|Year : 2008 | Volume
| Issue : 3 | Page : 321-323
Anaesthetic Management of Caesarean Section in A Patient with Ebstein's Anomaly
Suman Chatterjee1, I Sengupta2, R Mandal3, R Sarkar4, PS Chakraborty5
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 Acceptance||20-Mar-2008|
|Date of Web Publication||19-Mar-2010|
BC -103, Salt Lake, Kolkata 700064
Source of Support: None, Conflict of Interest: None
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 2019 May 22];52:321-3. Available from: http://www.ijaweb.org/text.asp?2008/52/3/321/60642
| Introduction|| |
Wilhelm Ebstein first described the clinical and anatomical features of an anomaly of the tricuspid valve in 1866  , 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  , with resulting tricuspid regurgitation  . Thus the proximal part of the right ventricle is 'atrialised', becoming thin walled and poorly contractile, along with an enlarged right atrium  . 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) , . Congestive heart failure and sudden collapse are the most common causes of death.
| Case report|| |
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|| |
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  . 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  , 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  . 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  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  . The advantages of epidural anaesthesia are minimal intravascular volume shift, decreased catecholamine levels, control of maternal hyperventilation and most importantly postoperative analgesia , . 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  . 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  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  .
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)  , 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  .
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|| |
|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. [PUBMED] |
|2.||Donnelly JE, Brown JM, Radford DJ. Pregnancy outcome and Ebstein's anomaly. Br Heart J 1991; 66 : 368 - 71. [PUBMED] [FULLTEXT] |
|3.||Dearani JA, Danielson GK. Surgical management of Ebstein's anomaly in the adult. Semin Thorac Cardiovasc Surg 2005; 17:148-54 |
|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. [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. [PUBMED] |
|6.||Khairy P, Ouyang DW, Fernandes SM et al. Pregnancy outcomes in women with congenital heart disease. Circulation 2006; 113 : 517 -24. |
|7.||Congenital heart disease. In :Stoelting RK, Dierdorf SF (editors). Anesthesia and Co-existing Disease (3rd edition). Churchill Livingstone ; 1993 : pp. 37 - 56. |
|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. |
|9.||Kotur PF. Evidence based management of post dural puncture headache. Ind J Anaesth 2006; 50 : 307 - 8. |
|10.||Kuczkowski KM. The spinal head ache in pregnant women. N J Obstet Gynaecol 2006 ;1:4-9. |