JACC: CASE REPORTS VOL. 3, NO. 6, 2021 ª 2021 THE AUTHORS. PUBLISHED BY ELSEVIER ON BEHALF OF THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION. THIS IS AN OPEN ACCESS ARTICLE UNDER THE CC BY-NC-ND LICENSE (http://creativecommons.org/licenses/by-nc-nd/4.0/). MINI-FOCUS ISSUE: TRANSCATHETER INTERVENTIONS CASE REPORT: CLINICAL CASE Acute Mitral Regurgitation Secondary to Spontaneous Left Atrial Appendage Occluder Migration Mario Salido, MD,a Marc Soriano, MD,a Estefanía Fernández-Peregrina, MD,b Dabit Arzamendi, PHD,b Alba Maestro, MD,c Jordi Sans-Roselló, MD,d Maria Vidal, MD,d Lidia Bos, MD,e David Viladés, MD,e Alessandro Sionis, MDd ABSTRACT A patient underwent left atrial appendage occlusion due to recurrent stroke despite new oral anticoagulant therapy. The patient later presented with severe acute mitral regurgitation secondary to occluder device migration, which was retrieved percutaneously from the descending aorta via the femoral artery. Mitral surgical repair was required and suc- cessfully performed. (Level of Difficulty: Intermediate.) (J Am Coll Cardiol Case Rep 2021;3:888–92) © 2021 The Au- thors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). HISTORY OF PRESENTATION examination revealed new systolic heart murmur, grade 5 intensity, at the lower left sternal border and Two weeks after atrial fibrillation (AF) ablation and signs of acute heart and respiratory failure: arterial left atrial appendage occlusion (LAAO), a 50-year-old blood pressure 110/50 mm Hg, heart rate 150 beats/ woman presented to the emergency department with min, and arterial oxygen saturation 85%. An electro- sudden dyspnea and palpitations. Physical cardiogram showed left bundle branch block and atrial flutter with rapid ventricular response (150 LEARNING OBJECTIVES beats/min), and chest radiography revealed bilateral perihilar haziness. A poor acoustic window did not  To review a rare complication: clinical pre- sentation of acute mitral regurgitation with allow transthoracic echocardiography evaluation. mitral chordae rupture secondary to LAAO Pharmacological cardioversion was achieved under device migration. amiodarone and digoxin treatment, and heart failure  To discuss the importance of focused and stabilization under initial diuretic and antihyperten- timely diagnostic work-up for this rare sive treatment. complication.  To summarize current evidence, effective- PAST MEDICAL HISTORY ness, and safety of the LAAO procedure.  Percutaneous LAAO could be useful in cases The patient had breast neoplasm in 1995, treated with of resistant stroke despite OAC therapy. radical mastectomy, adjuvant radiotherapy, and later breast reconstruction. Paroxysmal AF was diagnosed From the aCardiology Residency, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; bInterventional Cardiology Unit, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; cHeart Failure Unit, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; dCoronary Care Unit, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; and the eCardiac imaging department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain. 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 Author Center. Manuscript received September 24, 2020; revised manuscript received December 11, 2020, accepted January 21, 2021. ISSN 2666-0849 https://doi.org/10.1016/j.jaccas.2021.01.016 JACC: CASE REPORTS, VOL. 3, NO. 6, 2021 Salido et al. 889 JUNE 2021:888–92 Unexpected Spontaneous Left Atrial Appendage Occluder Migration in 2017 after a transient ischemic attack, and a new INVESTIGATIONS ABBREVIATIONS oral anticoagulant (OAC) therapy was started. AND ACRONYMS In 2018, the patient developed left middle cerebral Nonsignificant coronary stenosis, negative AF = atrial fibrillation artery stroke despite proper compliance with treat- troponin, and the absence of ventricle wall LAAO = left atrial appendage ment and negative coagulopathy studies. In 2020, left motion abnormalities rule out acute ischemic occlusion atrial spontaneous echocardiographic contrast was mitral regurgitation (type 1 myocardial OAC = oral anticoagulant evidenced in a control transesophageal echocardiog- infarction). TOE revealed normal aortic valve TOE = transesophageal raphy (TOE), treated with low-molecular-weight function, absence of hypertrophy, and echocardiography heparin for 3 months. Later, the patient underwent normal left ventricular outflow tract pressure effective pulmonary vein cryoablation and LAAO gradient, ruling out aortic stenosis and hypertrophic (Amplatzer Amulet, St. Jude Medical, Plymouth, myocardiopathy. Negative blood culture findings and Minnesota) for secondary prevention of resistant the absence of intracardiac vegetations rule out stroke. Peri-procedural TOE showed a correct place- infective endocarditis. ment of the LAAO implant and the absence of peri- device residual flow (Figure 1). MANAGEMENT DIFFERENTIAL DIAGNOSIS The patient was admitted to the cardiology area un- der the suspicion of mechanical peri-device compli- The differential diagnosis included acute ischemic cation and presented a favorable response within the mitral regurgitation, infective endocarditis, aortic first 48 h. On day 3, the patient was transferred to the stenosis, hypertrophic myocardiopathy, and inter- coronary care unit due to acute pulmonary edema ventricular communication. despite medical treatment. Intensive continuous F I G U R E 1 Pre- and Post-Procedural Imaging (A) Amplatzer Amulet implanted in the left atrial appendage under fluoroscopic control. (B) Peri-procedural transesophageal echocardiog- raphy showing mitral valve with normal leaflet coaptation. 2-dimensional transesophageal echocardiography 2 weeks after the procedure showing occluder device absence (C) and a new-onset mitral prolapse of the posterior leaflet (yellow arrow) due to primary tendinous chordae rupture (D). 890 Salido et al. JACC: CASE REPORTS, VOL. 3, NO. 6, 2021 Unexpected Spontaneous Left Atrial Appendage Occluder Migration JUNE 2021:888–92 F I G U R E 2 Severe Acute Mitral Regurgitation (A) Pulsed wave Doppler pulmonary veins showing systolic pulmonary flow reversal. (B and D) Color Doppler transesophageal echocardi- ography revealed severe eccentric mitral regurgitation. (C) 2-dimensional transesophageal echocardiography showing posterior flail leaflet (yellow arrow) with an eccentric jet directed to the interatrial septum. furosemide infusion and sodium nitroprusside were exponentially in developed countries due to an in- administered. In the following 24 h, the patient crease in life expectancy and a higher detection of exhibited progressive clinical improvement with he- silent AF. modynamic stability and resolution of respiratory According to current guidelines, OAC therapy is the failure. TOE revealed severe eccentric mitral prolapse first-line treatment used to prevent stroke, based on with posterior flail leaflet (P2 and P3 scallops), pri- the risk factors for stroke evaluated by using the mary chordae tendineae rupture, and the absence of CHA 2DS2-VASc score (2). the LAAO device (Figures 1 and 2, Videos 1, 2, 3, 4, LAAO has emerged as an alternative treatment to and 5). A device migration to the abdominal aorta was OAC therapy in patients with nonvalvular AF, as LAA evidenced on the admission chest radiograph. Use of is the primary substrate for thrombus formation (3). myotomy forceps (15-F diameter) was required for its LAAO has been shown to be noninferior to medical removal, via right the femoral artery, after failed at- management for stroke prevention in patients with tempts with a goose-neck snare and endomyocardial contraindication or intolerance to OAC therapy (4). biopsy forceps (Figure 3, Videos 6, 7, 8, and 9). LAAO should also be considered in patients who present with stroke on OAC despite therapy (resistant stroke), as in our case. Estimated frequency of resis- DISCUSSION tant stroke is 11%, and there is insufficient evidence, to date, to decide the management of these patients. AF is the most common cause of cardioembolic Nevertheless, new emerging cohort studies have stroke, which is the subtype of ischemic stroke with shown similar effectiveness and safety outcomes for greater size of the lesion, highest in-hospital mortal- LAAO in this group of patients, suggesting that LAAO ity, and highest rate of functional limitation at could be a viable alternative to medical treatment discharge (1). Its prevalence is increasing (5,6). JACC: CASE REPORTS, VOL. 3, NO. 6, 2021 Salido et al. 891 JUNE 2021:888–92 Unexpected Spontaneous Left Atrial Appendage Occluder Migration F I G U R E 3 Device Retrieval Under Fluoroscopic Control Goose-neck snare (A), endomyocardial biopsy forceps (yellow arrows) (B and C), and myotomy forceps (red arrow) (D) used for device retrieval through right femoral artery. LAAO has been exponentially performed over the cardiac surgery is needed (9). Implant dislocation last decade due to the increasing prevalence of AF, as and migration to mitral valve apparatus is very rare, well as improvements in technique and operator and it is usually diagnosed as an incidental finding expertise. Frequency of in-hospital adverse outcomes in a control echocardiography in asymptomatic pa- associated with the procedure is higher in the real- tients (10). world population than in clinical trials (7). This is the first case report described in which The latest evidence, mainly based on multicenter Amplatzer Amulet device aortic migration caused registries, indicates a high rate of percutaneous LAAO mitral chordae rupture and secondary symptomatic success (97% to 99%) and a low rate of overall com- acute severe mitral regurgitation. Dislocation of the posite major adverse events (4% to 8%). The most implant, defined as peri-device flow with a common major adverse events are major bleeding and jet $5 mm width, may be due to incomplete LAAO, pericardial effusion or tamponade, which occur at a generally secondary to device oversizing, although rate of 1.5% for both and usually appear within the effective LAA sealing was confirmed by TOE post- first days after the procedure. Cardiac surgery is procedure in this case (2). Spontaneous atrial required in <3% of overall cases (5,6,8). contraction in paroxysmal AF could also increase Device embolization is an infrequent (<1%) but dislocation risk, although there is no current severe complication. In most cases, it occurs in the evidence. peri-procedural period, but late embolization within the first months is possible, as in the current case. FOLLOW-UP Its removal is most likely to be done percutane- ously, as the device usually migrates to the aorta; The patient underwent successful mitral valvulo- otherwise, when the device is trapped in the mitral plasty, mitral chordae replacement, and LAA surgical apparatus or the left ventricular outflow tract, closure. The patient is in New York Heart Association 892 Salido et al. JACC: CASE REPORTS, VOL. 3, NO. 6, 2021 Unexpected Spontaneous Left Atrial Appendage Occluder Migration JUNE 2021:888–92 functional class I in the first month after discharge procedure, although randomized clinical trials are from hospital. needed to provide solid evidence. CONCLUSIONS FUNDING SUPPORT AND AUTHOR DISCLOSURES Acute mitral regurgitation secondary to occluder de- The authors have reported that they have no relationships relevant to vice migration is a rare complication, and it should be the contents of this paper to disclose. considered in patients with recent percutaneous LAAO and acute heart failure. Flail mitral leaflet, chordae tendineae rupture, and LAAO device absence ADDRESS FOR CORRESPONDENCE: Dr. Mario Salido are specific echocardiographic features. Iniesta, Calle Torrente, number 19, 2o Cerdanyola del Emerging data support LAAO in the secondary Vallès, Barcelona 08290, Spain. E-mail: msalido@ prevention of resistant stroke as an effective and safe santpau.cat. Twitter: @MSalido2. REFERENCES 1. Arboix A, Alió J. Cardioembolic stroke: clinical 5. Cruz-González I, González-Ferreiro R, Freixa X, up of left atrial appendage occlusion with features, specific cardiac disorders and prognosis. et al. 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