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Thorax: first published as 10.1136/thx.36.2.151 on 1 February 1981. Downloaded from

Thorax, 1981, 36, 151-152

Mitral using cold in a patient with sickle cell trait

I HUDSON, I A DAVIDSON, AND C G A McGREGOR From the Department of , Royal Infirmary, Edinburgh

Case reports Table Results of plasma haemoglobin and CKMB estimations A 13-year-old negro girl from Sierra Leone was referred for replacement. Her weight Time Plasma Hb CK-MB % CKMB oftotal CK was 40 kg and she had a history of progressive gil "/l /ll exertional dyspnoea and paroxysmal nocturnal dysp- Preoperative 0-002 <10 Prebypass 0117 7 7 noea. Medication consisted of digoxin 0-25 mg/day, End of bypass 0-048 16 9 frusemide 40 mg/day, and potassium supplements. 3 hours 0-18 24 5 At cardiac catheterisation the mean simultaneous 6 hours 0-08 20 4 was with a cardiac 9 hours 01 15 3 mitral valve gradient 17mmHg 12 hours 0 08 12 1 index of 2 8 l/m2/min, and left ventriculography 24 hours 0-076 17 1 showed good left ventricular function with gross reflux into an enlarged left . The haemoglobin was 10 8 g/dl. The presence of sickling had been noted on a haemoglobin were measured before operation and blood film. The SICKLEDEX test was positive and at 3, 6, 9, 12, and 24 hours after cardiopulmonary electrophoretic examination of the haemoglobin re- bypass. Electrocardiograms were obtained before for one The vealed one band in the A and one band in the S cperation and daily afterwards week. copyright. position. The haemoglobin F fraction was 13 % table shows the results of the plasma Hb and CKMB Quantitative estimation of haemoglobin S was not estimations. available. Recovery was uneventful and the patient was dis- was performed on charged on the thirteenth day after operation. There 13 September 1978. Before the institution of cardio- were no electrocardiographic changes indicative of operative myocardial injury. pulmonary bypass 1400 ml of fresh whole blood was http://thorax.bmj.com/ exchange-transfused with the addition of 50 ml 8 -4% sodium bicarbonate soluticn. Bypass flow rate was Discussion 2-4 l/m2/min using a disposable Harvey oxygenator. The initial haematocrit on bypass was 19%. The In 1967 Leachman et all reported a case of systemic patient was not systemically cooled but the tem- sickle cell thrombi in the lungs, kidneys, and perature was allowed to fall to 33°C. The was brain in a patient with sickle cell trait complicating cross-clamped and 500ml of cold (4°C) solution of replacement and resulting in the patient's dextrose 5% in 0-9% saline containing 10mmol death. Since then, several authors have reported potassium chloride was infused into the aortic root. successful in patients with Topical hypothermia was achieved by pouring cold sickle cell disease.2-4 These writers have stressed the on September 29, 2021 by guest. Protected (4°C) normal saline over the heart and by con- need for close monitoring of p02, pH, and tem- tinuous pericardial irrigation. perature to avoid hypoxia, acidosis, and hypothermia, There was no clot in the left atrium. The grossly factors known to precipitate the "vicious circle" of abnormal mitral valve was excised, and replaced by vaso-occlusion and irreversible capillary obstruction a 31 mm Carpentier-Edwards porcine prosthesis. The resulting in tissue injury. patient was rewarmed and the heart beat in sinus The use of tissue valves has been advocated to rhythm after one direct current shock (10 joules). avoid the increased blood trauma caused by mech- The h,eart maintained a good output without inotropic anical prostheses but Craenen inserted a Starr- support after the discontinuation of cardiopulmonary Edwards mitral prosthesis in a child with sickle cell bypass. The total bypass time was 65 minutes and anaemia and noted no increased haemolysis. Wenham the aortic cross-clamp time 43 minutes. et a15 showed no significant haemolysis in a patient Creatine kinase MB isoenzyme, total creatine wit,h sickle cell trait after kinase, urea stable lactate dehydrogenase, aspartate with a Bjork-Shiley prosthesis. Szentpetery felt on a transaminase, alanine aminotransferase, and plasma theoretical basis that topical hypothermia was con- traindicated in patients with sickle cell disease and Address for reprint requests: Mr CGA McGregor, Department of that local sickling and myocardial injury might Clinical Surgery, Royal Infirmary, Edinburgh EH3 9YW. result.4 151 Thorax: first published as 10.1136/thx.36.2.151 on 1 February 1981. Downloaded from

152 I Hudson, I A Davidson, and C G A McGregor In the present case there is no evidence either indicative of myocardial injury in the electrocardio- clinically, electrocardiographically, or enzymatically gram over the operative period. that significant myocardial injury occurred. Levels of CKMB of <25 ,u/l found in our patient indicate We would like to thank Mr RJM McCormack for that no significant myocardial injury had occurred. permission to report this case. The non-specific enzymes were likewis,e not signifi- cantly increased. Plasma free haemoglobin levels References were no higher than would be expected during the course of normal cardiopulmonary bypass. 1 Leachman RD, Miller WT, Atias IM. Sickle cell We therefore conclude that potassium-induced trait complicated by sickle cell thrombi after open heart surgery. Am Heart J 1967; 74:268-70. cardioplegia and topical hypothermia in conjunction 2 Yacoub MH, Baron J, Et-Etr A, Kittle F. Aortic with pre-bypass exchange transfusion and haemo- homograft replacement of the mitral valve in dilution can be safely used during cardiopulmonary sickle cell trait. J Thorac Cardiovasc Surg 1970; bypass in patients with tihe sickle cell trait. 59:568-73. 3 Craenen J, Kilman J, Hosier DM, Weinberger M. Mitral valve replacement in a child with sickle Post scriptum cell anemia. J Thorac Cardiovasc Surg 1972; 63: 797-9. A second patient with sickle cell trait has undergone 4 Szentpetery S, Robertson L, Lower RR. Com- valve replacement in this hospital using the tech- plete repair of tetralogy associated with sickle cell niques of myocardial protection described in this anaemia and G-6-PD deficiency. J Thorac Cardio- vasc Surg 1976; 72:276-9. paper. There was no evidence of any myocardial 5 Wenham PW, Scott GL, Wisheart JD. Red cell injury using enzyme and isoenzyme estimation and survival after aortic valve replacement with the patient had a negative postoperative technetium Bjork-Shiley prosthesis in presence of sickle cell pyrophosphate scan. There were likewise no changes trait. Br Heart J 1978; 40:703-4. copyright. http://thorax.bmj.com/ on September 29, 2021 by guest. Protected