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Table of Contents
Year : 2013  |  Volume : 16  |  Issue : 3  |  Page : 201-204
Use of dopamine infusion improved oxygenation in a patient of Ebstein's anomaly with atrial septal defect

1 Department of Anesthesiology, Pushpanjali Crosslay Hospital, Ghaziabad, Uttar Pradesh, India
2 Department of Anesthesiology, Command Hospital CC, Lucknow, Uttar Pradesh, India

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Date of Submission29-Jan-2013
Date of Acceptance26-Mar-2013
Date of Web Publication29-Jun-2013


We present the successful perioperative management of an adult patient with Ebstein's anomaly for abdominal rectopexy surgery. The patient developed mild hypotension and a fall in peripheral oxygen saturation (SpO 2 ) after administration of a graded epidural block. Correction of the fall in the blood pressure; however, did not improve the SpO 2 . The patient was administered an intravenous infusion of dopamine to improve the cardiac output and this led to improvement in the SpO 2 .

Keywords: Atrial septal defect, Dopamine, Ebstein′s anamoly, Non-cardiac surgery, Oxygenation

How to cite this article:
Kapoor MC, Kumar P, Chakravarti P. Use of dopamine infusion improved oxygenation in a patient of Ebstein's anomaly with atrial septal defect. Ann Card Anaesth 2013;16:201-4

How to cite this URL:
Kapoor MC, Kumar P, Chakravarti P. Use of dopamine infusion improved oxygenation in a patient of Ebstein's anomaly with atrial septal defect. Ann Card Anaesth [serial online] 2013 [cited 2021 Nov 29];16:201-4. Available from:

   Introduction Top

Ebstein's anomaly (EA) is a congenital malformation characterized by dysplastic abnormalities of the tricuspid valve (TV) leaflets resulting in tricuspid regurgitation. [1] EA is responsible for 1% of congenital heart defects (1 in 110,000 of the general population). [2] Consent of the patient was taken for publication of this case report.

   Case Report Top

A 46 kg, 56-year-old woman, a diagnosed case of EA was scheduled for abdominal rectopexy for complete rectal prolapse. Patient gave a history of dyspnea on mild exertion and cyanosis from many years and was unable to perform household chores. The patient had seizure 8 months back after a high parietal infarct. She was managed conservatively and had good neurological recovery. Patient had no other significant history.

Examination revealed a thin-built woman with a heart rate of 88/min and blood pressure was 104/70 mmHg. Patient had central and peripheral cyanosis with severe digital clubbing. Jugular venous pressure was not raised. Examination of spine revealed dorsal kyphosis. A grade 2/4 pansystolic murmur was heard over the tricuspid area. The hemoglobin was 18.2 gm%. Electrocardiogram revealed an incomplete right bundle branch block. X-ray chest revealed cardiomegaly with a globular configuration, a narrow pedicle with a prominent right heart border due to an enlarged right atrium (RA), inconspicuous main pulmonary arteries, and slightly decreased perfusion of peripheral lung fields [Figure 1]. Trans-thoracic echocardiography revealed a massively enlarged RA with a normal sized left atrium [Figure 2] and a 2 cm ostium secundum atrial septal defect (ASD) with a right to left (R-L) shunt. The septal leaflet of the TV was sail like and displaced apically by about 18 mm suggesting EA. The right ventricle (RV) was hypoplastic. There was severe normotensive TR on color flow imaging. Mild mitral regurgitation (grade 2/4) was also noted. Left ventricular (LV) ejection fraction was 0.45. Her daily medication included atenolol 25 mg, enalapril 3.5 mg, aspirin 75 mg, folic acid 5 mg, atorvastatin 20 mg, and phenytoin sodium 200 mg.
Figure 1: X-ray chest (Postero-anterior view) showing cardiomegaly, inconspicuous main pulmonary arteries, and poorly perfused peripheral lung fields

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Figure 2: Trans-thoracic echocardiography image showing a massively enlarged right atrium with a normal sized left atrium and an ostium secundum atrial septal defect. The septal tricuspid valve leaflet was sail like and displaced apically by about 18 mm suggesting Ebstein's anomaly

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On arrival in the operation theater, the heart rate was 84/min, blood pressure 102/72 mmHg and peripheral oxygen saturation (SpO 2 ) 74% on room air, and 82% after oxygen supplementation @ 5 L/min. Electrocardiogram showed sinus rhythm. Antibiotic prophylaxis against bacterial endocarditis was administered. After intravenous (IV) pre-loading with 300 ml Ringer's lactate solution, a graded epidural block was administered with a total of 16 ml 0.5% bupivacaine and 50 μg fentanyl to achieve a T-8 level over 15 min, after placing an epidural catheter through the L2-3 interspace. Soon after the epidural block was established, there was a mild fall in the blood pressure to 96/66 mmHg, which was managed with 200 ml Ringer's lactate and a 50 μg phenylephrine bolus. The blood pressure improved to 120/90 mmHg, but the SpO 2 , which had simultaneously fallen to 68%, despite oxygen supplementation by facemask @ 5 L/min, did not rise. Dopamine infusion @ 5 μg/kg/min was started to increase the cardiac output. Five minutes after starting the infusion, the SpO 2 improved to 82-86% while the blood pressure was maintained at 116-126/72-84 mmHg. The infusion was continued intra-operatively at the same rate and there was no fall in SpO 2 thereafter. There was no other adverse event and the vital parameters were within normal limit intra-operatively.

Post-operatively the patient was observed in the intensive care unit. and bupivacaine 0.125% with fentanyl 2 μg/ml @4 ml/h was infused epidurally for post-operative analgesia. Dopamine infusion was continued at the same rate. SpO 2 remained 82-86% and there was no worsening of cyanosis. On the second post-operative day, dopamine infusion was discontinued, but supplemental oxygen was continued, epidural catheter was removed and the patient shifted to the high dependency ward. On the third post-operative day, supplemental oxygen was discontinued and on the fourth post-operative day the patient was shifted to the ward.

   Discussion Top

EA is associated with apical displacement of septal and posterior TV leaflets, leading to atrialization of the RV (thin walled and poorly contractile, along with an enlarged RA) with a variable degree of malformation and displacement of the anterior TV leaflet. [3] The displacement of TV leaflets obstructs RV filling, decreases the functional size of RV, [4] causes TR and leads to right heart failure. The TV is usually incompetent but may even be stenotic. The mitral valve may also have abnormal placement of leaflets. Most patients have an inter-atrial communication, which helps maintain systemic perfusion by R-L shunting of blood. [5] The degree of abnormality of RV function and size of the inter-atrial communication determine the severity of the condition. Disease severity also depends upon pulmonary hypertension, ventricular, and supraventricular tachycardias and association with Wolf-Parkinson-White syndrome which is present in ~20% of patients. [5],[6]

Cyanosis is common due to R-L shunt and/or severe heart failure. It may be transient in neonatal life with recurrence in adult life or may appear for the first time in adult life. Transient appearance or worsening of cyanosis may occur in adult life due to paroxysmal arrhythmias. Those who survive to adulthood may become symptomatic due to onset of arrhythmias, or by pregnancy. Fatigue and dyspnea may occur due to poor cardiac output secondary to RV failure and decreased LV ejection fraction. [7] Symptoms of right heart failure such as ankle edema and ascites may be present. Less common presenting symptoms may be brain abscess, bacterial endocarditis, paradoxical embolism, stroke, and transient ischemic attacks. [5] A few remain asymptomatic even in adulthood, and the anomaly is discovered incidentally in them. [8] Fatal ventricular arrhythmias, congestive heart failure and sudden collapse are the common causes of death. Our patient had cyanosis due to the presence of an ASD with R-L shunt across it and had suffered a parietal cerebral infarct 8 months earlier.

Hazards during anesthesia include development of cardiac tachyarrhythmias and hypoxemia as the result of increases in the magnitude of the R-L shunt. Several modes of anesthetic management in patients with EA have been described. [3],[9],[10],[11] Delivery of general anesthesia (GA) avoids hypotension and endotracheal intubation in GA enables control of oxygen delivery; however, induction times are prolonged because of pooling of drugs in the large RA. [12] Arrhythmia and tachycardia may occur after intubation/extubation of the trachea and a light plane of anesthesia may cause a sudden rise in the pulmonary artery pressure. Tachycardia impairs filling of the functionally small RV. Hypotension may increase the R-L shunt. Hypoxia causes pulmonary vasoconstriction and may increase the R-L shunt, thus, the patient may enter in a vicious cycle of pulmonary hypertension, increasing R-L shunt and right ventricular failure, finally ending in circulatory failure.

Successful use of epidural anesthesia has been reported in a 58-year-old patient of EA for ankle fracture reduction. [13] The authors avoided central venous pressure (CVP) monitoring or pulmonary artery catheter insertion and rather used transthoracic echocardiography for evaluation of cardiac function. Epidural anesthesia, using a two catheter technique has also been successfully employed for elective cesarean delivery in a patient of EA. [14] Value of CVP monitoring is questionable in presence of severe TR and paradoxical air embolism through the ASD may occur with its use. Intra-cardiac catheter insertion may provoke serious arrhythmias [3],[13] and thus the CVP catheter tip should be kept within the superior vena cava. Insertion of a pulmonary artery catheter is technically difficult in the presence of TR and though its use may help evaluate cardiac preload, its insertion may promote tachyarrhythmias and paradoxical embolization. We did not place a pulmonary artery catheter. Transthoracic or transesophageal echocardiography is useful to monitor cardiac function and output, but we did not have the same at our center. Invasive arterial blood pressure monitoring may have helped us measure the blood gas parameters more frequently, but we unfortunately did not do so.

Excessive administration of fluid may lead to congestive heart failure or may increase R-L shunt and hypoxemia. [13] Epidural or spinal anesthesia may be appropriate in non-severe patients, but excessive administration of fluid should be avoided. [13] Hypotension is usually avoided and although this complication can be treated with fluids and vasopressors, their use may lead to further instability in these patients. Ephedrine use to correct hypotension may precipitate supraventricular tachycardia. We limited the fluid pre-loading to 300 ml and used a supplement of 200 ml fluid and bolus of phenylephrine to correct the hypotension. However, although the blood pressure improved there was no change in the oxygenation of the patient. The shunt possibly increased on the administration of the epidural block due to a fall in the systemic vascular resistance and offloading of the left side of the heart. With a decrease in left sided filling, there was an increase in R-L shunt from the over filled RA, which was possibly further aggravated by fluid loading. We used dopamine infusion to improve cardiac output. Perhaps, enhanced inotropy improved the forward flow from the RA into the RV and indirectly reduced the R-L shunt. The increase in the pulmonary perfusion improved oxygenation.

To summarize, we successfully managed an adult patient with EA with ASD for abdominal rectopexy surgery. We used dopamine to enhance the myocardial contractility that thereby, perhaps, improved right sided cardiac output and oxygenation.

   References Top

1.Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly in the adult. Semin Thorac Cardiovasc Surg 2005;17:148-54.  Back to cited text no. 1
2.Anderson KR, Lie JT. Pathologic anatomy of Ebstein's anomaly of the heart revisited. Am J Cardiol 1978;41:739-45.  Back to cited text no. 2
3.Misa VS, Pan PH. Evidence-based case report for analgesic and anesthetic management of a parturient with Ebstein's Anomaly and Wolff-Parkinson-White syndrome. Int J Obstet Anesth 2007;16:77-81.  Back to cited text no. 3
4.da Silva JP, Baumgratz JF, da Fonseca L, Franchi SM, Lopes LM, Tavares GM, et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: Early and midterm results. J Thorac Cardiovasc Surg 2007;133:215-23.  Back to cited text no. 4
5.Maranets I, Hines RL. Congenital heart disease. In: Hines RL, Marschall KE, editors. Stoelting's Anesthesia and Coexisting Disease. 5 th ed. Philadelphia PN: Churchill Livingstone; 2008. p. 43-60.  Back to cited text no. 5
6.Groves ER, Groves JB. Epidural analgesia for labour in a patient with Ebstein's anomaly. Can J Anaesth 1995;42:77-9.  Back to cited text no. 6
7.Haddad F, Doyle R, Murphy DJ, Hunt SA. Right ventricular function in cardiovascular disease, Part II: Pathophysiology, clinical importance, and management of right ventricular failure. Circulation 2008;117:1717-31.  Back to cited text no. 7
8.Mair DD. Ebstein's anomaly: Natural history and management. J Am Coll Cardiol 1992;19:1047-8.  Back to cited text no. 8
9.Bengtsson IM, Magno R, Wickström I. Ebstein's anomaly: Anaesthetic problems. A case report. Br J Anaesth 1977;49:501-3.  Back to cited text no. 9
10.Halpern S, Gidwaney A, Gates B. Anaesthesia for caesarean section in a pre-eclamptic patient with Ebstein's anomaly. Can Anaesth Soc J 1985;32:244-7.  Back to cited text no. 10
11.Xin X, Tang S, Wang L, Zhao J, Li GL, Guo LL, et al. Anesthetic considerations of an emergency decompressive craniotomy complicated with Ebstein's anomaly and atrial septal defect. Chin Med J (Engl) 2011;124:615-7.  Back to cited text no. 11
12.Elsten JL, Kim YD, Hanowell ST, Macnamara TE. Prolonged induction with exaggerated chamber enlargement in Ebstein's anomaly. Anesth Analg 1981;60:909-10.  Back to cited text no. 12
13.Horishita T, Minami K, Koga K, Ogata J, Sata T. Anesthetic management using echocardiography for surgery of lower extremity in a patient with Ebstein's malformation. Anesth Analg 2005;101:608.  Back to cited text no. 13
14.Linter SP, Clarke K. Caesarean section under extradural analgesia in a patient with Ebstein's anomaly. Br J Anaesth 1984;56:203-5.  Back to cited text no. 14

Correspondence Address:
Mukul C Kapoor
6 Dayanand Vihar, Delhi - 110 092
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.114247

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  [Figure 1], [Figure 2]