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a Section of Cardiac Surgery, Clement J. Zablocki Veterans Affairs Medical Center, Milwaukee, Wisconsin
b Division of Cardiothoracic Surgery, Clement J. Zablocki Veterans Affairs Medical Center, Milwaukee, Wisconsin
c Department of Urology, Medical College of Wisconsin, Milwaukee, Wisconsin
d Medical College of Wisconsin, Milwaukee, Wisconsin
Accepted for publication February 18, 2009.
* Address correspondence to Dr Almassi, 9200 W Wisconsin Ave, Milwaukee, WI 53226 (Email: halmassi{at}mcw.edu).
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Introduction |
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A 71-year-old woman presented with weakness, fatigue, and severe anemia. A series of scans including a bone scan, computed tomographic scan, and magnetic resonance imaging of the chest, were completed to rule out lymphoproliferative diseases. The workup led to the diagnosis of a large renal cell carcinoma extending into the inferior vena cava and the right atrium. The rest of the metabolic workup was negative, although the patient was found to have CA.
Consultations were made with the departments of urology, cardiothoracic surgery, and hematology to plan for this complicated surgery. An echocardiogram was obtained and revealed that the tumor extended into the right atrium but did not impinge on the tricuspid valve or extend into the right ventricle. A DHCA would be required to remove the tumor thrombus from the right atrium and vena cava, but this was complicated by the diagnosis of CA.
The patient was started on steroids (prednisone, 60 mg, daily for 1 week; 40 mg for 1 week; and then decreased by 5 mg every 3 days preoperatively), as well as rituximab (375 mg/m2 on an accelerated schedule for 2 weeks) to suppress her CA. Her CA titer was high at 1:512 (CA diagnosis, 1:70) with active hemolysis while on steroid tapering. The response to steroids was inadequate and the prednisone was increased to 40 mg per day until surgery. She was also admitted for plasmaphoresis the evening prior and the morning of surgery. The plasma exchange was done with fresh frozen plasma rather than albumin and crystalloids at one-and-a half plasma volume. A thermo-amplitude blood study revealed that hemolysis was occurring at 17°C. Cooling would not be less than 20° Celsius to minimize hemolysis. Blood warmers were recommended for all transfusions. After the complete workup, surgery was performed by two teams including the departments of cardiothoracic surgery and urology.
The kidney dissection was performed through a laparotomy; once that was complete, a median sternotomy was performed. Continuous transesophageal echocardiography revealed the tumor to be mobile within the inferior vena cava and the right atrium. Full heparinization occurred, the ascending aorta was cannulated distally, and a single 32-French venous cannula was placed in the right atrium at the right atrial appendage being careful to prevent breakage of the tumor mass.
The patient was placed on cardiopulmonary bypass and was cooled down to 21.5°C. With a flat line electroencephalogram, thiopental and steroids were given. Her head was packed in ice and placed in a trendelenburg position, circulation was stopped, and the venous blood was withdrawn into the pump reservoir.
Under circulatory arrest, a right artiotomy was made toward the inferior vena cava, and then at the kidney level, the cuff of the inferior vena cava at the attachment of the right renal vein was cut. The right kidney and the tumor were removed. The vena cava and the right atrium were repaired with 5-0 and 4-0 polypropylene sutures, respectively. Cardiopulmonary bypass was resumed after 19 minutes of circulatory arrest.
Warming continued to 36°C. The patient was weaned from cardiopulmonary bypass without the need for any inotropic support. During the course of the operation, all shed blood was suctioned and recirculated back into the patient. Blood products included packed red blood cells, fresh frozen plasma, and platelets for hemostasis. Recovery occurred in the intensive care unit, and the patient was discharged on postoperative day 4.
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Many surgical techniques have been described for this type of tumor, but DHCA provides a bloodless field and prevents tumor embolization [2–4]. An operative mortality of 0% to 10% is reported with DHCA [4]. When surgery is performed without the use of cardiopulmonary bypass a mortality rate of as much as 30% can be expected with bleeding being the highest complication [4]. This case was further complicated by a known diagnosis of CA.
The presence of cold agglutinins leads to hemagglutination at low temperatures followed by complement fixation and hemolysis when rewarming after cardiopulmonary bypass [6]. If the diagnosis of CA is unknown, the perfusionist may notice a separation of red blood cells and plasma, incomplete cardioplegic delivery or high pressures in the cardiopulmonary bypass circuit [6]. Further evidence of complications from CA includes hemolysis at the surgical site, microvascular occlusion, and decreased organ perfusion [1, 7]. Fortunately, this patient was diagnosed with CA preoperatively. All patients undergoing DHCA should have screening for CA to prevent life-threatening complications from CA, such as cerebral or myocardial infarction, hepatic or renal failure, and hemolysis [6, 7].
The plasma titer of CAs and the thermal amplitude at which hemagglutination occurs is critical to planning a successful surgery for a patient requiring DHCA [6]. Patients are considered positive for CA at a titer of 1:70 or higher [7]. Prescreening should include queries about acrocyanosis, Reynaud's phenomenon, hemoglobinuria, jaundice, and pallor [1]. This patient's CA titer was 1:512, and the thermal amplitude was 17°C. Preoperative treatment was aimed at lowering the titer, and intraoperative management focused on not cooling lower than 20°C.
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