McElhinney Doff B, Salvin Joshua W, Colan Steven D, Thiagarajan Ravi, Crawford Elizabeth C, Marcus Edward N, del Nido Pedro J, Tworetzky Wayne
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, USA.
Am J Cardiol. 2005 Aug 15;96(4):582-6. doi: 10.1016/j.amjcard.2005.04.024.
From 1984 to 2004, 66 patients were diagnosed with Ebstein's malformation (n = 61) or congenital tricuspid valve (TV) dysplasia (n = 5) in utero or during the first month of life. Of these, 33 were diagnosed by fetal echocardiography at a median gestational age of 22 weeks, and 33 were diagnosed postnatally at a median age of 1 day (range 1 to 27). In 8 of the 33 prenatally diagnosed patients (24%), the pregnancies were terminated; in 9 (27%), the fetuses died in utero, and in 16 (49%), the fetuses survived to birth. Seven prenatally diagnosed patients survived beyond the neonatal period (21% of 33). Of the 49 neonates, 35 (71%) survived to hospital discharge and beyond 1 month of age. Independent predictors of death by multivariable logistic regression analysis included right atrial area index >1, the absence of anterograde flow across the pulmonary valve, and diagnosis before 1997. Although outcomes in fetuses and neonates with congenital anomalies of the TV have improved in more recent experience, survival in patients at the severe end of the spectrum remains poor. To improve outcomes in this group of high-risk patients, novel approaches to management may be indicated.
1984年至2004年期间，66例患者在子宫内或出生后第一个月被诊断为埃布斯坦畸形（n = 61）或先天性三尖瓣发育异常（n = 5）。其中，33例通过胎儿超声心动图在孕龄中位数为22周时确诊，33例在出生后确诊，年龄中位数为1天（范围1至27天）。在33例产前诊断的患者中，8例（24%）终止妊娠；9例（27%）胎儿死于子宫内，16例（49%）胎儿存活至出生。7例产前诊断的患者存活至新生儿期以后（占33例的21%）。在49例新生儿中，35例（71%）存活至出院且年龄超过1个月。多变量逻辑回归分析显示，死亡的独立预测因素包括右心房面积指数>1、肺动脉瓣无顺行血流以及1997年之前的诊断。尽管近年来先天性三尖瓣异常胎儿和新生儿的预后有所改善，但病情严重患者的生存率仍然很低。为改善这组高危患者的预后，可能需要新的治疗方法。
