Cardiac surgery in patients with sickle cell disease
J Khandeparkar1, M Porwal1, V Mahajan2,
1 Department of CardioVascular and Thoracic Surgery, CHL Hospital, Indore, Madhya Pradesh, India
2 Department of Anesthesia, CHL Hospital, Indore, Madhya Pradesh, India
Department of CardioVascular and Thoracic Surgery, CHL Hospital, Indore, Madhya Pradesh
|How to cite this article:|
Khandeparkar J, Porwal M, Mahajan V. Cardiac surgery in patients with sickle cell disease.J Postgrad Med 2013;59:167-167
|How to cite this URL:|
Khandeparkar J, Porwal M, Mahajan V. Cardiac surgery in patients with sickle cell disease. J Postgrad Med [serial online] 2013 [cited 2022 Dec 4 ];59:167-167
Available from: https://www.jpgmonline.com/text.asp?2013/59/2/167/113827
Sickle cell hemoglobinopathy is an inherited disorder manifesting as heterozygous (HbAS) sickle cell trait or homozygous (HbSS) sickle cell disease (SCD).  The literature on cardiac surgery in SCD is limited to case reports or small series only.  We report herewith a 12-year-old female with SCD who underwent successful repair of the ostium secundum atrial septal defect (ASD).
A 12-year-old female with SCD was diagnosed to have an ASD measuring 30 mm × 24 mm with ill-defined margins with mild pulmonary arterial hypertension. On admission, her hemoglobin (Hb) was 9.1 g/dl, PCV was 28.1 and HbSS 79.5%. Her coagulation profile, electrolytes, hepatic, and renal functions were normal. She received an exchange transfusion (ET) of 1 unit of fresh homologous cross-matched packed cell transfusion. Her Hb improved to 10.5 g/dl. She underwent intracardiac repair of ASD under normothermic cardiopulmonary bypass (CPB) with warm blood potassium cardioplegia and fast track anesthesia protocol. CPB was conducted using membrane oxygenator and no filter was used.
Being pre-medicated with diazepam 4 h before surgery, anesthesia was induced with midazolam, fentanyl and propofol and maintained with sevoflurane. Muscle relaxation was achieved with pancuronium. An infusion of propofol 2 mg/kg/min was continued intraoperatively and during CPB. She received tranexamic acid during the surgery.
With CPB ready, she received an ET of 1 unit of fresh autologous packed cells before the initiation of CPB. The prime was warmed and oxygenated. Her Hb was 8.3 g/dl and PCV was 25 during CPB. The cross clamp time was 11 min and total CPB time was 15 min. The ASD was closed directly using 4'0 polypropelene suture. She was weaned off CPB without any inotropes. She was extubated at the end of surgery with adequate reversal of anesthesia. Post-operative analgesia was maintained using fentanyl infusion and paracetamol injections. Her post-operative Hb was 11.6 g/dl with normal hepatic and renal functions and had no residual shunt on echocardiography. She was discharged on tab folic acid 5 mg once a day on 6 th post-operative day. We adopted the technique of pre- and per-operative ET as our patient had a low Hb pre-operatively. As we anticipated a short CPB time, we did not use hemofiltration though, this has been practised by some.  It does not appear necessary to repeat Hb electrophoretic studies after an uneventful surgery. 
There is no consensus, however, about the per-operative management of the patient with SCD undergoing cardiac surgery under CPB. Though pre- and per-operative ETs have been recommended by some  a conservative management was found as effective.  While the technique of CPB has varied from moderate hypothermia  to normothermia  that of myocardial protection has been ventricular fibrillation  to cardioplegically arrested heart ,, to warm beating heart  with equal success. Intracardiac procedures can be safely performed under CPB in the patients with SCD. The cardinal principles of management of such patients are however to avoid the sickling triggers viz. hypoperfusion, hypothermia, acidosis and hypoxia during intracardiac surgeries.  The need for ET must be decided by the pre-operative Hb value, severity of the SCD and the complexity of the intracardiac procedure.
We are grateful to Dr. Rajesh Jain, Medical Director, CHL Hospital, Indore for his kind permission to use the hospital data and publish this paper.
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