Asian Cardiovasc Thorac Ann 1999;7:132-134
© 1999 Asia Publishing EXchange Pte Ltd
Tetralogy of Fallot and Hemophilia A: Successful Management of Intracardiac Repair
Emmanuel Rupert, MD,
Kanchi Muralidhar, MD,
Devi Prasad Shetty, MS
Department of Cardiac Anaesthesiology
Manipal Heart Foundation
Bangalore, India
|
|
For reprint information contact: Kanchi Muralidhar, MD Tel: 91 80 528 7742 Fax: 91 80 526 8912 email: mhfbg{at}giasbg01.vsnl.net.in Department of Cardiac Anaesthesiology, Manipal Heart Foundation, No. 98 Rustom Bagh, Airport Road, Bangalore 560017, India.
|
 |
Abstract
|
|---|
A 4-year-old boy with moderately severe factor VIII deficiency underwent successful intracardiac repair of tetralogy of Fallot under cardiopulmonary bypass with the aid of factor VIII concentrates and aprotinin.
|
Introduction
|
|---|
Patients with coagulation disorders undergoing open heart surgery present a challenge to the anesthetic and surgical team. Intracardiac repair of tetralogy of Fallot under cardiopulmonary bypass was carried out recently in our center with the aid of factor VIII concentrate infusion and aprotinin. This case demonstrated the usefulness of factor VIII concentrate and aprotinin in reducing the risk of performing open heart surgery on cyanotic children with hemophilia A.
|
Case Report
|
|---|
A 4-year-old male child weighing 13 kg with moderately severe hemophilia A was admitted to our center with a history of cyanosis and poor exercise tolerance. The diagnosis of hemophilia was made during an episode of excessive bleeding from the gums following trauma when he was one year old. He was transfused with one unit of cryoprecipitate at that time. He had no history of hepatitis or transfusion reaction and no known allergies. On examination, the patient was cyanosed, pulse and blood pressure were normal, and a systolic murmur was heard in the precordial area. Results of the following laboratory studies were within normal limits: complete blood count; platelet count; glucose; blood urea nitrogen; serum creatinine; serum electrolytes; liver function tests; urine analysis; prothrombin time; and thrombin time. Activated partial thromboplastin time was 70 seconds with a control of 28 seconds. Factor VIII activity was 1.7% and a screening test for inhibitors of factor VIII was negative on multiple occasions. Electrocardiography showed normal sinus rhythm, right axis deviation, and right ventricular hypertrophy. Chest radiography showed normal heart size, cardiac apex upturned, concave left upper border, and decreased pulmonary vascular markings. An echocardiogram and a color Doppler study revealed situs solitus, a large ventricular septal defect with a bidirectional shunt, severe infundibular stenosis (gradient 100 mm Hg), confluent pulmonary arteries, and a left aortic arch. In view of the satisfactory clotting factor recovery after factor VIII infusion and no evidence of inhibitors on multiple occasions, it was decided to proceed with surgery.
The patient was premedicated with oral atropine and diazepam. Intravenous access was established after application of lignocaine cream on the dorsum of the hand. A loading dose of 750 units of factor VIII concentrate (Emoclot; Octapharma, Vienna, Austria) was administered to raise the plasma factor VIII level to normal (100%). The priming dose was calculated as: units required = desired factor VIII increase (%) x body weight (kg)/2. A factor VIII assay 15 minutes later gave a level of 100% and his activated partial thromboplastin time was 41 seconds with a control value of 34 seconds. The patient was transferred to the operating room and anesthesia was induced with a combination of morphine, midazolam, and sodium pentothal. Orotracheal intubation was performed after administering pancuronium bromide. Following anesthetic induction with FiO2 of 50%, SpO2 was 52% and arterial blood gas analysis showed a PaO2 of 35 mm Hg with no acidosis. Anesthesia was maintained with isoflurane in oxygen and nitrogen, morphine, and intermittent positive-pressure ventilation. An infusion of dopamine 4 µg•kg–1•min–1 was started and aprotinin 20,000 units•kg–1 was infused in the prebypass period.
After sternotomy and pericardial reflection, the patient was given heparin 4.0 mg•kg–1 and the ascending aorta and venae cavae were cannulated in the usual fashion. Factor VIII 200 units (30 units per 80 mL of prime) and aprotinin 20,000 units• kg–1 were added to the nonhemic prime. Perfusion was initiated with a membrane oxygenator and the patient was cooled to 28°C. The kaolin-activated coagulation time was monitored and maintained above 600 seconds. Cold crystalloid cardioplegia (St. Thomas' Hospital solution) 15 mL•kg–1 was administered antegradely at 20 minutes intervals for myocardial protection. Intracardiac repair of the tetralogy of Fallot was performed. After termination of cardio-pulmonary bypass, heparin was neutralized with protamine sulfate and an additional 500 units of factor VIII (Emoclot) were administered. The aortic cross-clamp time was 67 minutes and total cardiopulmonary bypass time was 87 minutes.
The patient was extubated on the first postoperative day and the chest tubes were removed on the second postoperative day. Chest tube drainage in the first 24 hours was 130 mL and total drainage was 170 mL. On the third postoperative day, he had stridor due to a subglottic mucus plug, which responded to bronchoscopic aspiration. All of these procedures were conducted with factor VIII concentrate infusion. Factor VIII was assayed just prior to each dose of concentrate. Values were generally maintained above 70% and no unusual bleeding occurred. We infused factor VIII concentrate every 6 hours initially, gradually reducing to every 8 hours, and toward the end of therapy to every day. Total hospital stay was 16 days and total duration of factor VIII infusion was 10 days. Recovery was otherwise uneventful and the patient was discharged from the hospital on the 16th postoperative day.
|
Discussion
|
|---|
Hemorrhage after cardiopulmonary bypass is especially worrisome in the pediatric age group. Because of the smaller chest cavity, the total volume into which blood can collect is considerably less than in adults so the margin of error is small and tamponade can be an early catastrophic event. Factors that predisposed to bleeding in this patient were the preoperative coagulation disorder (hemophilia), the coagulation disorder seen in cyanotic heart disease, and the hemostatic alteration caused by cardiopulmonary bypass.1 Hemophilia A is an X-linked recessive inherited coagulation disorder caused by deficiency of factor VIII. It comprises approximately 80% of all cases of hemophilia. Hemophilia A is characterized by episodes of spontaneous bleeding that occur without recognized antecedent trauma. Operations on patients with hemophilia are performed in hospitals that meet the following requirements: a hematologist and diagnostic coagulation laboratory available on site; availability of adequate and appropriate replacement materials; and a surgical team familiar with the management of patients with coagulation disorders. The central concept for successful management of surgery is the documented preoperative correction of the clotting defect and its continued correction for an adequate period to permit wound healing. Failure to achieve these goals leads to postoperative hemorrhage, wound infections, and potential mortality. With the availability of clotting factor concentrates, almost any surgical procedure may now be performed safely in the hemophiliac without an inhibitor, regardless of the severity of disease. There have been some reported cases of patients with factor VIII or factor IX deficiency undergoing open heart surgery for both congenital and acquired cardiac disease with good results.2–6 Leggett and colleagues5 described a successful cardiac operation in a young boy with hemophilia, con-genital heart disease (double-chambered right ventricle with ventricular septal defect), severe factor VIII deficiency, and an acquired high titer of antibodies to factor VIII. A patient with moderately severe hemophilia B who underwent coronary artery bypass grafting with the aid of aprotinin and monoclonal antibody purified factor IX was reported by Palanzo and Sadr.6
Abnormalities of coagulation, hemostasis, qualitative and quantitative platelet defects, and fibrinolysis have been reported in cyanotic children and those with hematocrit values greater than 60%. Cardiopulmonary bypass alters the coagulation system through clotting factor and platelet hemodilution and platelet dysfunction due to activation and hypothermia. These qualitative and quantitative abnormalities are greatly exaggerated in infants.1 Several studies have demonstrated that aprotinin during cardiac surgery leads to an extensive reduction in intraoperative and postoperative bleeding tendency. This reduction is caused by attenuation of hemostatic activation during cardiopulmonary bypass.7,8 Dietrich and colleagues7 reported a dose-dependent attenuation of the deleterious effect of cardiopulmonary bypass on hemostatic activation as well as reduction of bleeding tendency in children undergoing cardiac surgery with aprotinin compared with a control group without aprotinin treatment. Therefore, they recommended the routine use of aprotinin in pediatric patients having cardiac operations. We chose to use aprotinin in our patient to combat the coagulation abnormalities found in cyanotic children. This case demonstrated the usefulness of factor VIII concentrate and aprotinin in overcoming the risks of performing open heart surgery on a cyanotic child with hemophilia A.
Presented at the 2nd Annual Conference of Indian Association of Cardiovascular and Thoracic Anaesthesiologists, Mumbai, India, November 13–15, 1998.
|
Acknowledgment
|
|---|
The authors gratefully acknowledge the services of the cardiology department of Manipal Heart Foundation, haematology and otorhinolaryngology departments of Manipal Hospital, and haematology department of St. John's Medical College, Bangalore, India for their valuable support offered during the hospital stay of the patient presented above.
|
References
|
|---|
-
Schulman SR, Kern FH, Greeley WJ. Coagulation disorders in congenital heart disease. In: Nichols DG, Cameron DE, Greeley WJ, Lappe DG, Ungerleider RM, Wetzel RC, editors. Critical heart disease in infants and children. St Louis: Mosby-Year Book, 1995:401–13.
-
Brockman SK, Aprill SN, Rabiner FS. Aortic valve replacement in hemophilia: report of a case. JAMA 1972;222:660–1.[Abstract/Free Full Text]
-
Lusher JM, Ravidranath Y, Arciniegas E, Green E. Open heart surgery in a hemophiliac patient. Hemotologic management. Am J Dis Child 1974;127:708–11.[Abstract/Free Full Text]
-
Tourbaf KD, Bettigole RE, Zizzi ZA, Subramanium S, Andresson MN. Coronary bypass in a patient with hemophilia B or Christmas disease. Case report. J Thorac Cardiovasc Surg 1979;77:562–9.[Abstract]
-
Leggett PL, Doyle D, Smith WB, Culpepper W III, Cooper S, Ochsner JL. Elective cardiac operation in a patient with severe hemophilia and acquired factor VIII antibodies. J Thorac Cardiovasc Surg 1984;87:556–60.[Abstract]
-
Palanzo A, Sadr FS. Coronary artery bypass grafting in a patient with hemophilia B. Perfusion 1995;10:265–70.[Abstract/Free Full Text]
-
Dietrich W, Mossinger H, Spannagal M, Jockum M, Wendt P, Barankay A, et al. Hemostatic activation during cardiopulmonary bypass with different aprotinin dosages in pediatric patients having cardiac operation. J Thorac Cardiovasc Surg 1993;105:712–20.[Abstract]
-
Marx G, Pokar H, Doering V, Tilsner V. Effects of aprotinin on hemostatic function during cardiac surgery. J Cardiothorac Vasc Anesth 1991;5:467–74.[Medline]
TOP
Abstract
Introduction
