Sharma Nalini, Lalnunnem Thiek Jion, Nandwani Megha, Santa Singh Ahanthem, Synrang Baingen Warjari
Department of Obstetrics and Gynaecology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, India.
Department of Cardiology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, India.
J Reprod Infertil. 2018 Apr-Jun;19(2):119-122.
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.
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 was delivered. Intra and postpartum period was uneventful.
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.
埃布斯坦畸形是一种罕见的复杂先天性心脏畸形，患病率为0.3%-0.5%。它在先天性心脏病病例中占1%。其特征是三尖瓣发育异常，累及三尖瓣叶的基部和游离附着部，间隔瓣叶和前瓣叶向下移位并延长，导致三尖瓣反流，右心室近端“心房化”，壁变薄且收缩力差，同时右心房扩大。患有这种畸形时，生育能力通常不受影响，即使是患有紫绀的女性也是如此。埃布斯坦畸形患者出生时的平均预期寿命为25至30岁。由于其罕见性以及孕期与埃布斯坦畸形相关的临床表现多样，本文报告了该病例。
一名24岁、孕2产1、妊娠39周6天的孕妇，已知患有埃布斯坦畸形，于2017年4月转诊至东北印度医学科学研究所进行进一步治疗，因为我们研究所拥有设备完善的心脏治疗设施。她的产前检查过程顺利。在妊娠40周3天时进行了择期剖宫产，分娩出一个体重2.5千克的健康婴儿。产时和产后过程均顺利。
由于孕期与埃布斯坦畸形相关的临床表现多样，此类女性在孕期应接受多学科方法的密切监测，以便诊断并发症并据此进行治疗。
