Asian Cardiovasc Thorac Ann 2007;15:526-527
© 2007 Asia Publishing EXchange Ltd
Aortic and Mitral Valve Replacement in a Patient with Hemophilia B
Roy Thankachen, MCh,
Biju George, DM1,
Vinayak Shukla, MCh,
Roy J Korula, MCh
Department of Thoracic and Cardiovascular Surgery
1 Department of Hematology, Christian Medical College and Hospital, Vellore, India
For reprint information contact: Roy J Korula, MCh, Tel: 91 416 222 2102 ext 2186/2106, Fax: 91 416 223 2035, Email: roykorula{at}hotmail.com, Department of Thoracic and Cardiovascular Surgery, Christian Medical College Hospital, Vellore 632 004, India.
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ABSTRACT
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A 25-year-old man with factor IX deficiency had an aortic and mitral valve replacement using a 2M Starr Edwards valve in the mitral position and a 22 Medtronic valve in the aortic position under cover of factor IX concentrate. The surgical procedure and the immediate postoperative period were uneventful except for a pericardial effusion which required a pericardiostomy. He was anticoagulated with heparin in the immediate postoperative period while the factor IX concentrate was being administered. Oral anticoagulation with acenocoumarol (Acitrom®) was started, maintaining the international normalized ratio between 1.5 and 2. He was doing well at follow-up 9 months later.
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INTRODUCTION
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Various surgical procedures are successfully performed on hemophiliacs; however, there is limited information on strategies to be used in hemophiliacs requiring cardiac surgery. Double valve replacement in patients with hemophilia has not been described before. The following is a case report of one such patient.
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CASE REPORT
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A 25-year-old man (weight, 46 kg) with no previous history of abnormal bleeding presented with an acute intracerebral bleed. On investigation he was diagnosed to have hemophilia B with a factor IX level of 14%. He also complained of chest pain and exertional dyspnea. Clinical evaluation and investigations revealed rheumatic mitral and aortic valvular disease requiring replacement of both valves. He presented for surgery 3 months later after complete neurological recovery.
Two hours prior to surgery 4000 IU of factor IX concentrate bolus (Alphanine® SD, alpha®; Therapeutic Corporation, Los Angeles, CA, USA) was administered through a central line, aiming for a factor IX level of 80%. The blood sample collected half an hour later showed it to be 88% and he was taken up for surgery.
After induction, another bolus of 1500 IU of factor IX was given. Systemic heparinization was done and the valves were replaced using a 2M Starr Edwards valve (Model 6120, Edwards Lifesciences, LLC, Irvine, CA, USA) in the mitral position and a 22 Medtronic valve (Medtronic, Inc., Minneapolis, MN, USA) in the aortic position. Our choice of the Starr Edwards valve in the mitral position was based on our excellent long-term follow-up of the use of this valve in this position.1 Reversal of heparin was done in the standard manner. Three units of fresh frozen plasma and one unit of rejuvenated red cells were infused after coming off bypass. The perioperative blood loss was around 200 mL.
Immediately after surgery, a bolus of 3000 IU of factor IX was given and a continuous infusion of the same was started at a rate of 3 IU·kg–1·hr–1 aiming for a factor IX level of 50%. Factor IX assay on the 1st postoperative day was 82%. The total drainage from the chest tubes was 670 mL in the first 24 hours. An infusion of heparin was started at a rate of 10 U·kg–1·hr–1 after the immediate postoperative drainage stopped. On the 3rd postoperative day, 2500 IU of Fragmin (Pharmacia & UpJohn AB, Stockholm, Sweden), injected subcutaneously twice daily, was substituted for heparin.
The factor IX level on that day was 54%. On the fourth day, the patient developed cardiac tamponade with oliguria and hypotension. A sub-xiphoid pericardiostomy was done and 300 mL of blood was drained under the cover of an additional dose of 1500 IU of factor IX. Although there was minimal perioperative bleeding, the patient developed non-oliguric acute renal failure. The creatinine levels rose to 7.7 mg·dL–1 but he required only conservative management without dialysis. The infusion of factor IX was slowly reduced to obtain a level of 30% by the 7th day and 20% by the 11th day. It was stopped on the 12th postoperative day. A total of 32,000 units of the concentrate were used. Fragmin was stopped on the 11th day and the patient was discharged on the 17th day without any anticoagulation. Low-dose oral anticoagulation with acenocoumarol maintaining an international normalized ratio between 1.5 and 2.0 was started once the renal failure resolved completely. The patient was doing well at follow-up 9 months later.
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DISCUSSION
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Various cardiac surgical procedures have been documented in patients with hemophilia A or B,2–4 but none have described management of a patient with hemophilia B undergoing double valve replacement. In managing this patient, we considered the following points: (1) adequate factor IX levels had to be maintained perioperatively; (2) judicious use of anticoagulation was required to prevent thrombosis of the valves; (3) overzealous anticoagulation had to be avoided considering the past history of an intracerebral bleed.
An immediate preoperative factor IX level of 80% was considered adequate for surgery. The patient was subsequently put on a continuous infusion protocol,5–6 aiming at a level of 50% for the first 3 days, 40% for the next 3 days, 30% for the next 4 days and 20% or less thereafter. Heparin and Fragmin were used to prevent valve thrombosis in the immediate postoperative period when factor IX levels were high. He was subsequently started on low-dose oral acenocoumarol.
Valve replacement surgery is safe in patients with hemophilia with appropriate management. The surgical and anesthetic protocols are unchanged. The main problem here is to decide on the amount of anticoagulation since it will be directly influenced by the factor IX levels. This is the first published case report of a patient with hemophilia B undergoing double valve replacement in the literature.
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ACKNOWLEDGMENTS
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We thank Mr. Elumalai Ravikumar for help in the preparation of the manuscript.
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REFERENCES
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ABSTRACT
INTRODUCTION
