Nataloni Maura, Mocchegiani Roberto
Department of Medicine, ASL 6, Fabriano (AN), Italy.
Ital Heart J. 2004 Sep;5(9):707-10.
Ebstein's anomaly, an uncommon malformation of the tricuspid valve, has an extremely variable natural history, depending on a wide spectrum of pathological features. We here described a case of a patient with Ebstein's anomaly who gave birth to 2 healthy unaffected full-term infants after two successful pregnancies; a third pregnancy miscarried at the 11th week. The anomaly was diagnosed during childhood, was not associated with other cardiac anomalies, cyanosis or preexcitation and the echocardiographic degree of severity was low (grade 1). All of these factors are considered predictors of a good survival and prognosis. During the pregnancies, no arrhythmias, cyanosis or signs of cardiac failure were observed and the patient's NYHA functional class (1) remained unchanged. Our case is the only published case of two successful term pregnancies in Ebstein's anomaly, it confirms the importance of echocardiographic evaluation and that the probability of maternal and neonatal events may be predicted from the baseline characteristics of the mother. Pregnancy is well tolerated in the absence of important maternal cardiomegaly, cyanosis and arrhythmias and in those patients with mild cardiac dysfunction as evaluated at echocardiography and a low NYHA class, but it is associated with an increased risk of abortions and prematurity.
埃布斯坦畸形是一种罕见的三尖瓣畸形，其自然病史变化极大，取决于一系列广泛的病理特征。我们在此描述了一例埃布斯坦畸形患者，该患者在两次成功妊娠后产下了2名健康未受影响的足月婴儿；第三次妊娠在第11周时流产。该畸形在儿童期被诊断出，与其他心脏畸形、青紫或预激无关，超声心动图显示的严重程度较低（1级）。所有这些因素都被认为是良好生存和预后的预测指标。在妊娠期间，未观察到心律失常、青紫或心力衰竭迹象，患者的纽约心脏协会（NYHA）功能分级（1级）保持不变。我们的病例是已发表的唯一一例埃布斯坦畸形患者成功足月妊娠两次的病例，它证实了超声心动图评估的重要性，以及可以根据母亲的基线特征预测母婴事件的可能性。在没有重要的母体心脏扩大、青紫和心律失常的情况下，以及在超声心动图评估为轻度心脏功能障碍且NYHA分级较低的患者中，妊娠耐受性良好，但与流产和早产风险增加有关。
