Aortic Stenosis and Mitral Regurgitation Complicated by Hemolytic Anemia and Positive Direct Coombs Test: A Case Report

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1 J Cardiol 2005 Sep; 46 3 : Aortic Stenosis and Mitral Regurgitation Complicated by Hemolytic Anemia and Positive Direct Coombs Test: A Case Report Shinjiro Hiroaki Naohito Makoto Toru Yoshihisa Takashi Jun Masanori Shiro Yoshinori TAMURA, MD KITAOKA, MD, FJCC YAMASAKI, MD OKAWA, MD KUBO, MD MATSUMURA, MD, FJCC FURUNO, MD TAKATA, MD, FJCC NISHINAGA, MD SASAGURI, MD DOI, MD, FJCC Abstract A 83-year-old man was admitted because of heart failure due to severe aortic stenosis and mitral regurgitation secondary to chordal rupture of the anterior leaflet. Mild anemia and elevated serum lactate dehydrogenase were present with reticulocytosis and haptoglobinemia. Direct Coombs test was positive. Coexistence of autoimmune hemolytic anemia was identified, but the main cause of his hemolysis was thought to be mechanical hemolysis due to stenotic valve and/or ruptured chordae because of the presence of red cell fragmentation. The patient successfully underwent double valve replacement. Improvement of anemia was coupled with reduction of the serum lactate dehydrogenase level. Valvular shear stress on the red cells and reduction of red cell deformability secondary to autoimmune hemolytic anemia were thought to be responsible for his hemolysis. J Cardiol 2005 Sep ; 46 3 : Key Words Valvular disease Mitral regurgitation Complications hemolytic anemia autoimmune hemolytic anemia : AIHA 1 83 : : Departments of Medicine and Geriatrics, and Cardiovascular Surgery, Kochi Medical School, Kochi Address for correspondence : DOI Y, MD, FJCC, Department of Medicine and Geriatrics, Kochi Medical School, Kohasu, Oko-cho, Nankoku, Kochi ; ydoi@med.kochi-u.ac.jp Manuscript received December 20, 2004; revised February 23, 2005 ; accepted March 1,

2 120 Fig. 1 Chest radiograph on admission showing bilateral pleural effusion and pulmonary congestion Table 1 Laboratory data on admission Blood cell count RBC Hb Ht MCV MCH / l 10.3 g/dl 30.6% 96 fl 32.3 pg Blood chemistry T-BIL I BIL AST ALT LDH 2.0 mg/dl 1.5 mg/dl 59 IU/l 17 IU/l 1,173 IU/l MCHC WBC Plt 33.7% 4,900/ l / l LDH1 483 IU/l LDH2 507 IU/l LDH3 191 IU/l Reticulocyte Immunological findings 4.1% LDH4 LDH5 51 IU/l IU/l 5 34 Direct Coombs test Fe 59 g/dl Indirect Coombs test TIBC 255 g/dl Sugar water test HAM test Ferritin 257 ng/ml Haptoglobin 4 mg/dl : normal range. : : 2080 :80 1 : 156cm 58 kg 110/46mmHg 100/min36.6 C Levine / 2 Levine / X : 68% Fig. 1 : 100/min : lactate dehydrogenase: LDH LDH Table 1 : 53mm 32 mm 53mm 6.08 m/sec 148 mmhg 92mmHg Fig : 20mmHg v 28mmHg 2.19 l/min/m 2 31/17 mmhg 25 mmhg : LDH LDH J Cardiol 2005 Sep; 46 3 :

3 121 Fig. 2 Transthoracic echocardiograms a: Parasternal short-axis view showing ruptured chordae tendineae arrow. b: Parasternal short-axis view showing prolapse of the anterior mitral leaflet. c : Parasternal short-axis view with color imaging showing massive mitral regurgitation. d: Parasternal short-axis view showing severely calcified tricuspid aortic valve. e : Doppler echocardiogram showing increased peak systolic pressure gradient across the aortic valve. 1,000 1,100IU/l g/dl LDH LDH AIHA HAM sugar water AIHA 20 mg/day 2 AIHA Fig. 3 10mg/day 44 St. Jude Medical #23 Carbo Medics #25 22 U Fig. 4 LDH J Cardiol 2005 Sep; 46 3 :

4 122 Fig. 3 Peripheral blood smear Wright stain, 400 demonstrating red cell fragmentation arrows Fig. 4 Clinical course After double valve replacement, anemia improved together with reduction of serum LDH level. POD post-operative day ; Hb hemoglobin ; LDH lactate dehydrogenase , Jacobson mmhg m/sec mmhg mmhg 47 6 Garcia J Cardiol 2005 Sep; 46 3 :

5 123 AIHA AIHA 1 10 AIHA AIHA deformability 10 Huang 5 1 Hsu 7 Huang AIHA AIHA AIHA nafamostat aprotinin 14,15 1 AIHA J Cardiol 2005 Sep; 46 3 : Lam BK, Cosgrove DM, Bhudia SK, Gillinov AM : Hemolysis after mitral valve repair : Mechanisms and treatment. Ann Thorac Surg 2004 ; 77: Doi YL, Matsumura Y, Yabe T : Haemolytic anaemia after mitral valve repair. Lancet 1996; 347: Yeo TC, Freeman WK, Schaff HV, Orszulak TA : Mechanisms of hemolysis after mitral valve repair : Assessment by serial echocardiography. J Am Coll Cardiol 1998; 32 : Upalakalin JN, Uhl L, Burger AJ : Mitral valve-induced microangiographic hemolytic anemia. Transfusion 2002 ; 42 : Huang HL, Lin FC, Hung KC, Wang PN, Wu D : Hemolytic anemia in native valve infective endocarditis : A case report and literature review. Jpn Circ J 1999 ; 63: J Cardiol 2005 Sep; 46 3 :

6 124 6 Nishiura T, Miyazaki Y, Oritani K, Tominaga N, Tomiyama Y, Katagiri S, Kanayama Y, Yonezawa T, Tarui S, Yamada T, et al : Aspergillus vegetative endocarditis complicated with schizocytic hemolytic anemia in a patient with acute lymphocytic leukemia. Acta Haematol 1986 ; 76: Hsu CM, Lee PI, Chen JM, Huang LM, Wu MH, Chiu IS, Lee CY : Fatal Fusarium endocarditis complicated by hemolytic anemia and thrombocytopenia in an infant. Pediatr Infect Dis J 1994 ; 13: Solanki DL, Sheikh MU : Fragmentation hemolysis in idiopathic hypertrophic subaortic stenosis. South Med J 1978; 71: Maeda T, Ashie T, Kikuiri K, Fukuyama S, Yamaguchi Y, Yoshida, Shimamoto K, Iimura O: Fragmentation hemolysis in a patient with hypertrophic obstructive cardiomyopathy and mitral valve prolapse. Jpn Circ J 1992 ; 56 : Harrison s Principles of Internal Medicine ed by Wilson JD, Braunwald E, Isselbacher KJ, et al, 12th Ed. McGraw- Hill, New York, 1991; pp Tsuji A, Tanabe M, Onishi K, Kitamura T, Okinaka T, Ito M, Isaka N, Nakano T : Intravascular hemolysis in aortic stenosis. Intern Med 2004; 43: Jacobson RJ, Rath CE, Perloff JK : Intravascular haemolysis and thrombocytopenia in left ventricular outflow obstruction. Br Heart J 1973; 35 : Garcia MJ, Vandervoort P, Stewart WJ, Lytle BW, Cosgrove DM, Thomas JD, Griffin BP: Mechanisms of hemolysis with mitral prosthetic regurgitation : Study using transesophageal echocardiography and fluid dynamic simulation. J Am Coll Cardiol 1996; 27 : Onoe M, Magara T, Yamamoto Y: Cardiac operation for a patient with autoimmune hemolytic anemia with warmreactive antibodies. Ann Thorac Surg 2001; 71: Hayashi Y, Ohtani M, Soma T, Sasako Y : Aortic valve replacement for a patient with warm-reactive autoimmune hemolytic anemia. Eur J Cardiothorac Surg 2002 ; 22: J Cardiol 2005 Sep; 46 3 :

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