaikom S. Mahadevan, MD, Scottsdale, Arizona; Salt Lake City, Utah; Portland, Maine, and San Francisco, California INTRODUCTION Transcatheter mitral valve edge-to-edge repair (TEER) has an established role in the management of severe mitral regurgitation (MR), with proven benefits to patients with primary MR at high risk for surgery as well as select patients with secondary MR.1 As experience with the use of the MitraClip (Abbott, Abbott Park, IL) for TEER continues to grow, the use of these devices is increasingly expanding to cases with unconventional and challenging anatomy. Cleft mitral leaflets, a typically rare finding, represent a situation featuring complex valve anatomy where the role of TEER has not been clearly established and surgery has been the default consideration for management.2,3 Patients with Down syndrome (DS) have a relatively higher incidence of atrioventricular septal defects (AVSDs) and concomitant cleft mitral/atrioventricular valve (AVV) leaflets potentially requiring multiple surgical repairs in childhood, thus placing them at higher risk of additional complications in adulthood.4-7 Prior case reports have demonstrated the use of TEER in cases of cleft mitral leaflets, highlighting possible interventional approaches to achieve an effective repair.8-13 Here we present a case with prior surgical repair of a complete atrioventricular canal (CAVC) defect with left AVV cleft who underwent successful TEER for severe regurgitation. CASE PRESENTATION A 33-year-old man with a history of DS with surgically repaired CAVC in the first year of life and repeat surgical intervention 11 years later, bicuspid aortic valve, symptomatic sinus bradycardia with permanent pacemaker implantation, nonsustained ventricular tachycardia, supraventricular tachycardia, obstructive sleep apnea, chronic kidney disease, and seizure disorder was evaluated for progressively From the Department of Cardiovascular Diseases, Mayo Clinic Arizona (R.M.), Scottsdale, Arizona; Division of Cardiovascular Medicine, Department of Internal Medicine, University of Utah School of Medicine (S.R.), Salt Lake City, Utah; Division of Cardiology, Maine Medical Center (G.L.J.), Portland, Maine; and Division of Cardiology, University of California, San Francisco (C.W., K.T., V.S.M.), San Francisco, California. Keywords: Transcatheter edge-to-edge repair, Percutaneous mitral valve repair, Mitral regurgitation, Cleft leaflet, Down syndrome Correspondence: Dr. Rohit Mital, Mayo Clinic Arizona, 13400 East Shea Boulevard, Scottsdale, Arizona 85259. (E-mail: ). Copyright 2022 by the American Society of Echocardiography. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/). 2468-6441 https://doi.org/10.1016/j.case.2022.09.008 worsening dyspnea on exertion and fatigue. Subsequent evaluation with transesophageal echocardiogram (TEE) confirmed surgical repair with inlet ventricular septal defect (VSD) patch and a common AVV with the presence of anterior left AVV cleft and severe regurgitation. Given the progressively worsening nature of the patient’s symptoms resulting in significant functional decline, the patient was evaluated by cardiac surgery for consideration of repeat surgical intervention. The patient was thought to be prohibitively high risk for repeat surgery in the setting of multiple prior sternotomies and presence of significant medical comorbidities. He was referred to the structural heart team for exploration of percutaneous therapeutic options. The decision was made to proceed with percutaneous valve repair with TEER. Intraoperative TEE demonstrated normal biventricular size and function, with a left ventricular (LV) end-diastolic internal diameter of 4.6 cm and an estimated LV ejection fraction of 55% to 60%. There was no evidence of flow across the site of inlet VSD repair by color and spectral flow Doppler. An anterior cleft of the left AVV was located at the lateral aspect of the A2 scallop with prolapse of the A2 and A3 scallops. There was severe left AVV regurgitation originating at the site of the anterior cleft with a posteromedially directed jet. The effective regurgitant orifice area was 0.59 cm2, with estimated regurgitant volume of 99 mL and regurgitant fraction of 74% along with systolic flow reversal in the right-sided pulmonary veins consistent with severe regurgitation. The peak and mean transvalvular gradients were 15 mm Hg and 5 mm Hg, respectively (Figure 1, panel A in Videos 1-4). The estimated mitral valve area by two-dimensional (2D) planimetry in the transgastric view was approximately 3.8 cm2, suggesting that the elevation in transmitral gradients was likely due to significant regurgitant flow. Posterior leaflet length was assessed preintervention to guide device selection and measured approximately 9 mm. The anterior leaflet measured 14 mm in length. With TEE guidance, transseptal puncture was performed using a radiofrequency wire, and the delivery sheath was advanced into the left atrium. Posterior leaflet length was important in determining appropriate device selection, with 6 mm of leaflet insertion needed for NT/NTW MitraClips, according to the manufacturer’s recommendations, and 9 mm of leaflet insertion needed for XT/XTW MitraClips. An NTW MitraClip was selected due to a posterior leaflet length of only 9 mm. The device was subsequently advanced into the left ventricle, and bileaflet capture was confirmed. Initially, the device was positioned at the A2/P2 scallops with orientation of the clip arms at 12 and 6 o’clock, essentially perpendicular to the valve plane. However, significant left AVV regurgitation remained with this approach, with difficulty in capturing the anterior leaflet within the clip arms due to the presence of the anterior cleft near the 12 o’clock position (Video 5). The device was repositioned with slight angulation with clip arms adjusted to 2 and 8 o’clock so that there was capture of 35 36 Mital et al VIDEO HIGHLIGHTS Video 1: Intraprocedural TEE with 2D views pre- and postTEER without color Doppler. Midesophageal biplane imaging of a long-axis (120 -155 ) and bicommissural view (55 -65 ) without color Doppler (A) demonstrating A2/A3 prolapse (V) and the location of the anterior cleft (Y). Similar views are shown (B) that demonstrate 2D placement of the device at the location of the anterior cleft. Video 2: Intraprocedural TEE with two-dimensional views pre- and post-TEER with color Doppler. Midesophageal biplane imaging of a long-axis (120 -155 ) and bicommissural view (55 -65 ) with color Doppler (A) demonstrating severe left AVV regurgitation originating at the site of the ante (...truncated)