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CASE REPORT Table of Contents   
Year : 2010  |  Volume : 13  |  Issue : 2  |  Page : 145-147
Atrial septal defect closure on cardiopulmonary bypass in a sickle cell anemia: Role of hydroxyurea and partial exchange transfusion

Department of Anesthesia and Critical Care, Grant Medical College and Sir JJ Hospital, Mumbai, India

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Date of Submission03-Aug-2009
Date of Acceptance01-Sep-2009
Date of Web Publication3-May-2010


Partial exchange transfusion during cardiopulmonary bypass, while conducting cardiac surgery may be a useful technique in patients with high level of sickle hemoglobin. Along with this preoperative use of hydroxyurea and alternative analgesic modalities such as transcutaneous electrical nerve stimulation in postoperative period may be beneficial, in our opinion. A 16-year-old female of Turner's syndrome having sickle cell anemia scheduled for closure of arterial septal defect on cardiopulmonary bypass was managed with partial exchange transfusion and warm cardioplegia.

Keywords: Cardiopulmonary bypass, partial exchange transfusion and hydroxyurea, sickle cell disease

How to cite this article:
Gosavi KS, Dash SK, Shah BN, Upasani C B. Atrial septal defect closure on cardiopulmonary bypass in a sickle cell anemia: Role of hydroxyurea and partial exchange transfusion. Ann Card Anaesth 2010;13:145-7

How to cite this URL:
Gosavi KS, Dash SK, Shah BN, Upasani C B. Atrial septal defect closure on cardiopulmonary bypass in a sickle cell anemia: Role of hydroxyurea and partial exchange transfusion. Ann Card Anaesth [serial online] 2010 [cited 2022 Dec 8];13:145-7. Available from:

   Introduction Top

Sickle cell anaemia is a disorder of hemoglobin structure in which shape of Red blood cells (RBC) changes to sickle in hypoxic environment and can cause vascular occlusion and ischemia of multiple organs. Hypothermia, stagnation of blood and acidosis are other factors which can precipitate it and can cause multiple infarcts. When subjected to cardiopulmonary bypass for cardiac surgery procedures, these patients need special precautions to prevent such episodes.

   Case Report Top

A 16-year-old female was admitted to the medicine department with complains of pain in the right thigh and abdomen for six days. This young female, known to suffer from of sickle cell anemia, was apparently alright three years back when she started getting shortness of breath (grade 1) along with recurrent attacks of common colds which progressed to NYHA grade 3 over last three years. It was accompanied by generalized dull aching bone pain which was precipitated by cold. She was transfused whole blood three times in last three years. Last whole blood transfusion was given one month prior admission. Both parents had sickle cell trait, but, led normal life.

The patient was short statured (height 4 ft 7 inches), weighed 34 kgs and appeared pale. Her heart rate was 110/min, blood pressure, 110/70 mm Hg, and respiratory rate, 24/min. Auscultation of chest was consistent with the findings of atrial septal defect (ASD). Abdominal examination revealed splenomegaly. Hemogram on admission showed hemoglobin (Hb) = 7.6 gm% (PCV = 33%), leucocytes count = 7,800/mm 3 , reticulocyte count = 6% and peripheral smear showed microcytes, macrocytes, target cells, poikilocytosis, anisocytosis. Hemoglobin electrophoresis finding was suggestive of homozygous sickle cell anemia with sickle hemoglobin (HbSS) 71%, hemoglobin A2 (HbA2) 5.6%, fetal hemoglobin (HbF) 20%. Two-dimensional echocardiography findings showed: ostium secundum type ASD of 24 mm size with L;R shunt and pulmonary arterial pressure = 35 mm Hg. Ultrasonography (USG) abdomen showed rudimentary uterus with streak ovaries. Karyotype (45XO) confirmed diagnosis of Turner syndrome. As the patient presented in sickle cell crisis she was managed with intravenous (i.v.) Ringer lactate (RL) to raise the central venous pressure (CVP) to 8 - 10 cm of water and then maintained with 2 ml/ kg/hr of RL infusion. Oxygen was supplied by ventimask at 6 litres/min. She was given intravenous Tramadol 50 mg three times a day for analgesia and Inj. Amoxycillin 500 mg + clavulinate 125 mg combination intravenous twice daily. She responded well and subsequently was started with hydroxyurea (500 mg/day) orally. Her general condition improved with above medications over next three weeks. Surgery for ASD closure was planned as per hospital schedule. Hydroxyurea was continued up to the day of surgery. Patient's Hb was 11.6 gm%, PCV = 36%, on day before surgery. With two months treatment with Hydroxyurea preoperative hemoglobin electrophoresis showed HbSS = 60%. HbA2 = 8.6%, HbF = 26%.

On night before surgery she was kept on intravenous infusion of ringer lactate at 2ml/kg/hr to avoid dehydration. Patient was explained about the procedure, anesthesia, and the recovery. An informed consent was obtained. Intraoperatively, she received dexamethasone (8 mg) i.v, midazolam(1.5mg) i.v, fentanyl (150μgm) i.v. After preoxygenation general anesthesia was induced with propofol (2 mg/kg) i.v and vecuronium (0.08mg/kg) i.v. Anesthesia was maintained with N 2 O and O 2 (50:50) on circle absorber system with sevoflurane at 1 MAC. CVP, arterial pressure, urine output, and temperature monitoring was carried out. The cardiopulmonary bypass circuit included venous reservoir of 3,000 ml capacity and a hollow fiber oxygenator a heat exchanger and air filter were installed with it.

Cardiopulmonary bypass circuit was primed with ringer lactate (1.5 L), whole blood (1 L), sodium bi carbonate (70 ml), 20% mannitol (70 ml), heparin (3,400 IU), methyl prednisolone (500 mg). After sternotomy, pericardial patch was obtained for closure of the defect. The patient was heparinised with 10200 units of heparin administered intravenously and the measured Activated Clotting Time (ACT) was 440 sec. Aortic and venous cannulations were carried out. Prior initiating bypass, 1000 ml of autologous blood was drained via a side port in the venous line and discarded. At the same time priming fluid was infused into the patient to avoid the possible hemodynamic disturbances. Cardiopulmonary bypass was initiated with flows 2.5 L/min. Mean perfusion pressure was maintained at about 65 mm Hg. After aortic cross clamping, warm blood cardioplegia was delivered. The temperature was maintained near 37 o C. Blood collected by the cardiotomy suckers was discarded. Anticoagulation was monitored with serial ACT at 60 mins and 75 mins and found to be 430 and 420 seconds respectively, requiring no further doses of heparin. The level in reservoir was maintained by addition of fresh blood to it while maintaining the hematocrit near 26% by addition of crystalloids. pH was strictly maintained above 7.4 by addition of sodium bicarbonate according to base deficit on atrial blood gas. The details of cardiopulmonary bypass were: total bypass time = 81 mins, total cross clamp time = 35 mins, rest time = 12 mins. Separation from CPB was facilitated by intravenous infusion of dopamine 10 mcg/kg/min. Residual action of the heparin was reversed with 150 mg of protamine sulphate which was injected very slowly through the arterial monitoring line to avoid fall in systemic blood pressure and rise in pulmonary vascular resistance. ACT was 120 seconds after reversal with protamine ( Base line ACT was 110 seconds). At the end of bypass, blood contained in the reservoir was not returned to patient, it was discarded. The patient was transfused one unit of fresh blood and ringer lactate to maintain the CVP near 10 cm. After surgery patient was transferred to critical care unit (CCU) and mechanically ventilated with Synchronized intermittent mandatory ventilation mode. Recovery was uneventful. Patient was started one more unit of blood (350 ml) as the immediate post operative hemoglobin was 9 gm%. Also, she was given transcutaneous electrical nerve stimulation (TENS) for pain relief. The patient was extubated with appropriate hemodynamic monitoring and pain relief were carried out.

   Discussion Top

Preoperative blood transfusion is indicated in sickle cell disease patient which increases the hematoctrit along with decrease in sickle cell Hb levels. [1],[3] Preoperative sickle cell Hb value of ≤30% was recommended but in our case, the preoperative sickle cell Hb value was brought down to 60% with three units of fresh blood transfusion in combination with hydroxyurea and hematinics which were administered preoperatively. Hydroxyurea in a dose of 10-30 mg/kg has been the main stay of treatment in patients with severe symptoms. [2] Hydroxyurea is known to increase HbF and exerts beneficial effects on red cell hydration, vascular wall adherence, and suppression of granulocytes. [2] A report by Sutton et al., [1] partial exchange transfusion and platelet sequestration have been recommended, but we planned to carry out partial exchange transfusion only using fresh blood (with in 24 hrs of collection). The transfusions were guided by the simple calculations:

Percentage change in hemoglobin after exchange transfusion of 1200 ml of blood=volume of RBC removed/total volume of RBC=36Χ1200/34Χ85=41.5%. So final HbSS=Previous HbSSΧ(1-% change in HbSS)=60Χ(1-41.5/100)=35.1%

It was calculated that with the exchange transfusion of 1200 ml of fresh blood the patient's HbSS level would decrease to 35-36%.

We conducted the CPB under normothermia, since hypothermia lead to vasoconstriction and stasis of red cells. For the same reason warm blood cardioplegia with high Pao 2 and low hematocrit (low viscosity) was administered. [6] Methylprednisolone was also administered in this case, as it can inhibit activation of endothelium caused by surgical stress and sickling, cytokines, and platelet aggregation. [5] We avoided the occurrence of respiratory acidosis postoperatively by avoiding potent opioids. Instead we used TENS to manage sternotomy pain [4] in postoperative period with diclofenac suppository.

   Conclusion Top

Managing sickle cell disease patients on cardiopulmonary bypass is a team work which includes anesthesiologist, physician, surgeon, perfusionist, chest physiotherapist, transfusion therapy and laboratory support. Partial exchange transfusion with fresh blood is an effective tool to optimise the sickle cell Hb value in absence of cell saver. Preoperative optimization of the patient, Intraoperative fluid monitoring, avoiding respiratory depression and acidosis and use of alternative strategies for pain management may have a significant role in management of such patients.

   References Top

1.Sutton SW, Hunley EK, Duncan MA, Rodriguez R, Meyers TP. Sickle cell disease and aortic valve replacement use of cardiopulmonary bypass, partial exchange transfusion, platelet sequestration and continuous hemofiltration. Tex Heart Inst J 1999;26:283-8.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson DL (Eds). Harrisons principles of internal medicine.16th editition.2005.McGraw-Hill, Inc;596-8.   Back to cited text no. 2      
3.Amna MA, Maddali MM. Cardiopulmonary bypass without preoperative exchange transfusion in sicklers. Asian Cardiovasc Thorac Ann 2006;14:51-6.  Back to cited text no. 3      
4.Benedetti F, Amanzio M, Casadio C, Cavallo A, Cianci R, Giobbe R, et al. Control of post-operative pain by transcutaneous electrical nerve stimulation after thoracic operations. Ann Thorac Surg 1997;63:773-6.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Hicks GL, Hill AA, DeWeese JA. Subendocardial protection during cardiopulmonary bypass, its use with methylprednisolone and glucose-insulin-potassium. Arch Surg 1979;114:302-4.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Caputo M, Bryan AJ, Calafiore A, Suleiman MS, Angelini GD. Intermittent antegrade hyperkalaemic warm blood cardioplegia supplemented with magnesium prevents myocardial substrate derangement in patients undergoing coronary artery bypass surgery Eur J Cardiothorac Surg 1998;14:596-601.  Back to cited text no. 6      

Correspondence Address:
Sananta Kumar Dash
Department of Anesthesia and Critical Care, Grant Medical College and Sir JJ Hospital, Byculla, Mumbai-400 008, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.62927

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