The use of cardiopulmonary bypass in a patient with Fallot's tetralogy and hereditary spherocytosis is described. A method of evaluating the fragility of the red cells is given.S. Afr. Med. J., 48, 1535 (1974)

Holloway, A.M.

Hutton, W.S.

Moyes, D.G.

English

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27 Julie 1974 S.-A. MEDIESE TYDSKRIF 1535Correction of Fallot's Tetralogy in a PatientSuffering from Hereditary SpherocytosisD. G. MOYES, A. M. HOLLOWAY, W. S. HUTTON-SUMMARYThe use of cardiopulmonary bypass in a patient witl!Fallot's tetralogy and heredilary spherocytosis is described.A method of evaluating the fragility of the red cells isgiven.s. Afr. Med. J., 48, 1535 (1974).Hereditary spherocytosis is a genetically determinedabnormality of the red blood cells. A defect in intra-cellular glycolysis results in a spherical cell which has ashorter life span than normal and exhibits increasedosmotic fragility in hypotonic saline solutions. Lys:s isreduced in glucose solutions.' ormal formed elements inblood are prone to destruction during cardiopulmonarybypass, due to trauma, cardiotomy suction, bubble oxy-genation and roller pumping.The problem of the imposition of cardiopulmonarybypass on patients suffering from excessive fragi'ity oftheir red cells give3 rise to concern for the safe conductof such a bypass.' Haemo:ysis normally occurs at a ratewhich is seldom less than 0,25 mg of free haemoglobinper minute, but renal function is usually able to tolerateplasma haemoglobin levels of up to 250 mg/1oo ml.The oxygen-carrying capacity is not seriously deranged.A case report is presented on the conduct of cardio-pulmonary bypass in a patient suffer;ng from hereditaryspherocytosis.CASE REPORTAn Indian boy, aged 5 years, pre3ented with Fallot'stetralogy and a typical history of tiring on exertion andsquatting. He was cyanosed. The clinical diagnosis wasconfirmed by cardiac catheterisation. This showed markedinfundibular narrowing of the right ventricle. with agradient of 67 mmHg; a patent foramen ovale; a right-to-left shunt of 56% with a satisfactory pulmonary artery.The pulmonary vascular resistance to the systemic vas-cular resistance ratio was calculated at 11.%. The child'sfather and 4-year-old sibling were known cases ofDepartment of Anaesthetics, University of Natal, DurbanD. G. MOYES, M.B. CH.B., I..R.C.P., F.F.A., Principal Anaesthe-tist and Senior LecturerA. M. HOLLOWAY, M.B. B.S., F.F.A., Principal AnaesthetistCardiothoracic Laboratory, Wentworth Hospital, Durban\v. S. HUTIO T, A.I.M.L.T., Principal TechnologistDate received: 17 April 1974.hereditary spherocytosis. The d;agnosis of hereditaryspherocytosis was established by the microscopic appear-ance of the blood smear, characteristic osmotic fragilityand a negative response to direct antiglobulin tests.Splenectomy had not been undertaken in this child.The serum bilirubin was elevated at 3,8% and thereticulocyte count ranged at about J%.In view of possibly excess fragility of the blood, a'mock' bypass was set up, using an infants' Temptroloxygenator, primed with 200 ml of Ringer's lactate andheparinised with 1 500 units of heparin; 125 ml of th~patient's blood was extracted into a donor bag containin,~I ml heparin and the mixture was circulated through acircuit similar to that to be used for the surgical operati8;J.The priming fluid was drawn from the helix reservoir ofthe bag by the 'arterial' roller pump, and pumped viaa 6-mm diameter tubing back into the 'venous' side ofthe oxygenator. Arterialisation of the priming fluid wasachieved by 3-litres flow of 97% oxygen in 3% carbon di-oxide mixture. Samples were taken every five nlinutes, andhaemoglobin, packed cell volume, platelets and electro-lytes, sodium, potassium and chloride were estimated.The osmotic fragility was estimated at the end of theprocedure. Cardiotomy was simulated by increasing thepump speed in combination with a Iow fluid level,allowing an air/blood mixture to be sucked through thepump during the last 30 minutes of the 'mock' procedure.The results (Table I) show that there was no importantblood destruction and that the plasma haemoglobin rosefrom 6 to 64 mg/l 00 ml. Osmotic fragility was notaltered at the conclusion of the procedure.It was decided to proceed with the planned operation.A 3-litre Temptrol oxygenator was primed with I litreRinger's lactate and 500 ml donor blood. The arterialpump was adjusted to deliver 2,5 litres/m'/min of bodysurface area-in this case 1,8 litres/min. The series oftests performed in the simulated bypass were repeatedand the abnormal red c~lls appeared to remain intact.Free plasma haemoglobin rose from 12 before to 45mg/l 00 ml after bypass, the level at 6 hours after bypassbeing 18 mg/lOO ml.The operation confirmed the cardiological findings, withthe exception of the patent foramen ovale, which wasnot found. The ventricular septal defect was closed byroutine patch closure and the infundibulum of the rightventricle opened by infundibulectomy. The postoperativecourse of the patient was uneventful and he was dischargedfrom hospital on the 20th postoperative day.After 6 months a sample of blood showed the haemo-globin to be 10,9 g/l 00 ml with a reticulocyte count of3°:., an excessive fragility of the red cells still being present.1536 S.A. MEDICAL JOURNAL 27 July 1974TABLE I. RESULTS (MCHC IN ALL SPECIMENS -+- 40%)Time onSpecimen bypass Hb PCV Na K Cl Plasma HbNo. (in min) (mgjl00 ml) (%) Platelets (mEqjL) (mEqjL) (m EqjL) Jmgjl00 ml)1 Prime 4,4 10 42000 137 4,5 111 62 5 3,9 10 73 10 3,7 10 84 15 3,7 10 131 4,7 116 115 30 3,5 10 23000 146 <;5 3,7 10 131 4,85 118 247 60 3,7 10 25000 129 4,85 118 348 75 4,1 10 24000 479 90 3,7 10 26000 135 5,1 118 64CONCLUSIONCardiopulmonary bypass appeared to be well toleratedin this patient with hereditary spherocytosis. The per-formance of a 'mock' bypass with an aliquot of theBoeke Ontvang :Environmental Medicine. Ed. by G. Melvyn Howe, M.Se.,Ph.D. and John A. Loraine, D.Sc., M.B., Ph.D., ER.C.P.E.Pp. xii + 271. Illustrated. £6,50. London: William Heine-mann Medical Books. 1974.The SmalI Intestine. Tutorials in Postgraduate MedicineVo!. 4. Ed. by B. Creamer, M.D., ER.C.P. Pp. ix + 310.Illustrated. £4.50. London: William Heinemann MedicalBooks. 1974. .Rorschach Responses in Old Age. Revised edition. By L. B.Ames, Ph.D., R. W. Metraux, M.A., J. L. Rodell, PhD.and R. N. Walker, Ph.D. Pp. xvi + 219. RI 1,00. Londonand Durban: Butterworths. 1974.Larrey: Surgeon to Napoleon's Imperial Guard. By R. G.Richardson. Pp. x + 266. Illustrated. £4,50. London: JohnMurray. 1974.patient's blood, gave a good indication of damage to thered cells.REFERENCESI. Dacie, J. V. (1963): The Haemolycic Anaemias. 2nd ed. London:J. & A. Churchill.2. Moyes, D. G., Rogers, M. A. and Coleman, A. J. (1971): Thorax,26, 131.Books ReceivedBone. farrow in Hypoxia and ·Rebound. By J. M. Yoffey,D.Sc., MD., F.R.C.S. (Eng.), Hon. Ll.D. (Manchester).Pp. x + 276. Illustrated. $12,50. Springfield, TIlinois:Charles C. Thomas. 1974.Heart Disease in Paediatrics. Postgraduate Paediatrics Series.By S. C. Jordan, M.D., f.R.C.P. and Olive Scot!, M.D.,ER.C.P. Pp. ix + 291. Illustrated. R10,50. London andDurban: Butterworths. 1974.Journal of Immunogenetics. Vo!. 1, No. 1. January 1974. Ed.by K. Bauer. Published bimontWy. Price of single issueR3,OO and subscription price £15,00. Oxford: BlackwellScientific Publications.Nonimasi\'e Cardiology. Ed. by A. M. Weissler, M.D. Pp. x +454. Illustrated. $19,00. New York: Grune & Stralton.1974.

Correction of Fallot's tetralogy in a patient suffering from hereditary spherocytosis

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Correction of Fallot's tetralogy in a patient suffering from hereditary spherocytosis

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