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Ann Thorac Surg 2003;75:579-581
© 2003 The Society of Thoracic Surgeons
a Division of Cardiothoracic Surgery, Duke University Medical Center, Durham, North Carolina, USA
b Division of Cardiology, Duke University Medical Center, Durham, North Carolina, USA
c Division of Hematology, Duke University Medical Center, Durham, North Carolina, USA
Accepted for publication August 22, 2002.
* Address reprint requests to Dr Milano, Department of Surgery, Duke University Medical Center, Box 3043, Durham, NC 27710, USA.
e-mail: milan002{at}mc.duke.edu
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Abstract |
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Introduction |
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Although reports have been published of patients with cold hemagglutination disease undergoing cardiac surgery [2–5], there have been none published about patients with the D-L antibody [4]. Performing conventional cardiac surgery in patients with cold-reactive proteins poses special management problems. Modern techniques of cardiac surgery include systemic hypothermia, cold cardioplegia, and topical cardiac cooling. Unfortunately, these techniques may lead to activation of cold-dependent proteins; therefore, alteration of standard operative strategy is required.
We present the case of a patient with mitral stenosis and documented long-standing PCH, who underwent mitral valve replacement. The operative techniques, which enabled successful mitral valve replacement without significant hemolysis, are described.
A 61-year-old woman was admitted to the general medicine service with congestive heart failure presumed secondary to rapid atrial fibrillation. The patient had been followed since 1999 for chronic hemolytic anemia with several acute exacerbations, which occurred in response to cold exposures and required blood transfusions. At the time of admission, she was being maintained on prednisone therapy (10 mg/d) and erythropoietin (10,000 U/wk) and had been advised to avoid the cold. Physical examination revealed a grade 2/6 diastolic murmur heard best at the left sternal border. Laboratory examination revealed compensated hemolysis with an elevated reticulocyte count (289,000) and an elevated lactic dehydrogenase concentration (1179 U/L), and persistently undetectable haptoglobin, all consistent with continuing hemolysis. Previous tests for the D-L antibody had been positive. A cold agglutinin titer, which detects only IgM antibodies, was positive at 1:10. The patient also had insulin-dependent diabetes and chronic obstructive pulmonary disease and thus she had previously not been considered a candidate for high-dose chemotherapy with autologous stem cell support.
Unfortunately, she experienced a worsening of her congestive heart failure and atrial fibrillation, which had been previously treated with sotalol. The patient underwent cardioversion and amiodarone was initiated. An echocardiogram and cardiac catheterization was performed. These studies revealed severe mitral stenosis with calcification of both leaflets, and a mean pressure gradient of 28 mm Hg. No significant coronary artery disease was detected. Pulmonary artery pressures were 65/24 mm Hg with a cardiac index of 2.7 and an ejection fraction of 0.66.
Given her symptomatic mitral stenosis, mitral valve replacement was indicated. The decision was made to operate with a strategy to minimize cold exposure. After a standard median sternotomy incision and systemic heparinization, cardiopulmonary bypass was initiated with cannulation of the ascending aorta and bicaval venous cannulation. Snares were placed around the cavae to isolate the heart completely from the systemic circulation. Systemic cooling was not used. Inflow cardiopulmonary bypass temperature was maintained at 37°C. The aorta was cross-clamped and the heart arrested with cold crystalloid antegrade and retrograde cardioplegia. Topical cooling was not used. The mitral valve was exposed with a transseptal incision. The severely calcified rheumatic valve was excised and replaced with a #25 Medtronic-Hancock pericardial valve. Total volume of cardioplegia was 3800 mL. Duration of cross-clamp was 139 minutes. Duration of cardiopulmonary bypass was 194 minutes. The patient was easily weaned from cardiopulmonary bypass. The minimal nasopharyngeal and bladder temperatures during the procedure were 36.5°C.
Postoperatively, the patient again required cardioversion for rapid atrial fibrillation but after initiation of sotalol, remained in sinus rhythm. We found no evidence of significant postoperative hemolysis. She required only two units of packed red cells on the day of surgery and no subsequent transfusions. At 1-year follow-up, the patient had clinically improved from New York Heart Association class 4 to class 2 congestive heart failure.
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In 1904, Donath and Landsteiner first described the cold-reactive autoantibody that is responsible for the complement-mediated hemolysis. They demonstrated that hemolysis was due to an "autolysin" that bound to autologous erythrocytes at low temperatures and that complement caused lysis of the sensitized cells when the temperature was raised [1]. Patients with PCH lack the high titers of cold-reacting agglutinins present in cold agglutinin disease. The D-L antibody is a nonagglutinating IgG that binds erythrocytes only in the cold. Upon rewarming, the antibody avidly binds complement and causes severe red cell lysis. Paroxysmal cold hemoglobinuria is usually diagnosed by characterizing the causative antibody through the Donath-Landsteiner test [6].
Patients with D-L antibodies can be divided into having either acute or chronic hemolytic anemia on the basis of the clinical course of the disease. In children, D-L autoimmune hemolytic anemia occurs as a single postviral episode. After the acute illness resolves, however, the patient is likely to suffer no recurrent hemolytic anemia. Recurrent PCH occurs in a chronic idiopathic form and patients have characteristic episodes of hemoglobinuria and systemic symptoms on exposure to cold. Most patients with chronic PCH, such as the woman in this report, survive for many years despite continued hemolysis [7].
Cardiopulmonary bypass with systemic hypothermia, cold crystalloid, or blood cardioplegia, and topical myocardial cooling are common techniques during modern cardiac surgery. Cardiopulmonary bypass with systemic temperatures at 30° to 34°C, topical 4°C slush, and cardioplegic solution at 4° to 10°C are conventionally used. Cold agglutinins and D-L antibodies have thermally regulated immunologic activity. This range of immune activity is referred to as thermal amplitude. Thermal amplitude may be narrow and extend only a few degrees above freezing or be broad and approach normal body temperature. Cold-reactive antibodies also typically demonstrate a maximum temperature, always less than normal body temperature, above which their activity as agglutinins or hemolysins ceases to be detectable in vitro. This maximum temperature is known as the critical temperature of the antibody [8]. IgM cold agglutinins may be primarily agglutinins that promote hemagglutination at low temperatures, microvascular thrombosis, and immediate or delayed intravascular or extravascular hemolysis leading to renal failure or perioperative myocardial infarction. The D-L antibody, because it is a complement-fixing IgG antibody, causes little hemagglutination but substantial hemolysis. Nevertheless, perioperative management of these patients should be the same as those with the IgM autoantibody.
Numeous reports describe various strategies for maintaining systemic temperature above the critical temperature of cold-reactive proteins [3, 4]. Specifically, these include crystalloid cardioplegia to flush blood out of the coronary circulation to prevent agglutination. The use of cold blood cardioplegia can cause agglutination, hemolysis, inadequate myocardial protection, myocardial infarction, or persistent postoperative hemolysis with hemoglobinuria. In our case, we used cold crystalloid cardioplegia rather than a blood-based cardioplegia, to minimize the potential for hemolysis. Furthermore, we carried out cardiopulmonary bypass using normothermic conditions and we initially administered normothermic antegrade cardioplegia to flush out the coronary circulation before administering cold retrograde cardioplegia. In addition, aortic cross-clamping and caval tapes completely isolated the cold heart from the rest of the systemic circulation and topical cooling was not used. Another option is fibrillatory arrest without cross-clamping or cardioplegia. We did not use this approach because we thought it would make the procedure technically more difficult in this already high-risk patient. Other steps taken to avoid problems attributable to cold-reacting antibody included keeping the operating room warm and warming all intravenous solutions before administration. Preoperative plasma exchange has also been described to lower the titer of the cold-reactive proteins before undergoing surgery [5]. However, such procedures are less effective when IgG antibodies are involved, because IgG has a considerable extravascular volume of distribution. Therefore, plasmapheresis is not indicated for PCH patients before heart surgery.
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This article has been cited by other articles:
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  Y. Naito, M. Nakajima, H. Inoue, and K. Tsuchiya Successful CABG in a patient with paroxysmal nocturnal hemoglobinuria Eur. J. Cardiothorac. Surg., March 1, 2004; 25(3): 468 - 470. [Abstract] [Full Text] [PDF]
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