A 29-year-old woman was referred to emergency services because of severe dyspnea developing into pulmonary edema. Medical therapy was given immediately and her clinical condition improved thereafter. She was consulted by the cardiology department and a diagnosis of severe mitral regurgitation associated with rheumatic heart disease was made. The patient was considered eligible for mitral valve repair. Mitral valve annuloplasty was performed at a private center. Intraoperative transesophageal echocardiography revealed minimal mitral regurgitation. Postoperative transthoracic echocardiography indicated + 1 mitral regurgitation. Hematological findings were normal before surgery. She had no anemia and lactate dehidrogenase (LDH) level was in normal range. Following the surgery there was progressive fatigue, palpitation, and jaundice developed over a 2 mo period. Physical examination showed 2/6 pansystolic ejection murmur, pallor, icterus, and tachycardia. Other findings were normal.
Laboratory results revealed a Coomb's-negative hemolytic anemia with hemoglobin of 5.9 gr/dl (13.6–17.2 gr/dl), aspartate aminotransferase (AST) of 75 IU/L (0–40 IU/L), total bilirubin of 7.4 mg/dl (0.1–1mg/dl), conjugated bilirubin of 2 mg/dl (0–0.3 mg/dl), LDH of 1552 IU/L (207–414 IU/L), haptoglobin of 0.2 gr/L (0.3–2gr/L), ferritin of 4.9 ng/ml (5–148 ng/ml; Table 1). Levels of vitamin B12, folate, and hemoglobin electrophoresis were normal. Urine hemosiderin was found positive. Drug-induced hemolytic anemia was not considered because there was no recovery after cessation of any drug except furocemide which was given before surgery. The hemolysis workup of the patient was performed. Signs of intravascular hemolysis were evident. A history of infection, accident, and dark urine were not present, excluding numerous causes of intravascular red cell breakdown. Schistocytes were demonstrated in the peripheral blood smear of the patient (Figure 1). Fecal occult blood test was found to be negative 3 times. An abdomen ultrasonography was normal except for hepatic venous dilatation. Splenic size was normal. Since the patient had mitral valve repair with a unique history and clinical findings, the diagnosis of valvular hemolysis was reached. In transthoracic echocardiography, the dimension of the left atrium, end-diastolic, and end-systolic diameters of the left ventricle were 8.1 cm, 6.1 cm, and 4.2 cm respectively. The ejection fraction was 63%. The degree of mitral regurgitation was + 3 (Figure 2). The patient refused transesophageal echocardiography, so that test was not performed. We suggested reoperation but our recommendation was not accepted by the patient. Iron therapy1 and 4 units of blood transfusion were administered to compensate for anemia-induced tachycardia and dyspnea. Fortunately the hemolysis was not apparent after 3 mo. During follow-up, blood tests revealed hemoglobin of 12.1 ngr/dl, LDH of 632 IU/L, and transthoracic echocardiography showed + 3 mitral regurgitation.
|Laboratory Results||Before operation||After operation|
|Hemoglobin (13.6–17.2 gr/dl)||13.8||5.9|
|White blood cell (4.4–11.3 × 103/μL)||5.1 × 103||6.3 × 103|
|Platelet (156–373 × 103/μL)||158 × 103||167 × 103|
|Erythrocyte mean corpuscular volume (80–95.5 fL)||83.3||79|
|Red cell distribution width (11.5%–14.5%)||unknown*||27.8|
|Reticulocyte % (0.5%–3.5%)||2.7||4.51|
|Haptoglobin (0.3–2 gr/L)||unknown*||0.2|
|Ferritin (5–148 ng/ml)||unknown*||4.9|
|Aspartate aminotransferase (0–40 IU/L)||36||75|
|Alanine aminotransferase (0–41 IU/L)||23||25|
|Lactate dehidrogenase (207–414 IU/L)||408||1552|
|Serum total bilirubin (0.1–1 mg/dl)||0.85||7.4|
|Conjugated bilirubin (0–0.3 mg/dl)||0.2||2|