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Ann Thorac Surg 2005;80:1504-1506
© 2005 The Society of Thoracic Surgeons

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Case report

Transfusion-Free Complex Cardiac Surgery With Cardiopulmonary Bypass in a 3.55-Kg Jehovah's Witness Neonate

^a Department of Cardiothoracic and Vascular Surgery, Berlin, Germany
^b Department of Perfusion, Berlin, Germany
^c Department of Anesthesia, Berlin, Germany
^d Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany

Accepted for publication May 3, 2004.

^_* Address reprint requests to Dr Huebler, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, Berlin D-13353, Germany (Email: koster{at}dhzb.de).

	Abstract

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Complex cardiac surgery using cardiopulmonary bypass normally requires the transfusion of autologous blood components, particularly in neonates. This is predominately caused by the relatively high priming volume of the circuit with subsequent extreme hemodilution and the often extended and complex perfusions leading to progressive consumption of platelets and coagulation factors. We report on a strategy to minimize the cardiopulmonary bypass circuit and adjust the perfusion technique that resulted in transfusion-free correction of tetralogy of Fallot with an absent pulmonary valve and an aneurysm of the left pulmonary artery in a 3.55 kg Jehovah's Witness neonate boy.

	Introduction

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The impact of Jehovah's Witnesses' categorical rejection of the transfusion of autologous blood components for blood-saving strategies in cardiac surgery is generally appreciated. In adult patients, transfusion-free surgery can often be achieved with a negligible underlying risk by combining preoperative therapy with erythropoietin, intraoperative use of cell savers (if accepted), administration of aprotinin, and adjustments of surgical and perfusion strategies. However, extended surgery with cardiopulmonary bypass (CPB) for the correction of congenital heart defects in neonates remains a challenge.

Transfusion-free CPB in 4.2 kg Jehovah's Witness neonates for complex cardiac surgery has been reported in 2 patients; however in 1 of these patients the blood was given at the end of the CPB after an unsuccessful attempt at weaning from bypass during a Norwood I procedure [1, 2].

We report on a 3.55 kg Jehovah's Witness neonate boy in whom transfusion-free cardiac surgery with extended CPB was performed for correction of tetralogy of Fallot with an absent pulmonary valve and an aneurysm of the left pulmonary artery. Despite suboptimal baseline conditions with a preoperative hemoglobin value of 12.5 g/dL, minimization of the CPB circuit and adjustment of the perfusion technique contributed to maintenance of hemoglobin levels in the safe range of 6.5 to 7 g/dL during the entire period of CPB. After surgery, the hemoglobin level was 8.5 g/dL, and no blood components were administered in the entire postoperative period.

Echocardiography and bronchography were used to diagnose tetralogy of Fallot, an absent pulmonary valve, and an aneurysm of the left pulmonary artery with compression of the left main bronchus in a 3.55 kg Jehovah's Witness neonate boy. Due to acute decompensation, the neonate was mechanically ventilated immediately after birth. After admission to our hospital, therapy was initiated with recombinant erythropoietin (1,000 IU/sec every 3rd day) (NeoReconorm, Roche, UK) and iron. On day 6 the patient was weaned from the respirator, but he needed immediate reintubation. Bronchial obstruction was suspected as a main reason for respiratory failure. Viewing the potential complications of prolonged ventilation, surgical correction was scheduled for day 7 without performance of preoperative heart catheterization and without waiting for a further increase of the hemoglobin level by erythropoietin therapy.

An arterial line (right femoral artery) and central venous catheter (right internal jugular vein) were positioned on the evening before surgery. The hemoglobin value before surgery was 12.5 mg/dL. After a median sternotomy, aprotinin (Trasylol [Bayer, Frankfurt, Germany]) was administered with a bolus of 4 x 10⁵ kallikrein inhibiting units to the patient, 4 x 10⁵ kallikrein inhibiting units in the priming volume of the CPB, and a continuous infusion of 1 x 10⁵ kallikrein inhibiting units during extracorporeal circulation. Heparin-level based heparin management was performed according to the Hepcon HMS Plus system (Medtronic Inc, Minneapolis, MN) with a target level of 6 IU/mL.

All components of the CPB system were nonheparin-coated. The priming volume consisted of an electrolyte solution (Thomaejonin [DeltaSelect GmbH, Pfullingen, Germany]) and 5,000 IU of heparin. Very short tubing connections were accomplished by positioning the arterial roller pump head close to the oxygenator inlet and the cardiotomy and positioning the vent roller pump heads close to the cardiotomy reservoir inlet using a designated neonatal CPB console (S3 Mast-Mounted Pump [Stoeckert, Munich, Germany]) with two remote double-head roller pumps on masts. All blood-containing components were brought into close proximity to the operation table. The tubing diameters were downsized to 4.76 mm in the entire system with the exception of the roller pump segments, which consisted of 6.35 mm silicone rubber tubing. The bubble detector was placed between the oxygenator and the arterial filter for maximum shortening of the arterial line.

A hollow fiber membrane oxygenator (Safe Micro [Polystan, Vaerlose, Denmark]) with a priming volume of 52 mL and a 40-µm arterial line filter (Newborn [Dideco, Mirandola, Italy]) with a priming volume of 40 mL were used. The total priming volume of the system was 190 mL.

With the onset of CPB, systemic cooling to 32°C (bladder) was initiated, the aorta was cross clamped, and 20 mL of crystalloid cardioplegic Kirsch solution (Mg-Aspartat Procain [Köhler-Chemie, Alsbach, Germany]) and 20 mL hydroxyl ethyl starch (Fresenius AG [Bad, Homburg, Germany]) were infused. The ventricular septal defect was closed with a Dacron patch (Sulzer Vascutek, Hamburg, Germany), and the aneurysm of the left pulmonary artery was resected. The cross-clamping time of 50 minutes was followed by a reperfusion period of 53 minutes in which a Contegra-valved conduit (Medtronic Inc, Minneapolis, MN) was implanted in the right ventricular-pulmonary artery position. With the onset of systemic rewarming, venous drainage was augmented by vacuum-assisted venous drainage facilitated by a negative pressure of 20 mm Hg with a vacuum regulator to achieve a venous return of 3 L/min/m².

During CPB, blood gas analyses (0.3 mL volume) were performed at intervals of 15 minutes. The pH ranged between 7.35 and 7.56. Base excess ranged between –1.4 and +1.7, whereas lactate remained in the physiologic range between 1.4 and 1.7 mmol/mL. Measurements of the heparin level were reduced to one per hour. No hemofiltration was performed. Forced diuresis (10 mL/kg/h) was achieved with two boluses of 2.0 mg furosemide on initiation of CPB and on rewarming. The hemoglobin level before CPB was 10.6 g/dL and after initiation of CPB it was 6.7 g/dL. During CPB, the lowest hemoglobin value was 6.4 g/dL (after induction of cardioplegia) and the highest was 6.9 g/dL.

Separation from CPB proceeded with moderate inotropic support (adrenalin, 0.05 µg/kg/min; milrinone, 0.01 mg/kg/min). During separation from bypass the venous line and volume of the venous reservoir were used for pre-loading of the heart. This procedure was augmented by short-term vasoconstriction with repeated boluses of noradrenalin (0.2 mL of a 1:100 solution). After termination of CPB, the volume left in the oxygenator and heat exchanger was directly transfused through the arterial line. The remaining blood left in the arterial filter and arterial line was directly reinfused into the central venous catheter. Contrary to institutional standards in neonates, no modified ultrafiltration was carried out, so as to prevent further critical hemodilution by the relative large priming volume (approximately 80 mL, including 20 mL of the infusion warmer system we use as the standard during modified ultrafiltration) of the system.

The chest of the patient was closed primarily, and the patient was transferred to the intensive care unit. The hemoglobin level after arrival to the intensive care unit was 8.5 g/dL. The total postoperative blood loss was 25 mL. Therapy with recombinant erythropoietin and iron was continued postoperatively. The patient was weaned from the ventilator after 5 days, and he was transferred from the intensive care unit with a hemoglobin level of 10.0 g/dL after 7 days.

	Comment

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The present case demonstrates that, even in small neonates, correction of complex congenital heart defects requiring the use of CPB can be safely performed without the use of blood products. We believe that the interaction of different strategies is important for successful management of these cases:

1 Preoperative therapy with erythropoietin and iron should be performed for as long as possible, although in the present case it showed only minor effects.

2 If possible, catheterization of the heart close to surgery should be avoided if diagnosis by echocardiographic methods is possible.

3 Arterial and central venous lines should be placed the day before surgery to prevent hemorrhage from these sites during the phase of full heparinization.

4 In particular, the significant reduction of the priming volume to 190 mL (compared with 410 and 420 mL in previous reports) contributed to successful management [1, 2]. This was achieved by reducing the length of the CPB lines, reducing the diameter of the CPB lines, and using a small oxygenator and arterial filter.

5 Use of the volume of the venous reservoir and line for pre-loading of the heart together with temporary vasoconstriction reduced the need for additional hemodilution during the phase of separation from CPB.

6 Although modified ultrafiltration in neonate cardiacsurgery is an established procedure, filling of the lines and filter requires volume and subsequent further hemodilution [3]. Therefore we abstained from performance of this procedure.

7 Attention should also be paid to the preservation of the hemostatic system and prevention of postoperative hemorrhage. Even though only scant data are available on neonate CPB, the use of high-dose aprotinin and the level based heparin-protamine management appear to have contributed to preservation of the coagulation system and prevention of hemorrhage [4, 5].

8 In the entire perioperative period, blood sampling for laboratory analysis should be strictly reduced to a minimum.

We conclude that strict minimization of the CPB system, while still incorporating safety features, such as the arterial filter and the careful use of blood conservation techniques, opens up the perspective for transfusion-free complex cardiac surgery, even in neonates. However, more data are needed to evaluate whether implementation of additional features (eg, the use of heparin-coated circuits) can contribute to further improvement of the current protocol.

	Acknowledgments

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We would like to thank Anne M. Gale of the Deutsches Herzzentrum Berlin for her editorial assistance.

	References

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1. Tsang VT, Mullaly RJ, Ragg PG, Karl TR, Mee RBB. Bloodless open-heart surgery in infants and children Perfusion 1994;9:257-263.[Medline]
2. Forest RJ, Groom RC, Quinn R, Donnelly J, Clark C. Repair of hypoplastic left heart syndrome of a 4.25 kg Jehovah's witness Perfusion 2002;17:221-225.[Abstract/Free Full Text]
3. Tweddell JS, Hoffman GM, Mussatto KA, et al. Improved survival of patients undergoing palliation of hypoplastic left heart syndromelessons learned from 115 consecutive patients. Circulation 2002;106(Suppl I)I-82–9.
4. Mossinger H, Dietrich W, Braun SL, Jochum N, Meisner H, Richter H. High-dose aprotinin reduces activation of hemostasis, allogen blood requirement, and duration of postoperative ventilation in pediatric cardiac surgery Ann Thorac Surg 2003;75:430-437.[Abstract/Free Full Text]
5. Codispoti M, Ludlam CA, Simpson D, Mankad PS. Individualized heparin and protamine management in infants and children undergoing cardiac operations Ann Thorac Surg 2001;71:922-927.[Abstract/Free Full Text]

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