Year : 2013 | Volume
: 3 | Issue : 2 | Page : 101--103
Anaesthetic management in a parturient with Ebstein's anamoly
KS Sushma, Safiya Shaikh, HR Ashwini
Department of Anaesthesiology, Karnataka Institute of Medical Sciences, Hubli, Dharwar, Karnataka, India
K S Sushma
Department of Anaesthesiology, Karnataka Institute of Medical Sciences, 206, Hubli, Dharwar - 580 021, Karnataka
Ebstein«SQ»s anamoly is a rare congenital heart defect comprising less than 1% of patients with congenital heart disease. Among the congenital heart lesions, Ebstein«SQ»s anamoly is one of the most diverse in presentation, severity and management. Even though pregnancy in women with Ebstein«SQ»s anamoly is usually well-tolerated, they are at increased risk of developing ventricular and supraventricular tachycardias, congestive cardiac failure, sudden collapse and even death. Here, we report a case of the pregnant woman with mild pre-eclampsia, known case of Ebstein«SQ»s anamoly posted for emergency caesarean section.
|How to cite this article:|
Sushma K S, Shaikh S, Ashwini H R. Anaesthetic management in a parturient with Ebstein's anamoly.J Obstet Anaesth Crit Care 2013;3:101-103
|How to cite this URL:|
Sushma K S, Shaikh S, Ashwini H R. Anaesthetic management in a parturient with Ebstein's anamoly. J Obstet Anaesth Crit Care [serial online] 2013 [cited 2020 Feb 24 ];3:101-103
Available from: http://www.joacc.com/text.asp?2013/3/2/101/123307
Ebstein's anamoly is a rare congenital heart defect comprising less than 1% of patients with congenital heart disease.  Among the congenital heart lesions, Ebstein's anamoly is one of the most diverse in presentation, severity and management.
The considerable cardiovascular and hormonal changes during pregnancy can challenge even healthiest of individuals. So pregnancy carries a greater risk to patients with tenuous cardiac physiology. 
Here, we report a case of the pregnant woman with mild pre-eclampsia, known case of Ebstein's anamoly posted for emergency caesarean section.
A 25-year-old primigravida with 38 weeks of gestation diagnosed with pregnancy induced hypertension with Ebstein's anamoly was referred to our hospital for emergency caesarean section. She gave a history of cardiac ailment since 10 years and was on digoxin and frusemide tablets since 2 years after an episode of hospitalization. She gave a history of intermittent chest pain since 2 years. She had exertional dyspnea New York Heart Association Class 2. She had no history of recurrent chest infections, cyanosis or palpitations.
On examination, pulse rate was 102 beats/min, regular, blood pressure was 140/100 mm of hg and jugular venous pressure was raised. Cardiovascular examination revealed apex beat in sixth intercostal space in midclavicular line, Grade 2 parasternal heave on palpation. Auscultatory findings were pansystolic murmur, increasing on inspiration in tricuspid area with muffled heart sounds. There was pansystolic murmur in the mitral area and ejection systolic murmur in aortic area.
Electrocardiogram showed sinus rhythm, large 'p' waves indicating right atrial enlargement. Chest X-ray showed cardiomegaly with prominent right heart border.
Recent echocardiogram showed atrialization of the right ventricle, dilated right atrium, Grade 3 tricuspid regurgitation, pulmonary artery systolic pressure of 50 mm of hg, moderate to severe pulmonary artery hypertension with tricuspid septal leaflet distance 5 mm with all echo findings confirming the diagnosis of Ebstein's anamoly.
Her blood investigation reports, including electrolytes were normal.
Fetal distress as an indication for caesarean section tilted our plan in favor of general anesthesia.
All routine and emergency drugs and equipments were kept ready in operation theatre. Antibiotic prophylaxis (injection ampicillin 2 g and injection gentamicin 80 mg intravenously [iv]) was given. Injection ondansetron 4 mg and injection ranitidine 50 mg iv was given. Proper precautions were taken to avoid air bubbles in the peripheral venous lines.
In operation theatre, monitors including electrocardiogram, pulse oximeter and non invasive blood pressure were attached. With the left lateral tilt patient was oxygenated with 100% oxygen. Injection glycopyrrolate 0.2 mg, injection fentanyl 100 mcg given iv. Injection Lignocaine 2% 80 mg given iv to attenuate the pressor response. Rapid sequence induction was performed with 250 mg thiopentone iv in slow titrated doses and 75 mg succinylcholine with 7.5 cuffed endotracheal tube. After confirmation with end tidal carbondioxide, tube secured and maintained with controlled ventilation with 50:50 N 2 O:O 2 and injection vecuronium 4 mg given for muscle relaxation. Soon after intubation, she developed ventricular ectopic beats about 6/min, which subsided within few min before any treatment was given. The incision to delivery interval was 4 min and baby weighing 2.5 kg was extracted. Cry was good and Apgar scores at 1 and 5 min were 8 and 10 respectively. Soon after extraction of baby, 10 units of injection oxytocin were given by slow iv infusion. Injection midazolam 1.5 mg iv was given.
Patient was hemodynamically stable throughout the procedure with mean arterial pressure between 80 to 100mm of hg and heart rate between 100 to 110 beats/min. Intraoperative end tidal carbon dioxide was ranging from 32 to 36 mm of hg and oxygen saturation 98-99%.
Surgery lasted for 45 min. At the end of surgery when patient regained respiratory attempts, neuromuscular blockade was reversed with injection neostigmine 2.5 mg and injection glycopyrrolate 0.5 mg. Extubation and recovery was smooth and uneventful. IV diclofenac infusions were used twice daily for post-operative analgesia.
During the 1 st 24 h post-operatively, patient was closely monitored in the intensive care unit. Post-operative period was uneventful. The patient and baby were discharged home on 7 th post-operative day with a referral for management by cardiology.
Ebstein's anamoly, a rare congenital cardiac defect, accounting for less than 1% of congenital heart diseases (1 in 100,000 general population), was described by Wilhelm Ebstein in 1866. 
Ebstein's anamoly 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 and sometimes stenosis. Therefore, varying amounts of the right ventricle effectively forms part of atrium (atrialization of the ventricle), with small functional right ventricle.  There can be interatrial communication through, which there may be right to the left shunting of blood. Cyanosis, pulmonary hypertension, cardiac dysrhythmias, pulmonary and systemic emboli, congestive cardiac failure and sudden cardiac collapse are anticipated complications. 
A total of 50% of patients with Ebstein's anamoly present in neonates and infants with cyanosis and congestive cardiac failure. Patients who survive to adulthood, symptoms may be precipitated by the onset of arrhythmias or by pregnancy.
Although literature suggests that pregnancy in women with Ebstein's anamoly is well-tolerated, it is associated with increased risk of prematurity, fetal loss and congenital heart disease in the offspring. The presence of arrhythmias, cyanosis and preeclampsia are associated with increased fetal and maternal risk.
Our patient even though acyanotic, had a history of cardiac ailment for 10 years and was on treatment with tablets digoxin and diuretics since 2 years after previous hospitalization, which was probably due to cardiac failure. She had cardiomegaly with moderate to severe pulmonary hypertension.
Basic goals in these patients are to maintain both pre-load and afterload and maintaining sinus rhythm.  General anesthesia has several advantages over regional anesthesia in these patients. Hypotension can be dangerous and the usual treatment with fluids and vasopressors can be hazardous as excessive fluid may lead to congestive heart failure or may increase the right to the left shunt and lead to hypoxemia. Ephedrine, commonly used vasopressor during caesarean section may precipitate supraventricular tachycardia in these patients.  General anesthesia with intubation enables control of oxygen delivery and seldom causes hypotension. 
Air bubbles were avoided in the peripheral venous lines, which could have caused paradoxical emboli. We also avoided central venous catheterization as it has risk of causing arrhythmias and infective endocarditis, in addition to being technically difficult. , Bedside echocardiography and transesophageal echocardiography are useful cardiac monitors during surgery in such cases,  which are not available in our institute.
Large doses of oxytocin have marked vasodilating effects and subsequent hypotension can be detrimental, so we used slow infusion. Methylergometrine and prostaglandins increase pulmonary vascular resistance and are generally avoided.
There is 20% incidence of supraventricular arrhythmias during general anesthesia induction. Effective treatment would be antiarrhythmics such as esmolol or amiodarone and in unresponsive cases, cardioversion is useful.
Even though general anesthesia is considered a safe alternative, there have been reports of patients being operated safely under epidural anesthesia for both obstetric and other incidental surgeries. ,
Pregnant women with Ebstein's anamoly are high risk patients and are better cared by multidisciplinary team. Knowledge of pathophysiology, hemodynamic derangements, thorough preparation and diligent care results in successful outcome of such rare congenital cardiac anomalies.
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