hemolytic anemia is one of the complications which may occur after mitral valve replacement or repair , mostly due to regurgitation around the prosthesis . we report two cases which qualitatively and quantitatively evaluated lesions of regurgitation around the mitral prosthesis using real - time 3-dimensional ( 3d ) transesophageal echocardiography ( tee ) . a 44-year - old male was referred to our cardiovascular center for anemia of unknown origin . about 23 years ago , he had had mitral valve replacement ( hall - kaster prostheses , 27 mm ) elsewhere , due to severe rheumatic mitral valve stenosis . since then , he has suffered with exertional dyspnea and dark - red - colored urine ; for financial reasons , he refused further evaluation , and got only red blood cell ( rbc ) transfusions every 3 - 4 month and several medications from the cardiovascular out - patient clinic , including carvedilol , candesartan , and digoxin . on physical examination there was a systolic murmur of grade vi / vi at cardiac apex ; as well as a systolic ejection murmur at the right upper sternal border . laboratory data showed white blood cell ( wbc ) 5020/mm , hemoglobin 7.7 g / dl , hematocrit 27.4% , and reticulocyte 7% ; serum iron 47 g / dl , total iron - binding capacity 352 g / dl , ferritin 14.21 ng / ml ; ldh 4214 iu / l , total bilirubin 3.59 mg / dl , direct bilirubin 0.95 mg / dl . his chest x - ray showed a markedly increased computed tomography ratio compared with the previous study three years ago . echocardiography was performed with a high suspicion of mechanical hemolytic anemia related with mitral mechanical prosthesis . in the routine 2-dimensional ( 2d ) transthoracic echocardiogram there were significant rheumatic valvular pathologies of aortic valve steno - regurgitation and tricuspid valve regurgitation , with moderate resting pulmonary hypertension . subsequent tee revealed a well - preserved opening of the uni - leaflet tilting disc ; however , there was significant paravalvular leakage along the outer - edge of the mechanical mitral prosthesis . a real - time 3d tee showed an en - face view paravalvular defect ( fig . 1a ) , and 3d color doppler demonstrated the dehiscence lesion combined with paravalvular leak during systole ( fig . the surgical findings confirmed the paravalvular leak from the mid - posterior area of the mitral prosthesis , which then was covered with pericardium and 4 ethibond sutures . four months after a successful repair of mitral , aortic , and tricuspid valves , a postoperative 2d echocardiogram showed well functioning cardiac valves with no paravalvular leakage , normal left ventricular cavity dimensions , decreased wall thickness , and no resting pulmonary arterial hypertension . even now , 14 months postoperatively , he is in good clinical condition , with a hemoglobin level of 14.9 g / dl , and does not require any transfusion . a 63-year - old woman was referred to our cardiovascular division for further work - up of aggravated exertional dyspnea for one year . four years ago , she had had mitral posterior annuloplasty ( tailor ring 27 mm ) , tricuspid annuloplasty ( an - core ring ) , and a maze operation , due to severe mitral regurgitation , moderate tricuspid regurgitation , and moderate resting pulmonary hypertension , combined with atrial fibrillation . on physical examination , laboratory findings revealed wbc 5270/mm , hemoglobin 6.8 g / dl , hematocrit 20.9% , and platelet 226000/mm , ldh 3487 iu / l , total bilirubin 1.76 mg / dl , direct bilirubin 0.56 mg / dl , bun / creatinine 26.3/1.66 mg / dl . a conventional multiplane 2d tee showed a para - ring tissue defect of posterior mitral annular ring & strangely located tricuspid annular ring which was widely apart from the native annulus . in real - time 3d tee , both a para - ring tissue defect ( 5 o'clock ) and tricuspid ring dehiscence were appreciated in the surgeon 's view at one shot ( fig . the mitral regurgitation was well demonstrated on live 3d zoom and 3d color doppler ( fig . the para - ring defect area was measured as 0.61 cm in the multi - planar rendering view ( fig . 5 ) . redo - mitral valve replacement ( sj mechanical 29 mm ) and tricuspid annuloplasty ( mc3 28 mm ) were performed . surgical findings fit the pre - operative 3d volumetric images ( fig . 4 and 5 ) . a 44-year - old male was referred to our cardiovascular center for anemia of unknown origin . about 23 years ago , he had had mitral valve replacement ( hall - kaster prostheses , 27 mm ) elsewhere , due to severe rheumatic mitral valve stenosis . since then , he has suffered with exertional dyspnea and dark - red - colored urine ; for financial reasons , he refused further evaluation , and got only red blood cell ( rbc ) transfusions every 3 - 4 month and several medications from the cardiovascular out - patient clinic , including carvedilol , candesartan , and digoxin . on physical examination there was a systolic murmur of grade vi / vi at cardiac apex ; as well as a systolic ejection murmur at the right upper sternal border . laboratory data showed white blood cell ( wbc ) 5020/mm , hemoglobin 7.7 g / dl , hematocrit 27.4% , and reticulocyte 7% ; serum iron 47 g / dl , total iron - binding capacity 352 g / dl , ferritin 14.21 ng / ml ; ldh 4214 iu / l , total bilirubin 3.59 mg / dl , direct bilirubin 0.95 mg / dl . his chest x - ray showed a markedly increased computed tomography ratio compared with the previous study three years ago . echocardiography was performed with a high suspicion of mechanical hemolytic anemia related with mitral mechanical prosthesis . in the routine 2-dimensional ( 2d ) transthoracic echocardiogram there were significant rheumatic valvular pathologies of aortic valve steno - regurgitation and tricuspid valve regurgitation , with moderate resting pulmonary hypertension . subsequent tee revealed a well - preserved opening of the uni - leaflet tilting disc ; however , there was significant paravalvular leakage along the outer - edge of the mechanical mitral prosthesis . a real - time 3d tee showed an en - face view paravalvular defect ( fig . 1a ) , and 3d color doppler demonstrated the dehiscence lesion combined with paravalvular leak during systole ( fig . the surgical findings confirmed the paravalvular leak from the mid - posterior area of the mitral prosthesis , which then was covered with pericardium and 4 ethibond sutures . four months after a successful repair of mitral , aortic , and tricuspid valves , a postoperative 2d echocardiogram showed well functioning cardiac valves with no paravalvular leakage , normal left ventricular cavity dimensions , decreased wall thickness , and no resting pulmonary arterial hypertension . even now , 14 months postoperatively , he is in good clinical condition , with a hemoglobin level of 14.9 g / dl , and does not require any transfusion . a 63-year - old woman was referred to our cardiovascular division for further work - up of aggravated exertional dyspnea for one year . four years ago , she had had mitral posterior annuloplasty ( tailor ring 27 mm ) , tricuspid annuloplasty ( an - core ring ) , and a maze operation , due to severe mitral regurgitation , moderate tricuspid regurgitation , and moderate resting pulmonary hypertension , combined with atrial fibrillation . on physical examination , laboratory findings revealed wbc 5270/mm , hemoglobin 6.8 g / dl , hematocrit 20.9% , and platelet 226000/mm , ldh 3487 iu / l , total bilirubin 1.76 mg / dl , direct bilirubin 0.56 mg / dl , bun / creatinine 26.3/1.66 mg / dl . a conventional multiplane 2d tee showed a para - ring tissue defect of posterior mitral annular ring & strangely located tricuspid annular ring which was widely apart from the native annulus . in real - time 3d tee , both a para - ring tissue defect ( 5 o'clock ) and tricuspid ring dehiscence were appreciated in the surgeon 's view at one shot ( fig . the mitral regurgitation was well demonstrated on live 3d zoom and 3d color doppler ( fig . the para - ring defect area was measured as 0.61 cm in the multi - planar rendering view ( fig . redo - mitral valve replacement ( sj mechanical 29 mm ) and tricuspid annuloplasty ( mc3 28 mm ) were performed . surgical findings fit the pre - operative 3d volumetric images ( fig . 4 and 5 ) . hemolytic anemia is one of the potentially serious complications of prosthetic heart valves.1 ) prosthesis - associated hemolysis can present clinically as anemia , heart failure , jaundice , dark urine , increasing serum lactate dehydrogenase , and a newly developed cardiac murmur.2 ) peripheral blood smear shows variable numbers of schistocytes and small red cell fragments . mechanical hemolysis is associated with rapid acceleration and deceleration of the regurgitant jet with or without high peak shear rates.3 ) the incidence of significant hemolysis has decreased from 5 - 15% in the 1960 - 1970s to less than 1% in the 1990s , with the introduction of new - generation mechanical valves , which is resistant to structural deterioration.4 - 6 ) by nature , bioprosthetic valves and posterior rings are rarely associated with mechanical hemolysis , except in the presence of paravalvular leakage . as in the two cases presented here , the main mechanism currently for prosthetic heart valve paravalvular leakage occurs mostly in the early postoperative period ; intraoperative post - bypass tee may encourage the surgeon to intervene immediately , before the patient leaves the operating room . contrarily , the patients in these case reports presented and late paravalvular leaks diagnosed 23 and 4 years , respectively , after replacement or repair of the mitral valve , dramatically later than usual reports.7 ) late - onset paravalvular leaks can be caused by suture dehiscence , localized infection , or focal distribution of mechanical shear forces . clinical suspicion and exact diagnosis are important in these cases so as not to miss the chance of surgical correction . to evaluate the prosthetic valves , tee imaging has become the modality of choice , providing better image quality and resolution without the lung and bone tissue interference in conventional transthoracic echocardiography ( tte ) . by 2007 , real - time 3d tee using a fully - sampled matrix array transducer ( x7 - 2 t , 2.0 - 7.0 mhz , 2500 elements ) offered 2d , doppler , and 3d imaging in a single probe with excellent spatial resolution , ease of use , and time efficiency.8 ) 3d tee provides excellent visualization of prosthetic mitral valves , permitting superior diagnostic accuracy as confirmed by surgical findings.9 ) as we presented here , routine tte can diagnose regurgitation around valves but was insufficient to assess the degree or the anatomic shape . using real - time 3d tee , we observed real - time changes of the dehiscence around the prosthesis throughout the cardiac cycle , and at the same time could quantify the regurgitation area ( fig . 3d tee has become the modality of choice for evaluation of mitral prostheses , with fast acquisition and immediate online display during the peri - procedural10 ) or intra - operative periods . if the gain is too low , false - positive tissue defects around the mechanical prosthesis could appear . 3d color doppler could differentiate true or false positives of dehiscence lesions as well as the nature of regurgitated jets of either paravalvular or valvular origin using various perspectives . for the diagnosis of late paravalvular leakage , clinical suspicion and imaging modalities we reported two cases of hemolytic anemia with paravalvular leakage evaluated by real - time 3d tee , which might be useful in the perceptional appreciation and precise localization of the defect .
hemolytic anemia is recognized as a rare complication of mitral valve replacement or repair . 
 we report on a 44-year - old man with shortness of breath and hemolytic anemia , 23 years after mitral valve replacement ( hall - kaster ) , and a 63-year - old woman diagnosed of hemolytic anemia , 4 years after mitral and tricuspid annuloplasty ( tailor ring , an - core ring ) . 
 routine 2-dimensional transthoracic echocardiography revealed paravalvular leakage around the prosthesis . 
 subsequent real - time 3-dimensional ( 3d)transesophageal echocardiography helped the perceptional appreciation of the leakage and the measuring of the regurgitant orifice area using the anatomically correct plane . 
 surgical findings of each case fit those of 3d volumetric images .