Rusdi Lusiani, Azizi Syahrir, Suwita Christopher, Karina Astrid, Nasution Sally A
Department of Internal Medicine, Faculty of Medicine Universitas Indonesia - Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
Acta Med Indones. 2016 Oct;48(4):325-326.
A 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein's anomaly three years ago, but preferred to be left untreated. The patient was not cyanotic and her vital signs were stable. Her ECG showed incomplete RBBB and prolonged PR-interval. Blood tests revealed mild anemia. Observation of two-dimensional echo with color flow Doppler study showed Ebstein's anomaly with PFO as additional defects, EF of 57%, LV and LA dilatation, RV atrialization, severe TR, and moderate PH with RVSP of 44.3 mmHg. The patient then underwent elective sectio caesaria at 30 weeks of gestational age; both the mother and her baby were alive and were in good conditions.
there was an increasing breathlessness in this patient so that there was an increasing need to take a decision for her pregnancy.Ebstein's anomaly is a complicated congenital anomaly. Medical treatment may be followed for many years in patients with mild forms of Ebstein's anomaly. Surgery should be considered if there is objective evidence of debasement such as significant enlargement of heart size, reduction of systolic function in echocardiography.
一名孕28周的27岁初产妇因呼吸急促加重入住我院。她三年前被诊断为埃布斯坦畸形，但选择不接受治疗。患者无发绀，生命体征稳定。她的心电图显示不完全性右束支传导阻滞和PR间期延长。血液检查显示轻度贫血。二维超声心动图彩色血流多普勒研究显示埃布斯坦畸形合并卵圆孔未闭等其他缺陷，射血分数57%，左心室和左心房扩大，右心室房化，严重三尖瓣反流，中度肺动脉高压，右心室收缩压44.3 mmHg。该患者随后在孕30周时接受了选择性剖宫产；母婴均存活且状况良好。
该患者呼吸急促加重，因此越来越需要对其妊娠做出决策。埃布斯坦畸形是一种复杂的先天性畸形。轻度埃布斯坦畸形患者可能需要多年的药物治疗。如果有诸如心脏显著增大、超声心动图显示收缩功能降低等病情恶化的客观证据，则应考虑手术治疗。
