Eur J Cardiothorac Surg 2000;18:724-726
© 2000 Elsevier Science NL
Correction of cor triatriatum sinistrum in a Jehovah's Witness infant
Vladimir Alexi-Meskishvili,
Stanislav Ovroutski,
Ingo Dähnert,
Thomas Fischer
Department of Cardiovascular and Thoracic Surgery and Department of Congenital Heart Disease; German Heart Institute Berlin, Augustenburger Platz 1, 13353 Berlin, Germany
Received 17 April 2000;
received in revised form 11 August 2000;
accepted 5 September 2000.
Corresponding author. Tel.: +49-30-4593-2010; fax: +49-30-4593-2110
e-mail: alexi{at}dhzb.de
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Abstract
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A cor triatriatum sinistrum was successfully treated by operation in a 14-week-old infant of a Jehovah's Witness family. The child was pretreated with erythropoietin until a hemoglobin level of 14 g/dl was obtained. There was no cardiac catheterization before the operation. The operation was performed with cardiopulmonary bypass. No blood products were transfused and the hemoglobin level after performing modified ultrafiltration was 11.5 g/dl. The infant was extubated on the same day and discharged from our institution on the eighth day after surgery. Two years after surgery the child is in sinus rhythm and is developing well.
Key Words: Cor triatriatum sinistrum • Jehovah's Witness • Surgery
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1. Introduction
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Cor triatriatum sinistrum occurs in 0.1% of cardiac malformations. It is characterized by the presence of a fibromuscular membrane that subdivides the left atrium. Depending on the size of the remaining opening, the lesion might remain asymptomatic into adulthood or lead to pulmonary congestion, pulmonary hypertension and congestive heart failure during infancy. Surgical treatment in infants is reported to have an inherently high risk of mortality, especially when symptomatic or combined with associated anomalies [1,2]. Because of potential bleeding and the non-acceptance of a blood transfusion, open heart surgery in small infants of Jehovah's Witness families bears a high risk. The risk factors for adult Jehovah's Witnesses patients, such as repeat cardiac operation, severe ventricular dysfunction and a hemoglobin level of less than 80 g/l on the first postoperative day, have been identified [3]. Similar data for children are not available.
We report on the successful surgical treatment of cor triatriatum in a 14-week-old female infant of a Jehovah's Witness family.
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2. Case report
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The child was born spontaneously near term after a normal pregnancy with a birth weight of 3.3 kg. The cardiac murmur was heard and diagnosis established at the age of 5 weeks. The child received digoxin, diuretics, iron and preoperatively erythropoietin (2000 units subcutaneously twice weekly for 4 weeks) and was admitted for surgical treatment at the age of 14 weeks (weight 5.3 kg) with signs of moderate respiratory distress and right ventricular failure.
ECG showed normofrequent sinus rhythm and signs of right ventricular overload.
Echocardiography revealed an interatrial septal defect of 15 mm diameter with a left-to-right shunt and an extremely enlarged right atrium and right ventricle with accelerated outflow due to volume overload. The tricuspid valve (ring diameter 19 mm) was competent. The flow through the membrane into the left atrium to the mitral valve (ring diameter 12 mm) was turbulent with a mean gradient of 5 mmHg.
Cardiopulmonary bypass (CPB) consisted of a total priming volume of 250 ml crystalloid solution and aprotinin 20 000 E/kg with no human albumin added. Flow rate was 2.5–3.4 l/m2 per min (150–200 ml/kg per min) with normothermia (oxygen delivery 275–400 ml/m2 per min). No metabolic acidosis occurred (Table 1). The configuration of the circuit was: arterial line 3/16 inch, venous line 1/4 inch, pump segment 1/4x3/32 inch silicon, oxygenator Safe Micro (Polystan), S-III infant machine (Stöckert/Sorin-Biomedica), and arterial filter (Dideco Newborn) (40 ml). The placement of the equipment was on the left side of the head of the patient to minimize the length of the lines. Priming volume of the modified ultrafiltration (MUF) circuit was 75 ml. The MUF circuit was parallel to the CPB circuit with continuous circulation of blood through a small shunt between the arterial line and venous reservoir [4]. After weaning from CPB it was replaced with fluid from the CPB circuit to avoid further dilution. During MUF, the complete CPB circuit volume was given to the patient and replaced with crystalloid solution. The total amount of filtration was 500 ml. Hemoglobin levels are presented in Table 1.
Anesthesia was induced by midazolam 0.2 mg/kg, sufentanil 2 µg/kg and pancuronium 0.1 mg/kg and maintained with sevofluran 1–2% and an air/oxygen mixture via endotracheal tube. A radial catheter and a jugular central venous catheter were inserted.
Before initiation of CPB, the child received mannitol 0.5 g/kg, prednisolone 30 mg/kg, sufentanil 5 µg/kg, midazolam 0.2 mg/kg, and heparin 400 units/kg. Activated clotting time was measured using a Hepcon HMS device (Medtronic Hemotec).
During MUF, the child received desmopressin 0.4 µg/kg (Minirin). At the end of MUF the heparin activity was antagonized by 50% of a precalculated protamine dose.
During the operation 4.5 ml blood for all diagnostic measurements were used.
Surgery was performed through median sternotomy with standard cannulation, normothermic CPB and cold crystalloid cardioplegia. The atrial septal defect was situated between the pulmonary veins and the left atrial obstructing membrane. Right atriotomy and enlargement of the atrial septal defect provided excellent visualization of the membrane. After excision of the obstructing membrane, the atrial septal defect was closed with an autologous pericardial patch. Aortic cross-clamping time was 32 min and cardiopulmonary bypass time 50 min. Total operation time was 120 min.
In the postoperative course no inotropic agents were used. The child was extubated on day 1 and discharged on day 8 after surgery without postoperative complications. Treatment with erythropoietin and supplemental iron was continued for 6 weeks. Two years after surgery the child is doing well with normal cardiac function.
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3. Comments
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Cor triatriatum sinistrum is a rare cardiac anomaly. If the correct diagnosis is made early, the mortality is low, whereas the prognosis of symptomatic patients without surgery is extremely poor, even in cases with only mild symptoms during infancy [2]. Therefore, removal of the obstructing membrane is indicated as soon as the diagnosis is established.
In this case, surgery was postponed because the parents refused the use of blood and blood products, including human albumin, for religious reasons. Because the child was only moderately symptomatic, there was no need for emergency treatment. Preoperative stimulation of the hematopoiesis with erythropoietin and iron was performed to increase the hemoglobin level [5–8]. Another expected positive effect of the delay of surgery was an increase in weight, which improved the relationship between the total blood volume of the child and the cardiopulmonary bypass circuit. Unfortunately, this effect is not to be expected in children with marked or severe heart failure. However, in this case, the effects of the delay of surgery contributed to the successful transfusion-free open heart surgery.
Since the first report of successful cardiac surgery in adult Jehovahs Witness patients [9], many successful surgical interventions have been performed in this special group of patients, even for complicated cardiac anomalies, such as transposition of the great arteries, double outlet right ventricle, complete atrioventricular septal defect and tetralogy of Fallot by the use of extreme hemodilution (hematocrit level 15–23.3%) and hypothermic CPB [4]. The smallest child reported to have undergone an arterial switch operation was aged 3 months and had a body weight of 4.2 kg [4]. A low weight (less than 5 kg) is considered as a contraindication for open heart surgery in Jehovah’s Witnesses by some, and palliative procedures are advocated [10]. However, there is no palliative surgical option for cor triatriatum sinistrum. A catheter interventional approach (dilation or Rashkind like ‘membranostomy’) was considered but ruled out as incalculably risky and of uncertain outcome.
To avoid additional risk, the elimination of the arterial filter from the extracorporeal circulation circuit, as encouraged by some [10], was not performed. Systemic normothermic perfusion was used to prevent the negative effects of hypothermia on coagulation. The combined approach of preoperative treatment with erythropoietin and supplementation of iron, miniaturization of the extracorporeal circuit, normothermic perfusion with high flow rates, meticulous attention to hemostasis, modified ultrafiltration and the limited use of blood samples provided adequate oxygen delivery during CPB and was crucial for a successful outcome in our patient.
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Acknowledgments
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We would like to thank Mr Ekkehard Gutsch, our perfusionist, for providing CPB and Tonie Derwent for editorial assistance.
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References
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Abstract
1. Introduction
