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 received an oral anticoagulant. For the last 5 years, 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, he had a pale conjunctiva and icteric sclera. 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/mm³, hemoglobin 7.7 g/dL, hematocrit 27.4%, and reticulocyte 7%; serum iron 47 µg/dL, total iron-binding capacity 352 µg/dL, ferritin 14.21 ng/mL; LDH 4214 IU/L, total bilirubin 3.59 mg/dL, direct bilirubin 0.95 mg/dL. A peripheral blood smear revealed helmet cells and fragmented RBCs (schistocytes). 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, significant paravalvular leakage was detected. 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. 1B).

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, she was acutely ill-looking with a pale conjunctiva. There was a grade IV-V/IV systolic murmur at cardiac apex. Laboratory findings revealed WBC 5270/mm³, hemoglobin 6.8 g/dL, hematocrit 20.9%, and platelet 226000/mm³, 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 chest X-ray showed marked cardiomegaly with pulmonary edema. 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. 2). The mitral regurgitation was well demonstrated on live 3D zoom and 3D color Doppler (Fig. 3). The para-ring defect area was measured as 0.61 cm² 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).