• Users Online: 2129
  • Print this page
  • Email this page

Year : 2008  |  Volume : 52  |  Issue : 3  |  Page : 321-323 Table of Contents     

Anaesthetic Management of Caesarean Section in A Patient with Ebstein's Anomaly

1 Assistant Prof, Department of Obstetrics & Gynaecology, Medical College & Hospital, Kolkata, India
2 Medical Officer, Department of Obstetrics & Gynaecology, Medical College & Hospital, Kolkata, India
3 Clinical Tutor, Department of Obstetrics & Gynaecology, Medical College & Hospital, Kolkata, India
4 Prof. & HOD., Department of Anaesthesiology, Medical College & Hospital, Kolkata, India
5 Prof. & H.O.D., Department of Obstetrics & Gynaecology, Medical College & Hospital, Kolkata, India

Date of Acceptance20-Mar-2008
Date of Web Publication19-Mar-2010

Correspondence Address:
Suman Chatterjee
BC -103, Salt Lake, Kolkata 700064
Login to access the Email id

Source of Support: None, Conflict of Interest: None

Rights and PermissionsRights and Permissions

The rare congenital heart defect of Ebstein's anomaly is characterized by downward displacement and elonga­tion of the tricuspid valve with poorly contractile right ventricle, an enlarged right atrium along with tricuspid regurgi­tation. Supra ventricular and ventricular arrythmias are common. Here we describe the successful management of a pregnant patient undergoing an urgent LSCS under epidural anaesthesia. There was inadvertent dural puncture while performing the block, which was treated with sterile epidural boluses of saline. Perioperative management was uncomplicated and both the mother and newborn were discharged without any untoward problem.

Keywords: Ebstein′s anomaly, Caesarean section, Epidural, PDPH

How to cite this article:
Chatterjee S, Sengupta I, Mandal R, Sarkar R, Chakraborty P. Anaesthetic Management of Caesarean Section in A Patient with Ebstein's Anomaly. Indian J Anaesth 2008;52:321-3

How to cite this URL:
Chatterjee S, Sengupta I, Mandal R, Sarkar R, Chakraborty P. Anaesthetic Management of Caesarean Section in A Patient with Ebstein's Anomaly. Indian J Anaesth [serial online] 2008 [cited 2021 Jun 19];52:321-3. Available from: https://www.ijaweb.org/text.asp?2008/52/3/321/60642

   Introduction Top

Wilhelm Ebstein first described the clinical and anatomical features of an anomaly of the tricuspid valve in 1866 [1] , which occurs in 1 percent of congenital heart defects (1 in 110,000 of the general population). It is characterized by dysplastic abnormalities of both basal and free attachments of the tricuspid valve leaflets, with downward displacement and elongation of the septal and anterior cusp [2] , with resulting tricuspid regurgita­tion [3] . Thus the proximal part of the right ventricle is 'atrialised', becoming thin walled and poorly contrac­tile, along with an enlarged right atrium [4] . Disease se­verity depends upon the degree of valvular abnormal­ity, presence of a patent foramen ovale with intracar­diac shunting, pulmonary hypertension, ventricular and supraventricular tachycardias and particularly associa­tion with Wolf-Parkinson-White syndrome (up to 20 % of patients) [2],[5] . Congestive heart failure and sudden collapse are the most common causes of death.

   Case report Top

A 23 year old woman was referred to our hospi­tal at the onset of labour. Four years back she had an uncomplicated caesarean delivery under general ana­esthesia (GA). She complained of occasional palpita­tions, and dyspnoea on severe exertion. She had no history of recurrent chest infections, cyanosis or heart failure in the past. On examination she had pansystolic (increasing with inspiration) and mid-diastolic murmurs with right parasternal thrill, along with widely split sec­ond heart sound. The ECG showed sinus rhythm with right bundle branch block. Chest X-ray showed en­larged right atrium with cardiomegaly, while echoca­rdiogram showed the presence of moderately enlarged right atrium, tricuspid regurgitation with downwardly displaced tricuspid valves, confirming diagnosis of Ebstein's anomaly. There was no atrial septal defect or pulmonary hypertension. Her blood reports were within normal limits.

Premature rupture of membrane during transport necessitated an early repeat caesarean section. We kept ready all routine and emergency drugs and equipments. Intravenous midazolam 1 mg was given slowly while preloading the patient in left lateral tilt. Subsequently she was shifted into the OT, monitors (ECG, pulse oxi­meter and NIBP) were attached, and given antibiotics for bacterial endocarditis prophylaxis. A sitting epidu­ral was tried at L3-4 interspace using a 16 G Tuohy needle but CSF gushed out suddenly, forcing us to abandon epidural at that space. On second attempt the epidural was sited at a higher interspace (L2-3), and after giving a smaller 'test' dose of 2 ml lidocaine 2% with adrena­line (1:2,00,000) a 18 G epidural catheter was inserted 4 cm into the epidural space. 8 ml of 2% lidocaine with adrenaline was premixed with 100 mcg fentanyl and administered in fractionated 'graded' doses of 5 ml every 10 min; while assessing the pulse, BP, respiration and keeping constant verbal contact. After 20 minutes she could just move her toes with sensory block height till T 6 .

Thereafter left lateral tilt and oxygen were con­tinuously administered to the patient. Another 0.5 mg midazolam (as anxiolytic), along with ondansetron 4 mg slow iv was given. The skin incision to delivery time was 140 seconds (U-D interval 45 seconds), and a small for gestational age baby of 2kg delivered. The baby was crying poorly and after bag mask ventilation her Apgar score was acceptable. After birth 10 units of oxytocin was administered intramuscularly to mother, while 5 unit of oxytocin was given into the hydroxythethyl starch 450 / 6% 500ml (the only intraoperative fluid). The operation was completed in 35 minutes with no untoward incidence.

Immediate postoperatively, 30 ml normal saline solution was given through epidural catheter as pro­phylaxis for anticipated post-dural puncture headache (PDPH). She was advised black coffee, plenty of wa­ter along with ambulation whenever feasible. Epidural boluses of 0.125% bupivacaine 10 ml with fentanyl 20 mcg were given 2 hourly for first 12 hours postopera­tively. Another 2 doses of 30 ml of epidural saline were administered aseptically at 6 th and 12 th postoperative hour and epidural catheter removed. Postoperatively daily examination for a week was done for develop­ment of any PDPH, change in murmur character or de­velopment of new symptoms.

   Discussion Top

In presence of Ebstein's anomaly, there is impaired right ventricular size and function, further deteriorated by the increased blood volume and cardiac output during pregnancy [2] . Increased right atrial pressure and volume both worsen tricuspid regurgitation. Raised catechola­mine levels in pregnancy further predispose to arrythmias, particularly with maternal hypoxaemia and stress.

Arrythmias in Ebstein's anomaly with pregnancy have been treated with various antiarrythmics and in resistant cases cardioversion [2] , without affecting the fe­tus. Haemorrhage along with excessive fluid resuscita­tion is generally poorly tolerated. Women with Ebstein's anomaly tolerate pregnancy well with good fetal out­comes. The presences of arrythmias, cyanosis or pre­eclampsia are associated with increased maternal and fetal risk [5] . The combined effect of increased circulating catecholamine, maternal heamodynamic instability and maternal hypoxaemia in mothers with congenital heart disorders is associated with prematurity, low birth weight and poor neonatal outcomes [6] and, 2-14% of these ba­bies have congenital heart disorders. The female baby of our patient with Ebstein's anomaly was normal.

The basic principles for anaesthetizing a patient with cardiac disease are maintaining both afterload and preload along with a sinus rhythm [7] . The advantages of epidural anaesthesia are minimal intravascular volume shift, decreased catecholamine levels, control of ma­ternal hyperventilation and most importantly postop­erative analgesia [4],[5] . Intrathecal anaesthesia may com­plicate a right to left cardiac shunt due to sudden de­crease in sympathetic vascular resistance.

GA is associated with increased induction time with right to left shunts (increasing aspiration risk), in­creased catecholamine levels and an increased intra­thoracic pressure which further increase intracardiac blood shunting [5] . Large doses of synthetic oxytocin have marked vasodilating effects and administered cautiously, while methylergometrine and prostaglandines increase pulmonary vascular resistance and are generally avoided. Oxytocin 10 IU intramuscularly [5] is commonly followed in obstetric protocols. Insertion of a central line in patients of Ebstein's anomaly is technically diffi­cult, and increases likelihood of arrhythmias, paradoxical emboli, and bacterial endocarditis [8] .

An interesting situation was the accidental dural puncture with 16 G Tuohy needle. The reported inci­dence of post dural puncture headache is up to 85% in these cases. Apart from the routine measures (like hy­dration, ambulation, caffeine) [9] , instillation of epidural saline (in 4 to 6 hourly boluses or by continuously) is reported as beneficial in this scenario. Recently, epidu­ral instillation of colloids (dextran, hydroxyethyl starches) for the same purpose as epidural blood or saline, have been reported. Another technique is to insert an epidu­ral catheter through the dural rent and treat it as a con­tinuous spinal catheter and leaving it for 12 to 20 hours [10] .

In conclusion, women with Ebstein's anomaly may present with a multitude of problems and, should be considered as high risk and cared for in tertiary centres by a multidisciplinary team including obstetricians, car­diologists and obstetric anaesthetists in pregnancy and during delivery. Epidural anaesthesia in a fractionated or 'graded' manner provides a haemodynamically stable patient with adequate analgesia, and may be preferred to general anaesthesia in these patients.

   References Top

1.Mann RJ, Lie JT. The life story of Wilhelm Ebstein(1836­1912) and his almost overlooked description of a con­genital heart disease. Mayo Clin Proc 1979; 54 :197-203.  Back to cited text no. 1  [PUBMED]    
2.Donnelly JE, Brown JM, Radford DJ. Pregnancy out­come and Ebstein's anomaly. Br Heart J 1991; 66 : 368 - 71.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]  
3.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. 3      
4.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. 4  [PUBMED]  [FULLTEXT]  
5.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. 5  [PUBMED]    
6.Khairy P, Ouyang DW, Fernandes SM et al. Pregnancy outcomes in women with congenital heart disease. Cir­culation 2006; 113 : 517 -24.  Back to cited text no. 6      
7.Congenital heart disease. In :Stoelting RK, Dierdorf SF (editors). Anesthesia and Co-existing Disease (3rd edi­tion). Churchill Livingstone ; 1993 : pp. 37 - 56.  Back to cited text no. 7      
8.Halpern S, Gidwaney A, Gates B. Anaesthesia for caesar­ean section in a pre-eclamptic patient with Ebstein's anomaly. Can J Anaesth 1985; 32 : 244 - 7.  Back to cited text no. 8      
9.Kotur PF. Evidence based management of post dural puncture headache. Ind J Anaesth 2006; 50 : 307 - 8.  Back to cited text no. 9      
10.Kuczkowski KM. The spinal head ache in pregnant women. N J Obstet Gynaecol 2006 ;1:4-9.  Back to cited text no. 10      


    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  In this article
    Case report

 Article Access Statistics
    PDF Downloaded558    
    Comments [Add]    

Recommend this journal