Ann Thorac Surg 2007;84:2114-2116. doi:10.1016/j.athoracsur.2007.07.002
© 2007 The Society of Thoracic Surgeons
Case Reports
A Child With Sickle Cell Disease and Anomalous Right Coronary Artery
Glenn Egrie, MD,
Kristina Woodson, MD,
Nelson Alphonso, FRCS,
Tom R. Karl, MD*
Pediatric Heart Center, Children’s Hospital, University of California at San Francisco, San Francisco, California
Accepted for publication July 2, 2007.
* Address correspondence to Dr Karl, Division of Pediatric Cardiac Surgery, University of California at San Francisco, 513 Parnassus Ave, San Francisco, CA 94143-0117 (Email: karlt{at}surgery.ucsf.edu).
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Abstract
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Sickle cell disease in children requiring open heart surgical repair is uncommon and requires specific perioperative interventions to avoid complications from intravascular red cell sickling. We present a rare case of a 14-year-old girl with sickle cell disease and an anomalous right coronary artery from the pulmonary artery.
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Introduction
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The presence of sickle cell disease in patients undergoing open heart surgical repair presents several perioperative management challenges. The tendency of erythrocytes containing hemoglobin S (HbS) to aggregate, change their shape, and precipitate intravascular thrombosis is increased by hypoxia, acidosis, hypothermia, low flow states, and the concentration of HbS [1]. Successful completion of procedures requiring cardiopulmonary bypass (CPB) requires the minimization of these factors.
A limited number of recent cases involving children with sickle cell disease undergoing open heart procedures have been reported [2–4]. None of the patients in these reports had the diagnosis of a coronary artery anomaly. We report a case of the repair of an anomalous right coronary artery arising from the pulmonary artery in a 14-year-old girl with sickle cell disease and describe our perioperative management. We used a combination of strategies that included red blood cell exchange transfusion and platelet and plasmapheresis immediately before CPB, mild hypothermia (34°C), and moderately hypothermic (26°C) crystalloid cardioplegia.
The patient is an asymptomatic 14-year-old African American girl with homozygous sickle cell disease (HbSS), with an HbS fraction of 84%. She had a history of multiple hospitalizations for acute febrile venoocclusive episodes. She also had evidence of choroidal ischemia, possible early sickle cell nephropathy, and recurrent acute chest syndrome requiring multiple packed red cell transfusions.
The patient had undergone yearly transthoracic echocardiograms to evaluate pulmonary artery pressure. Her most recent echocardiogram showed a dilated left coronary artery, a mildly dilated left ventricle (LV), and normal LV systolic function. The right coronary artery was not visualized. Cardiac catheterization revealed an enlarged left coronary artery and no filling of the right coronary ostium. The distal right coronary artery filled through collateral vessels from the left anterior descending and circumflex arteries. The right coronary artery emptied retrogradely into the main pulmonary artery. The right coronary artery arose from the anterior rightward aspect of the main pulmonary artery (Fig 1).
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Fig 1. An injection of contrast into the left coronary artery outlines the entire left and right coronary system, with filling of the main pulmonary artery from the anomalous right coronary.
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For the surgical procedure, the CPB circuit and oxygenator (Terumo, Tokyo, Japan) were primed with 1 L of Plasma-Lyte A (Baxter, Deerfield, IL), 250 ml of 5% human serum albumin, and 200 mg of calcium gluconate. Systemic anticoagulation was achieved with the administration of 300-IU/kg heparin. Cannulation for CPB was performed in the ascending aorta, superior vena cava, and inferior vena cava. Before the commencement of CPB, the venous line was opened and 1.5 L of blood was sequestered in a reservoir. Hemophoresis, plateletpheresis, and plasmapheresis were performed on the sequestered blood. The collected platelets and plasma (1 L) were transfused to the patient after the cessation of CPB. The red blood cells were discarded.
As CPB was initiated, the patient received 3 U of packed red blood cells and 3 U of fresh frozen plasma to replace the blood removed. Hemofiltration was performed during CPB. The patient’s temperature was maintained at 34°C. The fractional concentration of oxygen used during CPB was 1.0. The serum pH was kept between 7.34 and 7.44. After initiating CPB, the branch pulmonary arteries were snared and the aortic clamp applied. Crystalloid cardioplegia Plegisol (Abbott Laboratories, North Chicago, IL) was administered in the aortic root. The temperature of the cardioplegia was maintained at 26°C for the initial dose, and then decreased to 2°C for subsequent administration. The main pulmonary artery was transected, and additional cardioplegia was administered directly into the right coronary artery.
The anomalous right coronary artery was excised with a large cuff of pulmonary artery wall. A trapdoor incision was made in the ascending aorta, and the coronary artery was translocated to the aorta. Air was removed from the heart, and the aortic clamp was removed. The defect in the pulmonary artery was repaired with a rectangular patch of autologous pericardium treated with glutaraldehyde. The patient was weaned from CPB, and modified ultrafiltration was performed. The patient’s hematocrit was 27% before the operation and 28% at completion. The HbS fraction was 17%.
An intraoperative transesophageal echocardiogram revealed antegrade flow in both coronary arteries and normal left ventricular wall motion. The postoperative course was uneventful. The patient required no further transfusions, and the serum hematocrit at the time of discharge was 30%. A persistent pericardial effusion developed that eventually responded to diuretic and corticosteroid medications. Her left coronary artery remains mildly dilated, as expected, but the dimensions and contractility of her left ventricle remain completely normal.
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Comment
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For patients with sickle cell disease who require open heart procedures, preoperative exchange transfusion has been recommended to prevent erythrocyte sickling during CPB and sequelae thereof [2, 3, 5, 6]. The optimal level of HbS to maintain during operations using CPB to minimize the complications of erythrocyte sickling is not known. An approach of assiduously maintaining HbS levels of less than 30% before elective noncardiac procedures has been shown to prevent perioperative complications [5].
The reduction of HbS can be achieved by performing exchange transfusions during the days or weeks before operation or partial exchange transfusion at the initiation of CPB [4, 7]. Preoperative exchange transfusion allows the level of 2,3-diphosphoglycerate to increase such that increasing oxygen-carrying capacity is achieved [8]. We chose intraoperative partial exchange transfusion, which eliminated the need for multiple interventions in the weeks before the operation and attained a reduction of HbS levels from 84% to 17%. In addition, with this strategy, plateletpheresis and plasmapheresis could be performed. The collected platelets and plasma were reinfused at the completion of CPB to maintain blood volume, replace clotting factors, and improve clinical hemostasis [4].
Hypothermia in patients with sickle cell disease undergoing CPB has been used successfully [2, 4, 6] but can cause vasoconstriction and red cell sludging, which can increase capillary transit time and cause sickling [8]. For this reason, hypothermia is often avoided [3]. We used mild hypothermia in this patient, which also assisted in avoiding acidosis, another precipitating factor for red cell sickling. The initial dose of crystalloid cardioplegia was administered at 26°C for the rationale described, and crystalloid was used to clear the coronary bed of erythrocytes and prevent intracoronary sickling and stasis. Although we used CPB, it is possible that in some cases of anomalous coronary artery from the right pulmonary artery a coronary translocation or other corrective procedure could be done without CPB, especially in older or larger patients such as ours.
In summary, we report the repair of an anomalous right coronary artery arising from the pulmonary artery in a child with sickle cell disease. The techniques of blood component sequestration, red blood cell exchange at the onset of CPB, mild hypothermia during CPB, and moderate hypothermic crystalloid cardioplegia were used to achieve a successful result.
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References
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- Hoffman R, Benz E, Shattil S, et al. Hematology: basic principles and practice4th ed.. New York, NY: Churchill-Livingston; 2004.
- Baxter MRN, Bevan JC, Esseltine DW. The management of two pediatric patients with sickle cell trait and sickle cell disease during cardiopulmonary bypass J Cardiothor Anesth 1989;3:477-480.
- Bomfim V, Ribeiro A, Gouvea F, Pereira J, Bjork V. Sickle cell disease and mitral valve replacement Scand J Thorac Cardiovasc Surg 1989;23:75-77.[Medline]
- Shulman G, McQuitty C, Vertrees RA, Conti VR. Acute normovolemic red cell exchange for cardiopulmonary bypass in sickle cell disease Ann Thorac Surg 1998;65:1444-1446.[Abstract/Free Full Text]
- Vichinsky EP, Haberkern CM, Neumayr L, et al. A comparison of conservative and aggressive transfusion regimens in the perioperative management of sickle cell diseaseThe preoperative transfusion in sickle cell disease study group. N Engl J Med 1995;333:206-213.[Abstract/Free Full Text]
- Frimpong-Boateng K, Amoah AG, Barwasser HM, Kallen C. Cardiopulmonary bypass in sickle cell anemia without exchange transfusion Eur J Cardiothorac Surg 1998;14:527-529.[Medline]
- Chun PK, Flannery EP, Bowen TE. Open-heart surgery in patients with hematologic disorders Am Heart J 1983;105:835-842.[Medline]
- Marchant WA, Walker I. Anesthetic management of the child with sickle cell disease Paediatr Anaesth 2003;13:473-489.[Medline]
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Abstract
Introduction
