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  Table of Contents 
Year : 2023  |  Volume : 24  |  Issue : 1  |  Page : 79-80

Rhythm disturbances and their management in a case of Ebstein's anomaly

1 Department of Anaesthesiology and Critical Care, All India Institute of Medical Sciences, Bathinda, Punjab, India
2 Department of Anaesthesiology, Adesh Institute of Medical Sciences and Research, Bathinda, Punjab, India
3 Department of Anaesthesiology, Bharati Vidyapeeth, Pune, Maharashtra, India

Date of Submission31-Jul-2022
Date of Decision16-Sep-2022
Date of Acceptance17-Sep-2022
Date of Web Publication24-May-2023

Correspondence Address:
Dr. Navneh Samagh
Department of Anaesthesiology and Critical Care, All India Institute of Medical Sciences, Bathinda, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_82_22

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How to cite this article:
Samagh N, Panditrao MM, Panditrao MM, Singh N. Rhythm disturbances and their management in a case of Ebstein's anomaly. Indian Anaesth Forum 2023;24:79-80

How to cite this URL:
Samagh N, Panditrao MM, Panditrao MM, Singh N. Rhythm disturbances and their management in a case of Ebstein's anomaly. Indian Anaesth Forum [serial online] 2023 [cited 2023 Jun 1];24:79-80. Available from: http://www.theiaforum.org/text.asp?2023/24/1/79/377558


A 34-year-old male with perforation peritonitis was posted for exploratory laparotomy. There was no history of any comorbidities. Electrocardiography showed the presence of Q waves in leads II, III, and aVF suggestive of old c. Two-dimensional echocardiography revealed Ebstein's anomaly of tricuspid valve with mildly thickened valves, displacement of septal leaflets, and long anterior tricuspid leaflet. The right atrium and ventricle were dilated with moderate TR and moderate PAH. There was no regional wall motion abnormality, with an ejection fraction of 60%. Written informed consent was taken. Infective endocarditis prophylaxis was given. Standard ASA monitors were applied. Preoperative vitals were normal. Rapid sequence induction was done using injection propofol and injection succinylcholine. Injection fentanyl 2 mcg/kg was given. The patient was maintained on O2: Air (50:50) mixture, fentanyl, isoflurane, and vecuronium. Central venous catheter insertion was avoided as it might provoke life-threatening arrhythmias and could precipitate bacterial endocarditis.[1]

Intraoperatively, the patient developed supraventricular tachycardia (SVT) with a fall in oxygen saturation to 76%. Blood pressure was 96/60 mm of Hg. Chest examination was normal. About 100% O2 was started. Carotid massage, eyeball compression, and Valsalva were given with no effect on heart rate. Injection amiodarone followed by metoprolol was given in titrated doses, but it could not substantially bring down the pulse rate. Injection adenosine 6 mg bolus was then given which reverted the SVT to normal sinus rhythm and rate. There was an improvement in saturation and an Air: Oxygen (50:50) mixture was restarted. The patient was extubated after 2 h in the postanesthesia care unit and discharged after 7 days.

Ebstein's anomaly is a complex Congenital heart disease (CHD), in which there is a downward displacement of the tricuspid valve resulting in the displacement of septal and posterior leaflets of the tricuspid valve below the true annulus into the right ventricle. The anterior leaflet is larger and malformed. This in turn results in the formation of an atrialized right ventricle between the true annulus and the attachment of leaflets. This atrialized ventricle has fewer cells as compared to the normal ventricle resulting in its thinning, dilatation, and increased propensity for tricuspid regurgitation.[2] Patients may develop pulmonary hypertension, intracardiac shunting, dysrhythmias, Wolff- Parkinson- White (WPW) syndrome, infective endocarditis, congestive cardiac failure, and systemic and pulmonary embolization.[3]

Anesthetic management in these patients depends on clinical manifestations. Our patient had no history or clinical findings suggestive of cardiac disease. The presence of symptoms signifies altered hemodynamics and increases the likelihood of arrhythmias.[4] Hence, induction of anesthesia was done with fentanyl and incremental doses of propofol. Induction with ketamine is recommended in unstable patients with severe illness.[4] Tachycardia must be prevented as it is known to impair the filling of functionally small volume right ventricle.

The incidence of SVT in these patients has been found to be as high as 20%.[5] Our patient developed SVT intraoperatively. The tachycardia resulted in worsening of the right to left shunt which resulted in a fall in saturation. The resultant hypoxia causes pulmonary vasoconstriction which further worsens the right to left shunt. The vagal maneuvers such as carotid massage, eyeball compression, and Valsalva have been reported to abort SVT in 80% of patients.[6] If not effective then adenosine (6–12 mg intravenous [IV]) or verapamil (2.5–10 mg IV) are the agents of choice. Beta-blockers are second-line agents to terminate tachycardia.[6]

Thus, successful management of patients requires thorough knowledge of anatomical and physiological derangements and meticulous planning for the management of complications.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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. 1
Oliver WC, Lynch JJ. Congenital heart disease. In: Fleisher LA, editor. Anaesthesia and Uncommon Diseases. 5th ed. Philadelphia: Elsevier; 2006. p. 115-8.  Back to cited text no. 2
Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. Second of two parts. N Engl J Med 2000;342:334-42.  Back to cited text no. 3
Spitaels SE. Ebstein's anomaly of the tricuspid valve complexities and strategies. Cardiol Clin 2002;20:431-9.  Back to cited text no. 4
Oh JK, Holmes DR Jr., Hayes DL, Porter CB, Danielson GK. Cardiac arrhythmias in patients with surgical repair of Ebstein's anomaly. J Am Coll Cardiol 1985;6:1351-7.  Back to cited text no. 5
Page RL, Joglar JA, Caldwell MA, Calkins H, Conti JB, Deal BJ, et al. 2015 ACC/AHA/HRS guideline for the management of adult patients with supraventricular tachycardia: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice guidelines and the Heart Rhythm Society. Circulation 2016;133:e471-505.  Back to cited text no. 6


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