Year : 2013 | Volume
: 16 | Issue : 2 | Page : 147--150
Cardiac surgery in a patient with immunological thrombocytopenic purpura: Complications and precautions
Vivek Chowdhry1, BB Mohanty2, Dash Probodh3,
1 Department of Cardiac Anaesthesiology, Care Hospital, Bhubaneswar, Odisha, India
2 Department of Cardiac Surgery, Care Hospital, Bhubaneswar, Odisha, India
3 Department of Hematology, Hi-Tech Medical College Hospital, Bhubaneswar, Odisha, India
Department of Cardiac Anaesthesiology, Care Hopital, Chandrasekharpur, Bhubaneswar - 751 015, Odisha
Immune thrombocytopenic purpura (ITP) patients are at high-risk for bleeding complications during and after cardiac surgeries involving cardiopulmonary bypass. We report a patient with ITP with severe coronary artery disease and mitral valve regurgitation who underwent uncomplicated coronary artery bypass grafting and mitral valve replacement. Three weeks later, the patient was readmitted in a very low general condition with signs of pericardial tamponade. We describe our experience of managing the case.
|How to cite this article:|
Chowdhry V, Mohanty B B, Probodh D. Cardiac surgery in a patient with immunological thrombocytopenic purpura: Complications and precautions.Ann Card Anaesth 2013;16:147-150
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Chowdhry V, Mohanty B B, Probodh D. Cardiac surgery in a patient with immunological thrombocytopenic purpura: Complications and precautions. Ann Card Anaesth [serial online] 2013 [cited 2022 Jan 24 ];16:147-150
Available from: https://www.annals.in/text.asp?2013/16/2/147/109774
Immunological thrombocytopenia, previously referred to as "idiopathic thrombocytopenic purpura"(ITP) is a common hematological disorder which involves immune mediated platelet destruction and impaired platelet production.  It is characterized by isolated thrombocytopenia in the absence of other causes or disorders that may be associated with thrombocytopenia.  The main clinical problem of primary ITP is an increased risk of bleeding although bleeding symptoms may not always be present.  Concomitantly, the ITP patients also present an increased risk of thrombosis and atherosclerosis related to the presence of hemostatic factors and chronic steroid therapy. Although relatively rare, the association of ITP and coronary artery disease represents a complex therapeutic challenge.  Only a few case reports, describe management of patients with ITP undergoing heart surgery. We describe our experience of perioperative management in a patient of ITP who underwent coronary artery bypass grafting (CABG) and mitral valve replacement.
A 55-year-old man was admitted with shortness of breath (New York Heart Association Class III/IV), and stable angina; his exercise test was positive for inducible ischemia with metabolic equivalents of 6.6. Coronary angiography done elsewhere showed triple vessel disease (distal left main stenosis 80%, left anterior descending (LAD) artery stenosis 90%, mid left circumflex artery stenosis 70% and right coronary artery (RCA) stenosis 60%). Echocardiogram revealed anterior mitral leaflet prolapse with severe mitral regurgitation, akinetic distal inter-ventricular septum and apex, global hypokinesia and ejection fraction of 46%. Routine hematological examination was normal except a very low platelet count of 15,000/cmm of blood. His hematologic investigations showed a provisional diagnosis of ITP; a bone marrow biopsy showed normal erythroid and myeloid elements with marked megakaryocytic hyperplasia confirming the diagnosis of ITP. The patient was put on oral prednisolone 30 mg twice daily for 5 days and intravenous immunoglobulin (IVIG) was started at a dose of 400 mg/kg/day for 5 days. After 5 days of treatment, his platelet count increased to 1.3 lakh/cmm. Due to on-going chest discomfort, the patient was scheduled for CABG along with mitral valve replacement. The platelet count on the day of surgery was 64,000/cmm. In the operating room, patient was monitored according to the American Society of Anaesthesiologist standards. Patient was induced with 0.1 mg/kg midazolam and 10 μg/kg fentanyl; his trachea was intubated with a 8.5 mm cuffed endotracheal tube after administration of vecuronium bromide 0.2 mg/kg and he was put on mechanical ventilation with a mixture of 50% oxygen in air. Injection hydrocortisone 100 mg was given after induction. Anaesthesia was maintained with isoflurane and intermittent boluses of fentanyl and pancuronium bromide. After anticoagulation with heparin 2 mg/kg and achieving an activated clotting time of over 300 seconds venous grafts were anastomosed distally to LAD and RCA and proximally to ascending aorta on beating heart with the help of octopus stabilizing system. The venous grafting on beating heart was aimed to decrease the cardiopulmonary bypass (CPB) time. With an additional dose of heparin, ACT of 480 seconds was attained and CPB was initiated. Mitral valve was replaced through left atrial approach with a 29 mm mechanical valve (St. Jude Medical Inc. Minnesota, USA). Thereafter, distal graft to obtuse marginal was performed and proximal anastomosis to ascending aorta was done after release of aortic cross clamp. Patient weaned of CPB without any difficulty. Bleeding was checked thoroughly and heparin was reversed with protamine to normalize ACT. Two units of fresh frozen plasma (FFP) and two units of platelet concentrate were transfused after coming off CPB as per institutional protocol. Two chest tubes were placed, one in the mediastinum and the other in the pericardium. The chest was closed and the patient was transferred to the intensive care unit and put on ventilator with stable hemodynamics. Six hours later, platelets counts were 58,000/cmm of blood. Patient was extubated 18 h after surgery with stable hemodynamics and blood gases. On the 2 nd post-operative day (POD), as his platelet counts were 34,000/cmm, tablet prednisolone 30 mg twice daily was restarted and IVIG 400 mg/kg was given daily for 5 days. The chest drainage measured 450 ml on the day of surgery, 250 ml on 1 st POD, which gradually decreased and chest tubes were removed on 4 th POD. Platelet counts were assessed daily and showed a rising trend. On 7 th POD, the platelets counts were 108,000/cmm of blood. The patient was discharged on 9 th POD with a platelet counts of 96,000/cmm, with an advise to continue tablet prednisolone 30 mg twice daily for 7 days and regular follow-up with hematologist.
After 20 days of discharge, patient was brought to the hospital's emergency department in severe respiratory distress and un-recordable pulse and blood pressure. Immediately, cardio-pulmonary resuscitation was started. Patient was intubated and mechanically ventilated, inotropic agents were started and intra-aortic balloon pump was inserted. Blood was sent for complete blood counts, prothrombin time and international normalized ratio (INR). Echocardiography revealed severe pericardial effusion with signs of tamponade. Immediately, chest was opened on the bed side; pericardial cavity was filled with blood and clots, which was sucked out. However, no any active surgical bleeding site was found. The blood investigation reports showed hemoglobin 7.0 gm%, leukocyte counts 28,000/cmm, platelet count of 68,000/cmm of blood and INR of eight. The patient was given FFP and higher antibiotics. In spite of medical measures, patient's hemodynamic condition did not improve and he could not be saved.
ITP is characterized by an abnormally low platelet count of unknown cause. The incidence of ITP among adults, ranges from 1.6 to 2.68 per 100,000 per year,  the male to female ratio in the adult group varies from 1:1.2 to 1.7 and the median age of adults at the diagnosis is 56-60 year.  In ITP, IgG antiplatelet autoantibodies are produced against the platelet glycoprotein IIb/IIIa or GPIb/IX in about 75% of patients causing both platelet destruction and inhibition of thrombopoiesis.  The main problem in patients of ITP is an increased risk of bleeding although bleeding symptoms may not always be present. Bleeding after CPB surgery is common with about 7% of patients requiring re-operation to control bleeding.  Pre-operative thrombocytopenia, CPB induced thrombocytopenia  as well as platelet dysfunction and post-operative anticoagulation are expected to increases risk of pericardial effusion and cardiac tamponade, which may occur in early as well as late (5-7 days after surgery) post-operative periods.  The incidence of cardiac tamponade after cardiac surgery is about 0.5-5.8%.  Therefore, assessing the magnitude of bleeding risk in ITP patients is an important consideration in managing such cases.
Traditional frontline treatment, including corticosteroids and IVIG are effective and typically cause transient elevations in platelet counts. Second and third line therapies, including rituximab, splenectomy, thrombopoietin receptor agonists (TPO-A) and immune-suppressants are often successful and may cause a long-term increase in the platelet counts.  Patients with asymptomatic mild or moderate thrombocytopenia can be followed up with no treatment as platelet counts greater than 50,000/cmm are usually not associated with clinically important bleeding; such patients can safely undergo invasive procedures.  Glucocorticoid is the conventional initial modality of treatment, and usually produces a significant response in about 70% of patients; relapses are common when the glucocorticoid dosage is reduced. Platelet transfusion is useful in instances of severe hemorrhage, although it may be short acting.  Infusion of IVIG has an immediate effect in increasing platelet counts as it blocks the Crystallizable fragment (Fc) receptors of macrophages; as a result, the antibody-coated platelets cannot attach to macrophages, thus avoiding destruction by phagocytosis.  TPO-A increases the platelet count via stimulating megakaryocytopoiesis. ,
The present patient had an uneventful intraoperative course and there was no difficulty in obtaining surgical hemostasis. In the post-operative period also the patient did not have much bleeding and bleeding related complications. The decrease in the platelet count was managed successfully with platelet transfusion, steroids and IVIG. Patient was discharged with acceptable platelet counts and with an advice to take anticoagulants, timely INR monitoring and regular follow-up. The patient was re-admitted in a very poor general condition with respiratory distress, and un-recordable pulse and blood pressure; the leucocyte count and INR also was high. Although a definitive diagnosis could not be made sepsis, excessive anticoagulation and neglect in timely follow-up were the possible reasons for this deterioration.
The risk of bleeding could be more pronounced in ITP patients if patients are being treated with anticoagulants; perhaps, anticoagulation and increased risk of bleeding, lead to pericardial effusion and tamponade. Hence, patients with ITP, at the time of discharge, should be educated about the clinical features of pericardial effusion and tamponade (such as shortness of breath, dizziness, swelling of legs, and decrease in urine output and generalized weakness) and they should be advised to seek immediate medical attention in case of presence of such clinical features. Further, in the presence of clinical suspicion, such patients should be investigated by chest X-ray and echocardiography to exclude any pericardial effusion or tamponade. The INR and platelet counts should also be monitored aggressively; however, how often they should be monitored is not well defined. Preferential use of bio-prosthetic valve may also reduce the risk of bleeding complications owing to decreased anticoagulant requirement. Besides this, other factors or comorbid conditions like sepsis, diabetes, renal and hepatic dysfunction can also precipitate coagulopathy and potentiate the effects of oral anticoagulants.  Lastly, ITP should be managed aggressively using second and third line treatment modalities to have long-term benefits.
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