Reporte de Caso

This anomaly was described in 1866 by Wilhelm Ebstein after the autopsy of a young Polish worker (1). Its incidence is of 1:210,000 births. The sex distribution is 1:1. Its cause is unknown (1).

There is an association with septal defects like ostiumsecundum or foramen ovalis in 70 to 80 % of the patients, with arrhythmias like the preexcitation syndrome of Wolff – Parkinson –White (WPW syndrome) in 20 to 30 % of the cases and with right bundle branch block in 51 % (4).

The tricuspid valves is usually regurgitant but sometimes it can become stenotic (4,5).

On the other hand, the right ventricular degree of dysfunction and the size of the septal defect determines its severity (4).

The symptoms in adult patients are often related to the age (4).

These symptoms can be associated with WPWS in 0.01 % to 0.03 % of the cases (6), and these can worsen during pregnancy (6). Atrial fibrillation and flutter are commonly seen in adult patients (7).

During pregnancy, the main hemodynamic changes which increase the risks of complications for the mother and fetus include: increases in blood volume (50 %), cardiac output (30 % – 50 %), stroke volume (18 ML), heart rate (from 15 to 20 bpm), increasein concentrations of catecholamines and decreases in systemic vascular resistance (30 %) (8). Mothers with cyanotic congenital heart disease have higher incidence of fetal losses, preterm births and low weight neonates; however in Ebstein’s anomaly it is considered that even without cyanosis, fetal morbidity is high with an increased incidence of congenital problems (8).

The diagnosis of Ebstein’s anomaly is performed with echocardiography (4). It is confirmed with the apical displacement of 8 mm /m² of the tricuspid valve (7) (figure 1).

The electrocardiogram shows low-voltage, steep P waves in leads V1 and DII, which reflect right atrial enlargement and commonly, various types of right bundle blocks. In 20 % of the patients there is lengthening of the PR interval and between 4 to 26 % there is a WPW syndrome (1, 7).

This was a 37-year-old female patient with a 37 week 2nd pregnancy. She had a previous medical history for a cardiac murmur without any follow-up, and a surgical history of a prior cesarean section 13 years earlier under regional anesthesia without any complications, and 2 dilation and curettages with a complication of a uterus perforation which required a laparotomy under general anesthesia.

At her 17th week of pregnancy she developed dyspnea with a NYHA class I/IV which progressed to a NYHA class II/IV. On physical examination she had a systolic murmur in the tricuspid window, grade III/IV with a loud S2. She had an EKG with a sinus rhythm and a WPW syndrome with a right axis. The transthoracic echocardiogram showed a low implantation of the tricuspid valve with severe regurgitation, Carpentier B, right ventricular hypoplasia, an enlarged right atrium and moderate pulmonary hypertension. She had follow-up by high-risk obstetrics and cardiology. When the fetus was 32 weeks old, lung maturation was done. She had an episode of significant vaginal bleed with clotsfro which she was hospitalized and scheduled for emergent C-section.

On admission her blood pressure was 129/75 mm Hg, heart rate of 92 bpm, respiratory rate of 14 per minute, pulse oximetry of 92 % - 95 % with oxygen supplement by nasal prongs of 3 L per minute and a peripheral IV gauge 18. A 500 ml bolus of crystalloid was administered and a left radial arterial line with a 20gauge catheter was inserted for continuous invasive blood pressure monitoring.

In the left lateral position an epidural anesthesia was administered with prior local anesthetic infiltration. The catheter was inserted through an 18 gauge Touhy needle between L 3 and L4. A test dose of 3 ml of 1 % lidocaine with epinephrine did not demonstrate any cardiac changes. Thereafter, 25 mg of bupivacaine, 100 mg of lidocaine and 25 μg of fentanyl were administered in a total volume of 15.2 ml.

The C-section was performed without any complications and a male newborn was extracted with an Apgar score of 7/10 (1). After the umbilical cord clamping, 10 units of intravenous oxytocin were administered over 10 min.

Intraoperatively, although the patient was in prone position with a wedge under her right lumbar area, she developed a transient hypotensive episode 10 min after the administration of the anesthetic (mean blood pressure of 52 mm Hg [MAP]) which responded to a 500 ml bolus of crystalloid and a 2 mg bolus of ethylephrine to maintain hemodynamic stability and a MAP above 60 mmHg throughout the procedure. The catheter was removed at the end of the surgery.

The sensitive block reached the T8 dermatome. The pain assessed with the visual analogue scale (VAS) was 0. In the post anesthesia care unit she had close monitoring. She had a favorable evolution without any complications, and at 48 hours, both the patient and the child were discharged with written indications for any alarming signs with obstetric and cardiologic follow-up.

Ebstein’s anomaly was 1st described in 1866 (9); and the death related to it is usually due to cardiac arrhythmias (10). In the C-section she could have had an increase in the right to left shunt, and increasing pulmonary vascular resistance with increased risk of mortality (11). The risk of paradoxical embolism increases specifically with the increases of intrathoracic pressure during labor (12).

The position during surgery is very important: most studies show that displacing the uterus to the left avoids aortocaval compression and its subsequent hemodynamic impacts like cardiac output decreases, hypotension and decreased uterine and fetal blood flow. In this case even though the patient was in prone position a wedge was inserted under the right posterior lumbar area to shift the uterus to the left.

This patient was given 10 units of intravenous dilute oxytocin during 10 min using an infusion pump without relevant hemodynamic effects, although Jonnson (13) showed in a double- blind randomized clinical study with a total of 103 patients for elective C-section, that administering 10 units of oxytocin in bolus had a higher risk of ST segment depression compared to 5 units with a statistically significant difference (13,14).

Both epidural as well as general anesthesia have been used for C-section with good results (12,14).

These patients can have prolonged induction times with intravenous anesthesia which increases the risk of pulmonary aspiration. For epidural anesthesia is recommended to administer a 500 ml bolus of crystalloid with graduated compression stockings to avoid hypotension (2). In this case, the fluid bolus was administered without graduated compression stockings.

Ephedrine does not produce uterine artery vasoconstriction but can stimulate beta receptors and thus exacerbate tachycardia (2).

Rathna et al considered that epidural anesthesia is a good choice in pregnant women who have little clinical changes (4).

Chopra et al. described that pregnancies with asymptomatic Ebstein’s anomaly could even have normal delivery whenever they do not meet general indications for C-section. Most of the patients with cardiac diseases tolerate vaginal delivery. The indications for C-section are the same as for the general population, although some entities benefit more with vaginal delivery than with a C-section, as is the case of hemodynamic instability during labor. Patients with cardiac diseases with a NYHA functional class of I/II without fetal problems can be allowed a spontaneous vaginal delivery, whereas those with NYHA functional class of III/IV should have an elective delivery (15,16).

Perioperative abnormalities (4, 5)

• The right to left shunt can lead to cyanosis and dyspnea with moderate exercise, some patients may be asymptomatic.

• Arrhythmias can be present in 25 % of the patients, some may even develop syncope.

• Even though they have a higher risk of venous thrombosis during pregnancy, anticoagulation is not routinely indicated as generally the risks exceed the benefits with the exception of patients who have a clear indication of anticoagulation.

• The indications for anti-coagulation during pregnancy are basically the same as in general population: these include a recent episode of deep venous thrombosis, pulmonary embolism, mechanical heart valves, and atrial fibrillation.

• The EKG can show steep P waves, lengthening of the PR interval, WPW syndrome and paroxysmal supraventricular tachycardia.

• The chest x-ray can show enlarged hearts, right ventricular enlargement and poor pulmonary perfusion.

• Paradoxical embolization can occur as well as bacterial endocarditis, brain abscesses and congestive heart failure.

Anesthetic problems (4)

• The anesthetic induction is prolonged because medications are accumulated in the enlarged right atrium (4,17).

• Central venous catheters can produce arrhythmias when inserted (4,5,18).

• The presence of air in peripheral venous accesses can lead to paradoxical air embolism.

• Tachycardia is poorly tolerated because the right ventricle is small, dysfunctional and has diastolic abnormalities.

• Hypotension may increase the right to left shunt.

• Hypoxemia can produce pulmonary vasoconstriction worsening the right to left shunt.

• There is a risk of bacterial endocarditis in the presence of central venous catheters.

Management (17)

• The severity of the abnormalities should be established both clinically and through echocardiographic assessment.

• Heart failure and arrhythmias require treatment.

• Regarding antibiotic prophylaxis for bacterial endocarditis the American Heart Association (AHA) has made these statements:

- The AHA does not recommend antibiotic prophylaxis based only on the increased risk, there are no published data that convincingly show that the administration of prophylactic antibiotics prevents the bacteremia associated during any invasive procedure.

- Prophylactic antibiotic administration with the only purpose of preventing endocarditis is not recommended for patients that have gastrointestinal surgery or urinary tract procedures (class III, B level of evidence) (19).

- It is reasonable to consider antibiotic prophylaxis against bacterial endocarditis before vaginal delivery, with rupture of the amniotic sac, in the case of patients at high risk like those who have mechanical cardiac valves, cyanotic congenital heart disease without correction, or those corrected with shunts (class IIA, C level of evidence) (20).

• The tip of the central venous catheter should remain in the superior vena cava.

• Central venous catheters can be used to assess preload but their use is technically difficult and complications can occur liketachyarrhythmias and paradoxical embolisms.

• Tachycardia and hypertension should be avoided shifting the uterus to the left as stated earlier also using crystalloid carefully with a maximum bolus of 500 ml, vasoconstrictors (specifically phenylephrine), and avoiding beta mimetic drugs and vasoconstrictors that stimulate beta 1 receptors.

• Medications that promote cardiovascular stability like etomidate, fentanyl, alfentanyl, and vecuronium should be used.

Prognosis

The factors that could indicate poor prognosis are early age of diagnosis, NYHA functional class III or IV, severe cyanosis, severe tricuspid regurgitation, a cardiothoracic index above 0.65, abnormal left ventricular function, arrhythmias and the presence of concomitant injuries (1, 21, 22).

In patients with Ebstein’s anomaly, arrhythmias do not represent absolute contraindication for pregnancy, however tachycardia is poorly tolerated in this congenital heart disease (8). Ephedrine, commonly used in obstetrics, can produce beta adrenergic stimulation and could induce supraventricular tachycardia (17).

It is important to consider, on the other hand, that excess fluid administration can worsen the right to left shunt and thus produce hypoxemia and congestive heart failure (17). Likewise, oxytocin should be carefully administered. Beta adrenergic medications are commonly used in obstetrics but should be carefully used as they produce beta 1 receptor stimulation which could produce tachyarrhythmias.

To Álvaro Rodríguez, at the Hospital Cardiovascular de Soacha.

1. Thambo JB, Jiménez M, Espil G, et al. Anomalie d’Ebstein. Archives des maladies du coeur et des vaisseaux. 2002;95:1104-11.

2. 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.

3. Anderson KR, Lie JT. Pathologic anatomy of Ebstein’s anomaly of the heart revisited. Am J Cardiol. 1978;41; 739-45.

4. Rathna, Tejesh CA, Manjunath AC, et al. Anaestheisa for incidental surgery in a patient with Ebstein’s anomaly. SAARC J Anaesth. 2008;1:85-7.

5. Stoelting RK, Dierdorf SF. Anesthesia and coexisting disease. 4a ed. Philadelphia: Churchill Livingstone; 2002.

6. Matthew ST, Federico GF, Singh BK. Ebstein’s anomaly presenting as Wolff-Parkinson white syndrome in a postpartum patient. Cardiol Rev. 2003;11: 208-10.

7. Gatzoulis MA, Swan L, Therrien J, et al. Adult congenital heart disease: a practical guide. Malden: BMJ Books; 2005.

8. Íñigo Riesgo CA, Torres Gómez LG, Hernández Higareda S, et al. Anomalía de Ebstein y embarazo. Ginecol Obstet Mex. 2008;76:461-7.

9. Ebstein W. Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Archiv für Anatomie, Physiologie und Wissenschaftliche Medicin. 1866;33:238-54.

10. Sugrue D, Blake S, MacDonald D. Pregnancy complicated by maternal heart disease at the National Maternity Hospital, Dublin, Ireland, 1969 to 1978. Am J Obstet Gynecol. 1981;139:1-6.

11. Linter SP, Clarke K. Caesarean section under extradural analgesia in a patient with Ebstein’s anomaly. Br J Anaesth. 1984;56:203-5.

12. Macfarlane AJ, Moise S, Smith D. Caesarean section using total intravenous anaesthesia in a patient with Ebstein’s anomaly complicated by supraventricular tachycardia. Int J Obstet Anesth. 2007;16:155-9.

13. Jonsson M, Hanson U, Lidell C, et al. ST depression at caesarean section and the relation to oxytocin dose: a randomized controlled trial. BJOG. 2010;117:76- 83.

14. Montes FR, Riaño D. Manejo anestésico de paciente obstétrica con anomalía de Ebstein. Rev Col Anest. 2004;32:285-7.

15. Cifuentes Borrero R. Cardiopatias en el embarazo. En: Cifuentes Borrero R. Ginecología y obstetricia basadas en las evidencias. Bogotá: Distribuna; 2002. pp. 257-65.

16. Chopra S, Suri V, Aggarwal N, et al. Ebstein’s anomaly in pregnancy: maternal and neonatal outcomes. J Obstet Gynaecol Res. 2010;36:278-83.

17. Grover VK, Mahajan RP, Sharma S, et al. Anaestheisa for incidental surgery in a patient with Ebstein’s anomaly. Indian J Anaesth. 1987;35:223-8.

18. Elsten JL, Kim YD, Hanowell ST, et al. Prolonged induction with exaggerated chamber enlargement in Ebstein’s anomaly. Anesth Analg. 1981;60:909-10.

19. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116:1736-54.

20. Warnes CA, Williams RG, Bashore TM, et al. ACC/ AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008;118:e714-833.

21. Yetman AT, Freedom RM, McCrindle BW. Outcome in cyanotic neonates with Ebstein’s anomaly. Am J Cardiol. 1998;81:749-54.

22. Celermajer DS, Bull C, Till JA, et al. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23:170-6.

2. 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.         [ Links ]

3. Anderson KR, Lie JT. Pathologic anatomy of Ebstein's anomaly of the heart revisited. Am J Cardiol. 1978;41; 739-45.         [ Links ]

4. Rathna, Tejesh CA, Manjunath AC, et al. Anaestheisa for incidental surgery in a patient with Ebstein's anomaly. SAARC J Anaesth. 2008;1:85-7.         [ Links ]

5. Stoelting RK, Dierdorf SF. Anesthesia and coexisting disease. 4a ed. Philadelphia: Churchill Livingstone; 2002.         [ Links ]

6. Matthew ST, Federico GF, Singh BK. Ebstein's anomaly presenting as Wolff-Parkinson white syndrome in a postpartum patient. Cardiol Rev. 2003;11: 208-10.         [ Links ]

7. Gatzoulis MA, Swan L, Therrien J, et al. Adult congenital heart disease: a practical guide. Malden: BMJ Books; 2005.         [ Links ]

8. Íñigo Riesgo CA, Torres Gómez LG, Hernández Higareda S, et al. Anomalía de Ebstein y embarazo. Ginecol Obstet Mex. 2008;76:461-7.         [ Links ]

9. Ebstein W. Über einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Archiv für Anatomie, Physiologie und Wissenschaftliche Medicin. 1866;33:238-54.         [ Links ]

10. Sugrue D, Blake S, MacDonald D. Pregnancy complicated by maternal heart disease at the National Maternity Hospital, Dublin, Ireland, 1969 to 1978. Am J Obstet Gynecol. 1981;139:1-6.         [ Links ]

11. Linter SP, Clarke K. Caesarean section under extradural analgesia in a patient with Ebstein's anomaly. Br J Anaesth. 1984;56:203-5.         [ Links ]

12. Macfarlane AJ, Moise S, Smith D. Caesarean section using total intravenous anaesthesia in a patient with Ebstein's anomaly complicated by supraventricular tachycardia. Int J Obstet Anesth. 2007;16:155-9.         [ Links ]

13. Jonsson M, Hanson U, Lidell C, et al. ST depression at caesarean section and the relation to oxytocin dose: a randomized controlled trial. BJOG. 2010;117:76- 83.         [ Links ]

14. Montes FR, Riaño D. Manejo anestésico de paciente obstétrica con anomalía de Ebstein. Rev Col Anest. 2004;32:285-7.         [ Links ]

15. Cifuentes Borrero R. Cardiopatias en el embarazo. En: Cifuentes Borrero R. Ginecología y obstetricia basadas en las evidencias. Bogotá: Distribuna; 2002. pp. 257-65.         [ Links ]

16. Chopra S, Suri V, Aggarwal N, et al. Ebstein's anomaly in pregnancy: maternal and neonatal outcomes. J Obstet Gynaecol Res. 2010;36:278-83.         [ Links ]

17. Grover VK, Mahajan RP, Sharma S, et al. Anaestheisa for incidental surgery in a patient with Ebstein's anomaly. Indian J Anaesth. 1987;35:223-8.         [ Links ]

18. Elsten JL, Kim YD, Hanowell ST, et al. Prolonged induction with exaggerated chamber enlargement in Ebstein's anomaly. Anesth Analg. 1981;60:909-10.         [ Links ]

19. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116:1736-54.         [ Links ]

20. Warnes CA, Williams RG, Bashore TM, et al. ACC/ AHA 2008 Guidelines for the Management of Adults With Congenital Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008;118:e714-833.         [ Links ]

21. Yetman AT, Freedom RM, McCrindle BW. Outcome in cyanotic neonates with Ebstein's anomaly. Am J Cardiol. 1998;81:749-54.         [ Links ]

22. Celermajer DS, Bull C, Till JA, et al. Ebstein's anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994;23:170-6.         [ Links ]