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Ann Thorac Surg 2008;86:645-646. doi:10.1016/j.athoracsur.2008.01.101
© 2008 The Society of Thoracic Surgeons
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Case Reports

Hemolytic Anemia With Aortic Stenosis Resolved by Urgent Aortic Valve Replacement

Isamu Kawase, MDa,*, Tatsuro Matsuo, MDa, Koji Sasayama, CCPb, Hiroyuki Suzuki, MDc, Hideo Nishikawa, MD, PhDc

a Department of Cardiovascular Surgery, Mie Heart Center, Ohyodo, Meiwa-cho, Taki-gun, Mie, Japan
b Department of Medical Engineering, Mie Heart Center, Ohyodo, Meiwa-cho, Taki-gun, Mie, Japan
c Department of Cardiology, Mie Heart Center, Ohyodo, Meiwa-cho, Taki-gun, Mie, Japan

Accepted for publication January 31, 2008.

* Address correspondence to Dr Kawase, Department of Cardiovascular Surgery, Mie Heart Center, 2227-1 Aza-Komaitaru, Ohyodo, Meiwa-cho, Taki-gun, Mie, 515-0302, Japan (Email: ikaw63{at}hotmail.com).


   Abstract
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 Abstract
Introduction
 Comment
 References
 
A 78-year-old man with aortic stenosis complained of dark colored urine followed by recurrent chest pain and syncopal episodes. Echocardiography showed severely calcified aortic stenosis with the maximal pressure gradient of 125 mm Hg. Hemoglobin was 7.9 g/dL, lactate dehydrogenase was 2,295 IU/L, haptoglobin was less than 10 mg/dL, reticulocyte count was elevated, and Coombs' test was negative. We performed an urgent aortic valve replacement. After the surgery, the patient's urine became clear and his chest pain and syncope abated. All laboratory data returned to normal physiological values. In conclusion, the observed hemolysis was related to the aortic shear stress of a calcified aortic valve.


   Introduction
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 Abstract
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Intravascular hemolysis is occasionally encountered as a complication after prosthetic valve replacement or valve repair. However, we seldom experience hemolytic anemia problems with native valves. There are a few reports of intravascular hemolysis with aortic stenosis (AS) [1, 2]. We report a case of hemolytic anemia due to aortic stenosis, followed by syncopal episodes with concomitant chest pain, resulting in an urgent aortic valve replacement.

A 78-year-old man consulted a urologist at his local hospital, because he was concerned about his dark colored urine. During a 2-week admission to the urology ward, he experienced three episodes of severe chest pain followed by syncope. He was emergently transferred to our hospital because of his syncopal episodes. On admission, he was alert and well-oriented with mild chest discomfort. His blood pressure was 90/40 mm Hg and his pulse rate was 70 beats per minute. Oxygen administration with face mask relieved his chest pain. There was a 4/6 ejection systolic murmur at the right sternal border of the second intercostal space. A chest roentgenogram revealed cardiomegaly. An electrocardiogram showed regular sinus rhythm with left ventricular hypertrophy. Transthoracic echocardiography showed a severely calcified aortic valve with maximal pressure gradient of 125 mm Hg and aortic valve orifice area of 0.46 cm2, with no aortic regurgitation. There was left ventricular hypertrophy and the wall motion was normal with an ejection fraction of 79%. Coronary angiography showed no significant coronary artery disease. The peak-to-peak gradient of the aortic valve was 118 mm Hg with direct measurement. The patient had a hemoglobin of 7.9 g/dL, mean corpuscular volume of 94 femtoliter, mean corpuscular hemoglobin of 32 picogram, mean corpuscular hemoglobin concentration of 34%, and reticulocyte count of 1.6%. The patient's lactate dehydrogenase was 2,295 IU/L, his haptoglobin was less than 10 mg/dL, his Coombs test was negative, and his renal function was normal. With these laboratory findings, combined with the hematuria, we concluded that his anemia was due to the intravascular hemolysis.

After admission to our hospital by ambulance, his activity was restricted because of his unstable condition. In spite of restricted activity, he complained of severe chest pain on voluntary urination in a standing position at the bedside. Immediate electrocardiogram revealed deep ST-segmental depression in all precordial leads. After transfusion of 6 units of blood, his chest pain disappeared during periods of limited restricted activity. His clinical symptoms were obviously due to AS, as was his anemia, which was demonstrated by the laboratory findings. Therefore, we decided that an urgent aortic valve replacement was the optimal clinical solution.

At surgery, the aortic valve was bicuspid and severely calcified. The calcification extended to the anterior mitral leaflet and ventricular septal wall. The aortic valve was replaced with a 19-mm bioprosthetic valve (Mosaic Ultra; Medtronic, Inc, Minneapolis, MN). The patient had an uneventful postoperative course, and his urine color returned to normal after surgery. Postoperatively, his hemoglobin returned to normal (Fig 1A), his lactate dehydrogenase gradually approached the normal range (Fig 1B), the reticulocyte count was normalized, and haptoglobin was increased. Postoperative echocardiography revealed that the maximal gradient through the bioprosthetic valve was 18 mm Hg without any insufficiency. The patient was discharged in good condition on postoperative day 12. Follow-up shows he lives without chest pain, syncope, and with no restriction of activity.


Figure 1
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  		Fig 1. The change of laboratory data in the clinical course. (A) Hemoglobin level and (B) lactate dehydrogenase level. Preoperative blood transfusion was given on October 24 (left end of the graph) and on October 25. The values on October 24 were before transfusion, and the values on October 25 that were after transfusion are shown. The operation was done on November 1 (arrows on the graph). Additional blood transfusion was given during the operation. The values on November 1 were checked after the operation. (AVR = aortic valve replacement.)

 

   Comment
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Intravascular hemolysis and hemolytic anemia can be encountered sometimes as complications, such as perivalvular leakage after prosthetic valve replacement or residual regurgitation after valve repair. However, intravascular hemolysis with a native valvular disease is rarely experienced.

In our case, severe aortic stenosis was easily diagnosed. The patient's symptoms obviously worsened after the emergence of dark-colored urine, and his symptoms were ameliorated by blood transfusion. This fact strongly supports the relationship between symptoms of aortic stenosis and anemia. Hemolytic anemia was suggested by a normocytic and normochromic anemia, reticulocytosis, marked elevation of lactate dehydrogenase, decreased haptoglobin, and massive hematuria. There was no evidence for noncardiac cause of hemolytic anemia.

Intravascular hemolysis has been reported in patients with aortic stenosis and aortic and mitral regurgitation, as well as those with hypertrophic subaortic stenosis [3–5]. In patients with valvular disease, the mechanism for intravascular hemolysis is considered to be related to turbulence and shear stress produced by flow through stenosed or incompetent orifices. Red cell shearing stress above 3,000 dynes/cm2 can increase hemolysis [6, 7]. There is a report of a 50 mm Hg peak gradient through left ventricular outflow tract or aortic valve calculated to have a shear stress of 4,000 dynes/cm2 [8]. This patient had a maximal peak gradient of 125 mm Hg with echocardiography. We determined that aortic stenosis might cause hemolytic anemia in this case, as was evidenced by the laboratory findings and clinical presentation.

In this case, aortic stenosis caused hemolysis and angina, both of which were eliminated with aortic valve replacement.


   References
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 Abstract
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 References
 

  1. Tsuji A, Tanabe M, Onishi K, et al. Intravascular hemolysis in aortic stenosis Intern Med 2004;43:935-938.[Medline]
  2. Tamura S, Kitaoka H, Yamasaki N, et al. Aortic stenosis and mitral regurgitation complicated by hemolytic anemia and positive direct coombs test: a case report J Cardiol 2005;46:119-124.[Medline]
  3. Solanki DL, Sheikh MU. Fragmentation hemolysis in idiopathic hypertrophic subaortic stenosis South Med J 1978;71:599-601.[Medline]
  4. Eyster E, Rothchild J, Mychajliw O. Chronic intravascular hemolysis after aortic valve replacement Circulation 1971;44:657-665.[Abstract/Free Full Text]
  5. Myhre E, Dale J. Hemolysis in mitral valvular disease and mitral ball valve prosthesis Acta Med Scand 1971;189:547-550.[Medline]
  6. Nevaril CG, Lynch EC, Alfrey Jr CP, et al. Erythrocyte damage and destruction induced by shearing stress J Lab Clin Med 1968;71:784-790.[Medline]
  7. Maccallum RN, Lynch CE, Hellums JD, et al. Fragility of abnormal erythrocytes evaluated by response to shear stress J Lab Clin Med 1975;85:67-74.[Medline]
  8. Jacobson RJ, Rath CE, Perloff JK. Intravascular haemolysis and thrombocytopenia in left ventricular outflow obstruction Br Heart J 1973;35:849-854.[Free Full Text]



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This Article
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