JACC: CASE REPORTS VOL. 4, NO. 12, 2022 ª 2022 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: PROCEDURAL COMPLICATIONS CASE REPORT: CLINICAL CASE Hemolytic Anemia After Surgical Mitral Valve Repair Treated With Transcatheter Edge-to-Edge Device Adrià Ruiz-López, MD,a Chi-Hion Pedro Li, MD,a Pilar Valdovinos, MD,b Álvaro Rodríguez, MD,a Ana Bonet, MD,a Manel Tauron, MD,c Carmen Ligero, MD,b Xavier Millan, MD, PHD,a Josep M. Alegret, MD, PHD,b David Viladés, MD,b Dabit Arzamendi, MD, PHDa ABSTRACT An 80-year-old woman with mitral valve repair failure was admitted with hemolytic anemia secondary to the impact of a regurgitant jet on the annuloplasty ring. Transcatheter repair to treat both mitral regurgitation and hemolysis was favored because of surgical risk. Transcatheter edge-to-edge repair represents an alternative for treating hemolysis associated with mitral regurgitation. (Level of Difficulty: Advanced.) (J Am Coll Cardiol Case Rep 2022;4:727–731) © 2022 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 (grade 4 intensity) and bibasal crackles, arterial blood pressure of 112/57 mm Hg, heart rate of 95 beats/min, An 80-year-old woman was admitted to the emer- and arterial oxygen saturation of 94%. Blood analysis gency department with intense asthenia and choluria showed new onset hemolytic anemia (hemoglobin, 2 months after surgical mitral valve repair (MVRe). 75 g/L; mean cell volume, 100 fL; lactate dehydroge- Physical examination revealed jaundice, a new sys- nase, 3,470 U/L; haptoglobin, <0.06 g/L; bilirubin, tolic heart murmur at the lower left sternal border 3.14 mg/dL; and 3% of schistocytes in the peripheral blood smear), with a negative Coombs test result LEARNING OBJECTIVES (Figure 1). Transthoracic echocardiography revealed prolapse of the posterior mitral leaflet that led to se-  To recognize hemolytic anemia as a rare but vere mitral regurgitation (MR) secondary to an relevant complication after surgical MVRe. eccentric jet. Under the diagnosis of nonimmune he-  To demonstrate that hemolysis secondary to surgical MVRe can be corrected with TEER. molytic anemia, she was admitted to the cardiology  To highlight the importance of understand- department for further studies. ing the physiologic and echocardiographic PAST MEDICAL HISTORY mechanisms that lead to hemolysis after MVRe to ensure the best correction of this The patient had severe kyphoscoliosis and hyper- complication. tension. She had a first admission for iron deficiency From the aDepartment of Cardiology, Hospital de la Santa Creu i Sant Pau, Autonomous University of Barcelona, Barcelona, Spain; b Department of Cardiology, Sant Joan de Reus University Hospital, University of Rovira i Virgili, Reus, Spain; and the cDepartment of Cardiac Surgery, Hospital de la Santa Creu i Sant Pau, Autonomous University of Barcelona, Barcelona, Spain. Marco Barbanti, MD, served as the Guest Associate Editor for this paper. 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 December 13, 2021; revised manuscript received March 23, 2022, accepted March 23, 2022. ISSN 2666-0849 https://doi.org/10.1016/j.jaccas.2022.03.021 728 Ruiz-López et al JACC: CASE REPORTS, VOL. 4, NO. 12, 2022 Hemolytic Anemia Treated With TEER Device JUNE 15, 2022:727–731 ABBREVIATIONS anemia requiring red blood cell transfusion INVESTIGATIONS AND ACRONYMS and iron therapy. Endoscopic examinations showed a hiatal hernia and diverticulosis Different causes of hemolytic anemia were ruled out: MR = mitral regurgitation with no signs of active bleeding. Congestive pharmacologic treatment was revised, and no asso- MVRe = [surgical] mitral valve heart failure developed and was diagnosed ciation with hemolysis was found; the result of a repair by echocardiography showing severe MR direct Coombs test was negative; and physical ex- TEER = transcatheter-edge-to- edge repair resulting from prolapse of the posterior amination and abdominal ultrasound examination mitral leaflet, moderate tricuspid regurgita- excluded splenomegaly. Transesophageal echocardi- tion, and a preserved left ventricular ejection frac- ography revealed residual mitral P1-P2 prolapse by tion. Preoperative coronary angiography showed no neochord rupture and a severe eccentric MR jet to- coronary artery disease. She underwent MVRe with ward the annuloplasty ring (Figures 3A to 3D, Videos 1 triangular P2 resection, mitral annuloplasty with a and 2), without significant stenosis (mean gradient of Memo 3D ReChord 34-mm ring (LivaNova Group), 3.9 mm Hg and Doppler pressure half-time mitral implantation of 2 neochords, and tricuspid valve valve area of 3.5 cm2) (Figure 4), with mild to mod- repair with a Contour 3D 34-mm ring (Medtronic). erate residual tricuspid regurgitation and an esti- Intraoperative transesophageal echocardiography mated pulmonary artery systolic pressure of showed a good surgical result with mild residual 60 mm Hg. MR (Figures 2A to 2E). The patient had an episode of paroxysmal atrial fibrillation, but her postoperative MANAGEMENT course was otherwise uneventful, and she was able to be discharged on day 8 after surgery. Trans- The patient required several blood transfusions and thoracic echocardiography performed before hospital diuretic therapy to relieve congestive symptoms. The discharge showed moderate MR with an eccentric jet patient had a high surgical risk (Society of Thoracic directed toward the anterior and septal wall of the Surgeons [STS] score, 2.75% risk of mortality; Euro- left atrium and no mitral stenosis (mean gradient, pean System for Cardiac Operative Risk Evaluation 2.3 mm Hg). [EuroSCORE] II, 8.74% risk of in-hospital mortality). The case was discussed by the heart team, and DIFFERENTIAL DIAGNOSIS transcatheter repair was favored to avoid surgical reintervention. Within the percutaneous options, we The differential diagnosis included autoimmune he- prioritized repair over valve replacement (although molytic anemia and hemolytic anemia related to the mitral ring would have been favorable for a dysfunction of MVRe. percutaneous valve-in-ring procedure), and a trans- catheter edge-to-edge repair (TEER) therapy was chosen on the basis of our experience. We hypothesized that hemolysis originated from F I G U R E 1 Hemolysis Parameter Evolution the mechanical impact of blood moving toward the surgical ring. For TEER intervention, the smallest MitraClip device (MitraClip NTR, Abbott Vascular) was chosen to avoid mitral stenosis. The risk of ste- nosis that we suspected was confirmed. When the clip was placed in a central A2-P2 position with “aggres- sive” leaflet capture, the transmitral mean gradient increased up to 12 mm Hg. We had to move the device more in a more ventricular direction to reduce tissue capture, and we were able to achieve a transvalvular mean gradient of 6 mm Hg (with a mitral valve area of 2.6 cm 2) and resolve the eccentric jet (Figures 5A to 5E, Videos 3 and 4). Immediately after the procedure, hemoglobinuria disappeared and hemolysis parameters improved (hemoglobin, 92 g/L; lactate dehydrogenase, 990 U/L; Temporal evolution of hemolysis parameters related to surgical and percutaneous mitral haptoglobin, 0.72 g/L; bilirubin, 0.97 mg/dL) valve repair. Hb ¼ hemoglobin; TEER ¼ transcatheter edge-to-edge repair. (Figure 1). No additional blood transfusions were needed, and she was discharged 4 days later. JACC: CASE REPORTS, VOL. 4, NO. 12, 2022 Ruiz-López et al 729 JUNE 15, 2022:727–731 Hemolytic Anemia Treated With TEER Device F I G U R E 2 Mitral Valve Before and After Surgical Repair Transesophageal echocardiographic view of posterior leaflet flail with (A) 2-dimensional and (B) 3imensional images before surgery. (C to E) The same perspectives after surgery repair achieving mild mitral regurgitation (E) at the end of the intervention. Transthoracic echocardiography showed mild MR and collision jets is associated with improvement of he- mild mitral stenosis (mean gradient, 5 mm Hg; mitral molysis parameters. valve area, 2.38 cm2 ). Therapies for symptoms associated with hemolysis include blood transfusions, urine alkalization, he- DISCUSSION: ASSOCIATION WITH CURRENT modialysis, or b-blockers. However, up to 90% of GUIDELINES, POSITION PAPERS, AND patients require a new surgical correction. Trans- CURRENT PRACTICE catheter techniques using different occluders and plugs have been used in selected cases to MVRe is recommended over mitral valve replacement repair paravalvular leaks. TEER is an accepted strat- for patients with degenerative MR. Hemolysis is a egy for patients with severe degenerative MR who are well-recognized complication after prosthetic valve at high surgical risk.2 However, the use of mitral replacement, especially associated with paravalvular valve TEER for treating hemolysis after MVRe leaks, but it is a rare complication after MVRe (<1%), has been reported only once.3 TEER of the mitral and it generally occurs early, within the first 3 months valve constitutes an appealing and effective of MVRe.1 alternative therapy to avoid redo surgery in Rapid acceleration, fragmentation, and collision patients with early failure of MVRe and hemolytic jets against different materials, as well as disrupted anemia. sutures and dehisced annuloplasty rings, are associ- ated with erythrocyte high shear stress and hemoly- FOLLOW-UP sis. Echocardiographic features of residual MR may help predict the origin of hemolysis. However, the At 4-month follow-up, the patient remains free of definitive diagnosis is established when correction of readmission and has no hemolysis or heart failure 730 Ruiz-López et al JACC: CASE REPORTS, VOL. 4, NO. 12, 2022 Hemolytic Anemia Treated With TEER Device JUNE 15, 2022:727–731 F I G U R E 3 Mitral Regurgitation Causing Hemolysis Mitral regurgitation on admission for hemolysis. Note the broken neochords (arrows) prolapsing into the left atrium in the 3-dimensional mitral valve view with and without color (A, B), as well as in the 2-dimensional image (C) and the eccentric jet directed toward the surgical mitral ring. F I G U R E 4 Mitral Valve Gradients Before and During TEER Intervention bpm ¼ beats per minute; grad ¼ gradient; max V ¼ maximum velocity; MV ¼ mitral valve; PHT ¼ pressure half-time; TEER ¼ transcatheter edge-to-edge repair. JACC: CASE REPORTS, VOL. 4, NO. 12, 2022 Ruiz-López et al 731 JUNE 15, 2022:727–731 Hemolytic Anemia Treated With TEER Device F I G U R E 5 Transcatheter Mitral Valve Repair The transcatheter edge-to-edge repair procedure, (A) starting with the transcatheter edge-to-edge repair “grasping view” and the capture or residual mitral prolapse, (B) with final mild regurgitation and (C) significant improvement of pulmonary vein hemodynamics (top, an inverted S-wave; bottom, a recovered profile). (D and E) 3-dimensional imaging showing the final result. signs. Control transthoracic echocardiography was FUNDING SUPPORT AND AUTHOR DISCLOSURES performed and showed mild MR and mild mitral ste- nosis (mean gradient, 4 mm Hg; mitral valve area, Drs Li, Millan, and Arzamendi have reported received proctoring and speaker fees from Abbott. All other authors have reported that they 2.34 cm 2). have no relationships relevant to the contents of this paper to CONCLUSIONS disclose. Hemolytic anemia secondary to MVRe failure is a rare complication. Echocardiography plays an important ADDRESS FOR CORRESPONDENCE: Dr Chi-Hion role in the suspicion of pathophysiologic cause, and Pedro Li, Department of Cardiology, Hospital de la TEER of the mitral valve can be successfully used for Santa Creu i Sant Pau, C/ Mas Casanova 90, 08041 treating hemolytic anemia caused by residual eccen- Barcelona, Spain. E-mail: ch.pedroli@gmail.com. tric MR. Twitter: @CHPedroLi. REFERENCES 1. Abourjaili G, Torbey E, Alsaghir T, Olkovski Y, 3. Sanchis L, Jorda P, Pereda D, et al. MitraClip repair, transcatheter-edge-to-edge Costantino T. Hemolytic anemia following mitral implantation for hemolytic anemia treatment after repair valve repair: a case presentation and literature surgical mitral valve repair. J Am Coll Cardiol Intv. review. Exp Clin Cardiol. 2012;17(4):248–250. 2020;13(9):e85–e86. 2. Davidson LJ, Davidson CJ. Transcatheter treat- A PPE NDI X For supplemental ment of valvular heart disease: a review. JAMA. KEY WORDS hemolysis, videos, please see the online version of this 2021;325(24):2480–2494. mitral regurgitation, mitral valve article.