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Ann Thorac Surg 2000;69:935-937
© 2000 The Society of Thoracic Surgeons


Case Reports

The Ross operation in a Jehovah’s Witness: a paradigm for heart surgery in children without transfusion

Kagami Miyaji, MDa, Robert L. Hannan, MDa, Jorge W. Ojito, CCPa, Jeffrey A. White, MSa, Redmond P. Burke, MDa

a Division of Cardiovascular Surgery, Miami Children’s Hospital, Miami, Florida, USA

Address to reprint requests to Dr Burke, Division of Cardiovascular Surgery, Miami Children’s Hospital, 3200 SW 60 Court, Suite 102, Miami, FL 33155-4069


    Abstract
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 Abstract
 Introduction
 Comment
 References
 
A 3-year-old 18 kg male child of the Jehovah’s Witness faith presented with severe aortic regurgitation. A successful Ross procedure was performed using a pulmonary autograft, without the use of blood or blood product transfusion. Blood conservation strategy included: (1) preoperative treatment with recombinant human erythropoietin; (2) intraoperative strategies, including technical modifications to the Ross procedure, and the prophylactic use of fibrin glue; (3) utilization of a heparin-bonded cardiopulmonary bypass circuit and assisted venous drainage; and 4) the use of prebypass phlebotomy, cell-saving device and autotransfusion. The patient was discharged home on postoperative day 7 with a hemoglobin level of 11.9.


    Introduction
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 Abstract
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Blood conservation techniques, such as blood scavenging with a cell-saving device, autotransfusion in a closed-loop system, or the preoperative use of recombinant human erythropoietin now allow cardiac operations in patients of the Jehovah’s Witness faith who are unwilling to undergo blood transfusions [13]. These children present an extraordinary dilemma for surgeons trained to strive for early complete repairs. We report a successful Ross operation [4] for severe aortic regurgitation, without the use of homologous blood or blood products, in a Jehovah’s Witness child.

A 3-year-old boy of the Jehovah’s Witness faith, weighing 18 kg, was referred to us with diagnoses of severe aortic regurgitation. He was diagnosed with severe congenital aortic stenosis and a bicuspid aortic valve with dysmorphic valve leaflets by echocardiography at birth. At 8 months of age he underwent transcatheter balloon dilatation of the aortic valve with improvement in his stenosis, and the creation of aortic insufficiency. He has been followed by echocardiography and noted to have increasing left ventricular dimensions and worsening aortic regurgitation. To prevent progression of left ventricular dysfunction, elective surgical repair was recommended. There was good match of the pulmonary valve annular size and the size of the aortic annulus (16 mm and 17 mm, respectively). After discussing the potential options with the parents, we elected to proceed with a Ross aortic root replacement to allow for the child’s growth and avoid the risk of a mechanical valve. The patient was in good hemodynamic condition at the time of operation. His parents were adamant that he should not be given blood or blood products. Recombinant human erythropoietin (2550 u/week) was given to the patient for 4 weeks preoperatively and resulted in an increase in his hemoglobin level from 12.1 to 13.2 g/dL before admission.

The patient then underwent repair using cardiopulmonary bypass with hypothermia at 20°C. At the beginning of bypass, 500 mL of undiluted heparinized whole blood, which had not been exposed to cardiopulmonary bypass, was reserved in a closed circuit connected to the venous drainage of the bypass circuit. This blood was reinfused after bypass was terminated. One million units of aprotinin were administered before bypass, and another one million was administered during bypass. The cardiopulmonary bypass circuit was completely heparin coated (Carmeda BioActive Surface; Minneapolis, MN), consisting of a centrifugal pump (Bio-Medicus, Eden Prarie, MN), and membrane oxygenator (Medtronic, Inc, Minneapolis, MN) with active venous drainage from the centrifugal pump. Heparin (300 IU/kg) was given before cannulation and activated clotting time was maintained above 480 seconds throughout the bypass. After cross-clamping of the aorta, the aorta was transected about 1 cm above the coronary ostia. The previous valvuloplasty had created a tear into the anterior leaflet, and the valve was not repairable. The left ventricular outflow tract was examined endoscopically, using a 4-mm, 30 degree face angled endoscope. No subvalvar obstruction was detected. The pulmonary valve cylinder was inspected and was normal, then excised completely, taking care to avoid the septal perforator of the left anterior descending coronary artery. Both retrograde and antegrade cardioplegia were infused after the excision of the autograft to check for bleeding from the autograft bed. The native aortic wall tissue was excised from the aortic root area and three orienting sutures were placed at the base of each commissure to align the pulmonary autograft in the aortic root. The pulmonary autograft was inverted into the aortic root and the suture line between aortic and pulmonary annulus was completed using a running 6-0 Prolene (Ethicon, Somerville, NJ) suture. Once the suture line was completed, a second circumferential buttress line between the remnant of aortic wall tissue and pulmonary autograft wall was placed using a running 6-0 Prolene suture, to further minimize anastomotic leak. The coronary ostia were excised and trimmed back to 6-mm buttons. The coronary anastomoses were constructed without torsion using running 6-0 Prolene suture. After the end-to-end aortic anastomosis was completed, the suture lines were coated with fibrin glue (Baxter, Inc, Irvine, CA). A 22-mm pulmonary homograft was then implanted to reconstruct the right ventricular outflow tract (Fig 1). The left side of the heart was de-aired, and the aortic cross-clamp was removed after completion of pulmonary homograft anastomoses. The patient was rewarmed and weaned from cardiopulmonary bypass with low dose of dopamine (5 µg · kg-1 · min-1) and milrinone (0.25 µg · kg-1 · min-1). Intraoperative transesophageal echocardiography showed no aortic regurgitation and good ventricular function. A cell-saver device (Medtronic, Inc) was used throughout the operation. After protamine was administered, the phlebotomized blood that had been stored in continuous contact with the patient, was reinfused. Two anterior mediastinal drainage tubes were placed and connected to a continuous reinfusion circuit. During dilutional cardiopulmonary bypass, the hemoglobin level ranged from 5.6 to 6.2 g/dL.



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Fig 1. A 22-mm pulmonary homograft was implanted to the right ventricular out flow tract. The Ross procedure was completed.

 
The patient’s postoperative course was uneventful and he was extubated on the first postoperative day. On the second postoperative day, chest tubes were removed, and the patient was transferred to the ward. He was discharged home on the seventh postoperative day. He had a viral urinary tract infection with delayed discharge. No homologous blood transfusion was required, and his hemoglobin level was 11.9 g/dL at discharge.


    Comment
 Top
 Abstract
 Introduction
 Comment
 References
 
Henling and colleagues [5] reviewed 110 cardiac operations for congenital heart disease in children of Jehovah’s Witnesses faith. However, there have been few clinical reports of complex open heart operations, such as the Ross operation [4]. In our patient, the preoperative hemoglobin level was maximized by treating with recombinant human erythropoietin for 4 weeks preoperatively. By reserving 500 mL of undiluted whole blood in a closed circuit, it was possible to manage cardiopulmonary bypass without using homologous blood or blood products (hemoglobin level: on bypass ranged from 5.6 to 6.2 g/dL). After cardiopulmonary bypass, the 500 mL of blood were reinfused. The extensive suture lines in the Ross procedure are potential sites of bleeding, particularly in patients with ventricular hypertrophy, who are often hypertensive postoperatively. In this case, we utilized a running suture, rather than interrupted sutures, to implant the autograft, and reinforced the autograft-annular suture line with a second butressing suture for additional hemostasis.

All suture lines were reinforced with fibrin glue while dry and under no distension. Heparin-coated bypass circuits reduce the blood activation, resulting in decreased platelet adhesion and improved platelet function [6]. The centrifugal pump causes less hemolysis than the roller pump [7]. We believe the combination of a heparin-coated circuit, the centrifugal pump, and venous assist, which provides less priming volume, is an optimum circuit for blood conservation.

In conclusion, a successful Ross procedure was performed in an 18 kg 3-year-old male child of the Jehovah’s Witness faith without the use of homologous blood or blood products. We used the following techniques: (1) preoperative recombinant human erythropoietin and intraoperative aprotinin; (2) surgical technique to prevent bleeding, including modifications to our usual technique for the Ross operation, and Fibrin glue for suture line; (3) a heparin-coated cardiopulmonary bypass using a centrifugal pump; and (4) a cell-saving suction system and autotransfusion within a closed-loop system.


    References
 Top
 Abstract
 Introduction
 Comment
 References
 

  1. Ott D.A., Cooley D.A. Cardiovascular surgery in Jehovah’s Witnesses. JAMA 1977;238:1256-1258.[Abstract/Free Full Text]
  2. Rosengart T.K., Helm R.E., DeBois W.J., Garcia N., Krieger K.H., Isom O.W. Open heart operations without transfusion using a multimodality blood conservation strategy in 50 Jehovah’s Witness patients. J Am Coll Surg 1997;184:618-629.[Medline]
  3. Miyaji K., Furuse A., Takeda M., Chikada M., Ono M., Kawauchi M. Successful conduit repair using aortic homograft in a child of Jehovah’s Witness. Ann Thorac Surg 1996;62:590-591.[Abstract/Free Full Text]
  4. Ross D.N. Replacement of aortic and mitral valves with a pulmonary autograft. Lancet 1967;2:956-958.[Medline]
  5. Henling C.E., Carmichael M.J., Keats A.S., Cooley D.A. Cardiac operation for congenital heart disease in children of Jehovah’s Witnesses. J Thorac Cardiovasc Surg 1985;89:914-920.[Abstract]
  6. Gorman R.C., Ziats N., Rao A.K., et al. Surface-bound heparin fails to reduce thrombin formation during clinical cardiopulmonary bypass. J Thorac Cardiovasc Surg 1996;11:1-12.
  7. Moen O., Fosse E., Dregelid E., et al. Centrifugal pump and heparin coating improves cardiopulmonary bypass biocompatibility. Ann Thorac Surg 1996;62:1134-1140.[Abstract/Free Full Text]
Accepted for publication July 10, 1999.




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This Article
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Robert L. Hannan
Redmond P. Burke
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