Ebstein Anomaly with Pregnancy: A Rare Case.

Background: Ebstein anomaly is an uncommon, complex congenital malformation of the heart with prevalence of 0.3-0.5%. It occurs in 1% of congenital heart disease cases. It is characterized by dysplastic abnormalities of tricuspid valve which involves both basal and free attachments of the tricuspid valve leaflets, with downward displacement and elongation of the septal and anterior cusp which resulting in tricuspid regurgitation, the proximal part of the right ventricle is "atrialised", becoming thin walled and poorly contractile, along with an enlarged right atrium. With this anomaly, fertility is usually unaffected, even in women with cyanosis. The average life expectancy at birth of patients with Ebstein anomaly is 25-30 years. Due to its rarity and varied clinical presentations associated with Ebstein anomaly during pregnancy, this case was presented in this paper.

Case Presentation: A 24 year old G2A1 at 39 weeks 6 days gestation with a known case of Ebstein anomaly was referred to NEIGRIHMS in April 2017 for further management as our institute is having well equipped cardiac facilities. Her antepartum period was uneventful. Elective LSCS was done at 40 weeks 3 days and a healthy baby weighing 2.5 kg was delivered. Intra and postpartum period was uneventful.

Conclusion: Due to varied clinical presentations associated with Ebstein anomaly during pregnancy, such women should undergo close surveillance with multidisciplinary approach during the antenatal period to be diagnosed in terms of complications and hence be treated accordingly.