Successful Pregnancy Outcome In A Patient With Ebstein’s Anomaly

Author Information

Changede P, Gupta S, Chavan N.
(* Assistant Professor, ** Registrar, Additional Professor. Department of Obstetrics and Gynecology, LTMMC & LTMGH, Mumba, India.)

Abstract

Ebstein's anomaly (EA) is an uncommon malformation of the tricuspid valve presenting with a wide array of clinicopathological features. The incidence of Ebstein's anomaly in the general population has been estimated as 1 in 210000 or less than 1 % of all congenital heart defects and it has an equal sex ratio. Ebstein’s anomaly is well tolerated in pregnancy in the absence of cardiomegaly, cyanosis and arrhythmias and in patients with mild cardiac dysfunction and a low Newyork heart assosciation (NYHA) functional class. We present one case of Ebstein’s anomaly who underwent cesarean section under general anesthesia with good fetomaternal outcome.

Introduction

Ebstein’s anomaly is an uncommon congenital heart disease. There is dysplastic abnormality of basal tricuspid valve leaflets. Tricuspid regurgitation occurs due to elongation and downward displacement of the septal and anterior cusps of the valve. Free attachments of tricuspid valve is also noted.^[1,2] There is atrialization of proximal part of the right ventricle. The right ventricle and an enlarged right atrium becomes thin walled and poorly contractile.^[3,4] Congestive heart failure is the most common cause of death. A considerable proportion of women with this disease reach childbearing age and pose a challenge to the treating obstetrician.

Case Report

29 years old female was admitted to our tertiary hospital with a diagnosis of Gravida 7 Para 2 Living 2 Abortion 4, with 38 weeks and 5 days with Ebstein’s anomaly for safe confinement. She had previous two full term cesarean delivery and four spontaneous abortions for which check curettage was done. No congenital disease was detected in her previous offspring’s. Intrapartum and postpartum period of both pregnancies was uneventful. On admission, patient was asymptomatic. There was no history of breathlessness, chest pain, palpitation, syncopal attacks or cyanotic spells. On verification of past records, she was diagnosed as a case of Ebstein’s anomaly with severe tricuspid regurgitation just before conception, when she had complaints of retrosternal chest pain, shortness of breath on exertion and generalized weakness. On examination, she was found to have normal vital parameters with no signs of cardiac failure. Examination of cardiovascular system revealed the presence of a pansystolic murmur with no clicks, best heard in the tricuspid area. All hematological and radiological investigations were within normal limits.

Echocardiography done at term was suggestive of congenital Ebstein’s anomaly with severe tricuspid regurgitation , mild pulmonary artery hypertension (38 mm), normal left ventricular systolic function and mild right ventricular systolic dysfunction . She had a small patent foramen ovale with right to left shunt. There was apical displacement of tricuspid leaflets by 23 mm. Anterior tricuspid leaflet (ATL) was freely mobile sail like with restricted movement of Anterior tricuspid leaflet tip. Septal tricuspid leaflet (STL) was tethered to the septum with tip prolapsing into the functional right atrium. Right ventricular pressure overload pattern was noted, with ejection fraction of 55 %. Similar echocardiography findings were present prior to conception. She was taken for an emergency cesarean section under general anesthesia in view of previous two cesarean sections with Ebstein’s anomaly with severe tricuspid regurgitation in labour. Antibiotic prophylaxis was given. A 2.360 kg female child was delivered with good Apgar score. Her postoperative stay in the hospital was uneventful. No congenital heart disease was detected in the baby during follow up. Patient was asymptomatic at follow up visit of 3 months. Husband was counselled and was willing for permanent method of sterilization.

Discussion

Ebstein’s anomaly is a rare congenital heart defect with a prevalence of 0.3–0.5 % characterized primarily by abnormalities of the tricuspid valve and right ventricle.^[5] In 1866, Wilhelm Ebstein has first described this condition but this report was overlooked and not quoted in the literature till the year 1900.^[6] A considerable proportion of these patients remain asymptomatic and reach childbearing age. The physiological changes of pregnancy may be aggravated in patients with Ebstein’s anomaly. Pregnant patients with Ebstein’s anomaly are usually acyanotic, but those with interatrial shunting can develop shunt reversal and cyanosis in pregnancy. Cyanotic patients have with increased rate of premature deliveries, low birth weight and thromboembolic complications.^[7] Connolly and Warnes reported the outcome of 111 pregnancies in 44 women with Ebstein’s anomaly. In this study 16 patients were cyanotic, 20 had an interatrial communication (atrial septal defect/ patent foramen ovale). 76% of pregnancies resulted in live birth, 89% patients delivered vaginally, 11% were delivered by cesarean section. Low mean birth weight was noted in infants born to cyanotic women as compared to acyanotic women. (2530 vs. 3140 g, p < 0.001).^[8] 42 pregnancies in 12 patients of Ebstein’s anomaly were described by Donelly et al.^[9] Pregnancy was well tolerated in most cases, resulting in 36 live births. Mild dyspnea in third trimester of pregnancy was common, 1 patient had right heart failure and 1 had arrhythmia.

The vaginal route of delivery is preferred in almost all cases. All factors leading to congestive heart failure, cyanosis and arrhythmias,^[10] should be avoided during labor. In this case, no arrhythmias, cyanosis or signs of cardiac failure were observed in present as well as previous pregnancies and the patient's NYHA functional remained unchanged.

Conclusion

A multispeciality team approach including an obstetrician, neonatologist, physician and a cardiologist is beneficial for good fetomaternal outcome in patients with Ebstein’s anomaly. Though pregnancy is well tolerated and fetal outcome is good in most of the patients, restricting family size and contraceptive advice should be given to all the patients.

References

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Citation

Changede P, Gupta S, Chavan N. Successful Pregnancy Outcome In A Patient With Ebstein’s Anomaly. JPGO 2015. Volume 2 Number 9. Available from: http://www.jpgo.org/2015/09/successful-pregnancy-outcome-in-patient.html