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Year : 2007  |  Volume : 51  |  Issue : 6  |  Page : 541-545 Table of Contents     

Open heart surgery for cyanotic heart disease in a child with immune thrombocytopenic purpura:a case report

1 MBBS, MD, Associate Prof, Department of Cardiac Anaesthesia, Department of Cardiac Anaesthesia, Cardiothoracic Sciences Center, All India Institute of Medical Sciences, New Delhi, India
2 MBBS, MD, Senior Resident, Department of Cardiac Anaesthesia, Department of Cardiac Anaesthesia, Cardiothoracic Sciences Center, All India Institute of Medical Sciences, New Delhi, India
3 MBBS, MD, Professor & HOD, Department of Cardiac Anaesthesia, Department of Cardiac Anaesthesia, Cardiothoracic Sciences Center, All India Institute of Medical Sciences, New Delhi, India

Date of Acceptance25-Oct-2007
Date of Web Publication20-Mar-2010

Correspondence Address:
Usha Kiran
Professor & HOD, Department of CardiacAnaesthesia, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

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Acute immune thrombocytopenic purpura in children, though a benign self limiting condition, at times complicated with life threatening haemorrhage. In spite of bleeding episode it is inevitable that surgical procedure will be performed on patients with this disease. The anaesthetic implications therefore need to be addressed. Although few reports describing the perioperative management of patients with chronic idiopathic thrombocytopenic purpura are available, literature regarding open heart surgery for cyanotic heart disease is lacking. We describe the management course and raise some anaesthetic considerations for a two year old child with acute immune thrombocytopenic purpura and tetralogy of Fallot's who underwent emergency surgical correction of the cardiac problem under extracorporeal circulation.

Keywords: Acute immune thrombocytopenic purpura, Children, Cardiac surgery

How to cite this article:
Choudhury M, Pal N, Kiran U. Open heart surgery for cyanotic heart disease in a child with immune thrombocytopenic purpura:a case report. Indian J Anaesth 2007;51:541-5

How to cite this URL:
Choudhury M, Pal N, Kiran U. Open heart surgery for cyanotic heart disease in a child with immune thrombocytopenic purpura:a case report. Indian J Anaesth [serial online] 2007 [cited 2021 Jun 19];51:541-5. Available from: https://www.ijaweb.org/text.asp?2007/51/6/541/61197

   Introduction Top

Immune thrombocytopenic purpura is primarily a disorder of increased platelet destruction mediated by autoantibodies to platelet membrane antigens. [1] There­fore surgery in this group of cases carries the risk of major bleeding [2] .Although there are a number of reports describing the management of the patients undergoing obstetric or general surgical procedures [3],[4],[5] ; literature on perioperative management of idiopathic thrombocy­topenic purpura (ITP) patients undergoing open heart surgery are not plenty and there is no report describing acute ITP patient undergoing corrective surgery for cy­anotic heart disease.

We report the perioperative management of a child with acute immune thrombocytopenic purpura (ITP) who successfully underwent emergency surgical correction of the underlying cardiac problem.

   Case report Top

A two-year-old male baby weighing 10 kg, diag­nosed case of Down's syndrome; admitted to our hospi­tal with the historyof increasingcyanosis since one month of age, breathlessness for one month, fever and easy bruising for five days. Clinical examination revealed the presence of petechiae around ankle, back of the body and the spleen was enlarged by 2 cm below the costal margin. Laboratory investigation showed abnormally large platelets (3-5µ in diameter) and more than normal variation in shape. There was presence of platelet micro particles. The mean platelet volume was 9.9fl. The total platelet count was 12000 mm 3 .The total leukocyte count and differential count were normal. The results of tests of blood coagulation, including prothrombin time, partial thromboplastin time, and fibrinogenwas normal. The level of fibrinogen degradation products was 7µgm/ml. Bone marrow examination revealed megakaryocytosis, giant megakaryocyte with scanty cytoplasm and relatively few granules than normal platelets. The clinical haematologist confirmed the diagnosis of acute immune thrombocy­topenic purpura (ITP). The chest x-ray, electrocardio­graph and echocardiography examination identified him as a case of tetralogy of Fallot's. He was treated with oral prednisolone at a dose of 4mg.kg -1 .day -1 for 7 days taper to 21 days. Post treatment his platelet count was 1, 35000/mm 3 .Although an operation had been offered, his parents refused due to financial difficulties. One month later he was brought to the emergency department with the complaint of increased breathlessness and recurrent cyanotic spells. He had two bouts of epistaxis immedi­ately after hospitalization. Physical examination revealed the presence of petechial haemorragic spots on the skin, respiratory rate 40-45/ minute, heart rate 135/minute, regular; and blood pressure 105/85mmHg. On auscultation, his chest was clear; there was presence of systolic murmur at the left upper sternal border. Abdominal ex­amination did not reveal any organomegaly. Treatment was started with oxygen inhalation, fluid therapy, intra­venous morphine, propranolol and sodium bicarbonate. Simultaneously other investigations were carried upon. Haematological examination revealed; haemoglobin level of 18.8 gm.dL -1 with normocytic normochromic RBCs; a platelet count of 20,000/mm 3 , abnormally large plate­lets and presence of platelet microparticles. Auto anti­bodies to platelet glycoprotein IIb and IIIa were present (detected by antigen capture ELISA test). The total leu­kocyte count and differential count were normal. He had deficient clot retraction and prolonged bleeding time; which was 24 min (control: 12 min). The prothrombin time, partial thromboplastin time and fibrinogen level were within normal limits; however the fibrin degradation prod­uct level was 12 gm.dL -1 . Coomb's test was negative. Specific assay for individual coagulation factor was not done. Electrocardiograph showed the features of right ventricular hypertrophy. X ray chest demonstrated a normal sized boot shaped heart with decreased pulmo­nary vascular marking. For the bleeding episode he was treated with one unit of single donor apheresis platelet transfusion, one day of immunoglobulin therapy at a dose of 1g.kg -1 and methylprednisolone at a dose of 30 mg.kg­1 day -1 for two days. Post treatment his platelet count was 150,000/mm 3 . He underwent diagnostic cardiac catheterization under general anaesthesia which revealed the presence of tetralogy of Fallot's. The child was pre treated with topical EMLA cream applied at the groin one hour prior to local lidocaine infiltration at the start of catheterization. He was given 0.05 mg.kg -1 midazolam intravenously at the onset of the procedure and 1 mg.kg­1 intravenous katamine during the procedure. There was presence of moderate sized aortopulmonary collaterals. The coronary anatomy was normal. There was no ex­istence of associated aortic valve regurgitation, patent ductus arteriosus and coarctation of aorta. At the 6 th hour following cardiac catheterization he had four more episodes of hyper cyanotic spell. The partial pressure of oxygen (PaO 2 ) dropped down to as low as 20mmHg during the spell which was 58 mmHg while the child used to be comfortable. As the "tet spell" was non re­spondent to the medical management, emergency surgi­cal correction was decided upon. The child received 0.2 mg.kg -1 morphine sulphate and 0.02 mg.kg -1 propranonol hydrochloride intravenously before shifting to the opera­tion theatre.After startingelectrocardiograph, pulse oxim­etry and non invasive blood pressure monitoring anaes­thesia induction was started with intravenous ketamine (2 mg.kg -1 ). As soon as the child was asleep oxygen mask was placed over his face and rocuronium bromide 0.06 mg.kg -1 was given. Number 5.0 size uncuffed en­dotracheal tube was passed orally with the help of a stylet to secure the airway. The child was mechanically ventilated with a mixture of oxygen and air in a mixture of 50:50. Anaesthesia was maintained with fentanyl, midazolam and pancuronium bromide. Optimal care was taken to avoid injury during laryngoscopy, intubation and temperature probe insertion. Continuous pulse oximetry, nasopharyngeal temperature, invasive blood pressure, end- tidal carbon dioxide, inspired concentration of oxy­gen( FiO 2 ), central venous pressure , bispectral index and urine output were monitored throughout the course of anaesthesia and surgery. The haemoglobin level and platelet count as sent before shifting the patient before surgery showed a value of 19gm.dl -1 and 1, 20, 000/mm 3 respectively.The post intubation blood gas value revealed a PaO 2 of 59mmHg, PaCO 2 35mmHg. There was no feature of metabolic acidosis. During the prebypass pe­riod care was taken to maintain the optimal haemodynamics, oxygenation and intraoperative factor which could precipitate the cyanotic spell. He received methylprednisolone 30mg.kg -1 and aprotinin 100,000 KI Units.kg -1 .hr -1 during the intraoperative period. The base line activated clotting time (ACT) was 136 seconds and came to 265 seconds after systemic heparinization with 300 units.kg -1 body weight of heparin .After addition of 200 units.kg -1 of heparin theACT raised to 325 seconds. Keeping the possibility of heparin resistance secondary to antithrombin III (AT III) deficiency blood sample was sent for AT III and platelet factor 4 (PF 4 ) estimation and one unit fresh frozen plasma was transfused which raised the ACT to 425 seconds and cardiopulmonary bypass was initiated. The cardiopulmonary bypass (CPB) circuit was primed with 2 units of blood, 500 ml of Ringer lactate, 50 ml of 20% mannitol, and 50 mg of heparin and 20 ml of sodium bicarbonate. Heart was arrested with antegrade hyperkalemic cold blood cardioplegia. The patient was cooled to a venous return temperature of 28 0 C while on bypass and was rewarmed to a rectal temperature of 36 0 C before separation from CPB. Two units of fresh frozen plasma and 10 ml of 20% albumin were added to the pump during CPB to maintain the prime volume. The haematocrit was maintained above 30% during the course of CPB.Additional 100 units.kg­1 of heparin was added in the pump and the ACT re­mained between 400-565 seconds through out. Separa­tion from cardiopulmonary bypass was accomplished with dopamine 5µg.kg -1 .min -1 and sodium nitroprusside 0.5µg.kg -1 min -1 . The haemoglobin level remained be­tween 10 to 13 gm.dL -1 during CPB and 14 gm.dL -1 in the immediate post CPB period. Protamine neutraliza­tion of heparin resulted an ACT level of 131 seconds. The total duration of surgery, CPB time and aortic cross clamp time were 185 min 112 min and 68 min respec­tively. One unit of single donor apheresis platelet (SDP) was transfused as the platelet count dropped as low as 24,000 mm 3 during cardiopulmonary by pass. Haemostasis after CPB required the same time as the usual cases of similar group. No residual solution from CPB circuit was transfused to the patient. At the second postoperative hour his bleeding time, prothrombin time and activated partial thromboplastin time were found to be normal. He was extubated on the sixth post operative hour. There was no significant bleeding after 24 hour of surgery in spite a platelet level of 42,000/mm 3 . The bleeding time was 25 min at the same time. Hence to prevent a sud­den onset of bleeding episode the authors had to give platelet transfusion (one unit SDP) to maintain a platelet count more than 125,000/mm 3 . On the 3rd postoperative day he developed sudden onset of features of cardiac tamponade which was confirmed by transoesophageal echocardiography. The tamponade was released under general anaesthesia with midazolam 0.05 mg.kg -1 and fentanyl 2µg.kg -1 along with oxygen and air in the ratio of 50:50. It was found to be caused by the migration of pacing wire into one of the coronary veins.Approximately 200 ml of collected blood was taken out. He received one unit of fresh blood transfusion as the haemoglobin level dropped down to as low as 10gm.dL -1 . He was extubated 3 hours later. From the 4th post operative day onwards platelet count remained stable at 135,000/mm 3 . Chest tubes were removed on the 5th post operative day with a cumulative drainage of 225 ml which was little higher than the similar cases in our setup. During the whole postoperative course he received two unit of fresh blood, one unit of fresh frozen plasma and one unit of SDP .He was discharged on the 8th post operative day. The pre CPB blood sample revealed no abnormality in PF4 where as the AT III level was found to be 60% and the result was included in the discharge summary for the future precaution.

   Discussion Top

The American Society of Hematology Panel de­fined idiopathic thrombocytopenic purpura as "isolated thrombocytopenia with no clinically apparent associated conditions or other causes of thrombocytopenia, eg. sys­temic lupus erythematosus and stressed that the diagno­sis of idiopathic thrombocytopenic purpura is primarily one of the exclusion. [6] Acute immune thrombocytopenic purpura in childhood though a self limiting disorder , at times complicated by serious bleeding. [7] The risk of life threatening haemorrhage is highest within the first few weeks so affected children should avoid trauma and re­strict physical activity as much as possible. [8] Current treat­ment strategies for acute ITP include oral or intrave­nous corticosteroids, anti-Rh(D), intravenous immunoglobin and observation alone. [9] Although there is no published data on the efficacy of different treatments for the management of children with urgent life threat­ening bleeding, there is universal agreement that such children require aggressive therapy. Appropriate inter­ventions in this group include high dose intravenous cor­ticosteroid,intravenousimmunoglobulin andplatelet trans­fusion. [10]

Platelet consumption and dysfunction is common sequelae of CPB technique among a whole array of potential complications. Resultant bleedingdiathesis com­pounded by an accompanying cascade of coagulopathy can become a major perioperative concern, culminating in significant transfusion and reexploration . [11] On the other hand; immune thrombocytopenic purpura can it­self contribute to a very low platelet count leading to feared complications like intra cranial haemorrhage [12] . The association of cyanotic heart disease, deranged plate­let function, coagulation factor dysfunction and increased incidence of excessive post operative bleeding is well known. [13] Thus it is possible for the platelet count to fall to excessively low levels during and after cardiopulmo­nary bypass in such patients suffering from ITP leading to a complicated course. Therefore intensive perioperative management should be planned for patients with ITP in an attempt to avoid the depletion of platelets as well as the coagulation factors.

In patient suffering from ITP preoperative immu­nosuppressive therapy and corticosteroid treatment seem to be unsuitable due to high incidence of post operative infectious complications. High dose gamma globulin therapy was found to be more appropriate in such cases. [14] However in spite of pre-treatment with high dose corti­costeroid and immunoglobulin our patient did not show any evidence of infection during the post operative pe­riod.

We support Sato, Inoue and colleagues who sug­gested that ITP cases may possibly undergo cardiac sur­gery under cardiopulmonary bypass without a markedly enhanced risk for bleeding complications, despite a more than usual transfusion requirement and significantly low platelet count perioperatively. [15],[16] As the haemorrhagic risk increase with haemodilution the haematocrit level was maintained more than 30% during cardiopulmonary bypass which was the usual period of haemodilution. [17] Jobes and colleagues recommended fresh whole blood as part of the priming to improve haemostasis after car­diopulmonary bypass. [18] It is also an established fact that anaemia is lessened with fresh whole blood, whereas excessive bleeding and transfusion are minimized. [19],[20] The above truths were achieved by blood priming and addi­tion of albumin and fresh frozen plasma rather than crys­talloid to maintain the pump volume during cardiopulmo­nary bypass. Hypothermia, another contributory factor for bleeding especially in cardiac surgical procedure has been associated with dysfunction of enzymatic function, reduced platelet activity and /or altered fibrinolysis. [21] To reduce the hypothermia related bleeding episode tem­perature was maintained at 36-37 0 C through out the post­operative course with the help of warming blanket.

The role of prophylactic platelet transfusion has been described in severely thrombocytopenic patients without evidence of bleeding but no randomized data prove the safety or efficacy of this approach. [22] The is­sue regarding the role of prophylactic platelet transfu­sion before surgery in thrombocytopenic surgical patients is also controversial. [23] Lemmer advocated prophylactic transfusion of one platelet dose (either one apheresis platelet or six random donor unit) after administration of protamine in case of qualitative platelet defect caused by abciximab.Additional platelets and other blood prod­ucts should be transfused as indicated by usual clinical and laboratory measurements [24] . Therapeutic platelet transfusion have been documented to control bleeding , and the mortality rates are not increased when compar­ing patients receiving therapeutic to that seen in patients receiving prophylactic platelet transfusion. [25] Threshold platelet counts for perioperative transfusion have not yet been clearly defined and should be determined by the existence of haemorrhagic factors. The standard thresh­old for haemorrhagic risk for surgery is 50,000/mm 3 in­cluding cardiac surgery with cardiopulmonary bypass. [26] Our patient needed platelet transfusion after hospitaliza­tion because of severity of the disease condition and emergency of surgical management. The child required platelet transfusion both intra and postoperatively as the platelet count dropped down below the safety level. Multimodality approach was applied to him because the severe acute presentation of ITP. We chose single do­nor apheresis platelet transfusion to reduce donor expo­sure and risk for virus transmission or HLA alloimmunization and of its cost effectiveness. [27],[28] .

Haemorrhagic diathesis due to ITP may cause con­siderable risk of bleeding and aspiration into the lungs. Traumaduringmaskventilationandlaryngoscopyandeven trivial trauma by the adhesive electrodes and adhesive tapes used for the fixation of the endotracheal tube may precipitate bleedingin patients with ITP. Possibility of dif­ficult intubation in our case due to the presence of large tongue and high arched palate was a potential risk factor for additional trauma and bleeding during laryngoscopy andintubation.This wasavoidedbysmooth andatraumatic intubation with an appropriate size of endotracheal tube, expert help and good muscle relaxation.

Our report showed that emergency cardiopulmo­nary bypass and cardiac surgery can be safely performed in cyanotic patient with acute ITP combined with AT III deficiency. Preoperative platelet transfusion, combined immunoglobulin and corticosteroid treatment may be helpful if the presentation of ITP is severe and emer­gency surgery is required. Strict vigilance and optimum care should be taken during cardiopulmonary bypass and there after to avoid bleeding due to dilutional anaemia and low platelet count. Use of single donor apheresis platelet transfusion can minimize the volume over load in a small child without causing any excessive bleeding, coagulopathy and thereby decreasing the additional de­mand from the blood bank.

   References Top

1.Sandler SG. Review: immune thrombocytopenic purpura: an update for immunohematologists . Immunohematol 2004; 20: 112-117.  Back to cited text no. 1      
2.Koner O, Cetin G, Karaoglu k, Seren S, Bakay C. Fresh whole blood and immunoglobulin permit coronary artery bypass graft surgery in patients with idiopathic thrombocytopenic purpura. J Cardiothorac Vasc Anesth 2001: 15:483-484.  Back to cited text no. 2      
3.Govindswamy S, Chandler J Latimer R, Vuylsteke A. Manage­ment of the patient with coagulation disorders. Current opin­ion in Anaesthesiology 2002; 15:19-25.  Back to cited text no. 3      
4.Delaitre B, Blezel E, Samama G, et al. Laparoscopic splenec­tomy for idiopathic thrombocytopenic purpura. Surg Laparosc Endosc Percutan Tech 2002; 12: 412-419.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]  
5.Rao MS, Sherke RR.Anaesthetic management of splenectomy in Evan's syndrome during pregnancy induced hypertension. J Postgrad Med 2001; 47: 196-198.  Back to cited text no. 5  [PUBMED]  Medknow Journal  
6.George JN,Woolf SH,Raskob GE,et al. Idiopathic thrombocy­topenic purpura: a practice guideline developed by explicit methods for the American Society of hematology. Blood 1996;88:3-40.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]  
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8.Rostoj S,Hedlund -Treutiger I, Rajantie J, et al; NOPHO ITP working group. Duration and morbidity of newly diagnosed idiopathic thrombocytopenic purpura in children: a prospec­tive study of an unselected cohort. J Pediatr 2003; 143:302­-307.  Back to cited text no. 8      
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11.Paparella D, Brister SJ, Buchann MR. Coagulation disorder of cardiopulmonary bypass: a review. Intensive Care Med 2004; 30: 1873 - 1881.  Back to cited text no. 11      
12.Butros LJ, Bussel JB. Intra cranial haemorrhage in immune thrombocytopenic purpura: a retrospective analysis. J Peditr Hematol Oncol 2003; 25: 660 -664.  Back to cited text no. 12      
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18.Jobes DR,Nicolson Sc,Steven JM. Inhibition and restoration of hemostasis in the young cardiac surgical patient. Cardiol Young 1993; 3:370-377.  Back to cited text no. 18      
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22.Heal JM Blumberg N. Optimizing platelet transfusion therapy.Blood Rev 2004; 18: 149- 165.  Back to cited text no. 22      
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24.Lemmer JH, Metzdorff MT, Krause AH, et al. Emergency coronary artery bypass graft surgery in abciximab treated pa­tients. Ann Thorac Surg 2000;69:90-5.  Back to cited text no. 24      
25.Slihcter SJ. Relationship between platelet count and bleeding risk in thrombocytopenic patients. Transfus Med Rev 2004;18:153-67.  Back to cited text no. 25      
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27.Ness PM, Campbell-Lee SA. Single donor versus pooled ran­dom donor platelet concentrates. Curr Opin Hematol 2001; 8:392-6.  Back to cited text no. 27  [PUBMED]  [FULLTEXT]  
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