Starnes V A, Pitlick P T, Bernstein D, Griffin M L, Choy M, Shumway N E
Department of Cardiovascular Surgery, Stanford University School of Medicine, Calif.
J Thorac Cardiovasc Surg. 1991 Jun;101(6):1082-7.
Ebstein's anomaly appearing during the neonatal period carries a high mortality rate. These infants exhibit cyanosis, acidosis, and congestive heart failure. The pathophysiologic characteristics consist of severe tricuspid regurgitation and functional pulmonary atresia. As a result of the inability of the right ventricle to generate forward flow through the pulmonary arteries, these infants remain dependent on ductal patency. Since May 1988, five newborn infants with severe Ebstein's anomaly have been admitted for treatment at our institution. At initial examination, they weighed 3.6 +/- 1.8 kg and had a mean oxygen tension of 29.6 +/- 2.3 mm Hg and a mean pH of 7.20 +/- 0.05. Chest roentgenography demonstrated a mean cardiothoracic ratio of 0.81 +/- 0.02. As determined by echocardiography, the right atria were massively enlarged, severe tricuspid regurgitation was present in all patients, and the pulmonary valves were not opening. All infants were dependent on prostaglandin E1 and attempts to wean them from this drug were unsuccessful. Palliative treatment consisted of tricuspid closure with autologous pericardium and an aortopulmonary shunt of 4 mm polytetrafluoroethylene tubing. There were no operative or late deaths. At discharge, mean oxygen tension was 42.2 +/- 0.85 mm Hg and mean systemic oxygen saturation was 83.2% +/- 1.94%. Infants have grown satisfactorily during the follow-up period. Three infants have since returned for further surgical intervention. One infant, at 11 months of age, underwent a Glenn anastomosis for progressive oxygen desaturation. Two infants have returned, at ages 23 and 22 months, for Fontan procedures, which represent their definitive operative management. We believe this new procedure offers excellent palliative treatment for Ebstein's anomaly in critically ill neonates. Feasibility of later definitive correction is demonstrated by the good results obtained with the Fontan procedure in two infants.
新生儿期出现的埃布斯坦畸形死亡率很高。这些婴儿表现为紫绀、酸中毒和充血性心力衰竭。其病理生理特征包括严重的三尖瓣反流和功能性肺动脉闭锁。由于右心室无法通过肺动脉产生前向血流，这些婴儿仍依赖动脉导管通畅。自1988年5月以来，我院收治了5例患有严重埃布斯坦畸形的新生儿。初检时，他们体重为3.6±1.8千克，平均氧分压为29.6±2.3毫米汞柱，平均pH值为7.20±0.05。胸部X线检查显示平均心胸比率为0.81±0.02。经超声心动图检查确定，右心房均显著扩大，所有患者均存在严重的三尖瓣反流，且肺动脉瓣未开放。所有婴儿均依赖前列腺素E1，试图停用该药物均未成功。姑息治疗包括用自体心包进行三尖瓣闭合术和使用4毫米聚四氟乙烯管进行体肺分流术。无手术死亡或晚期死亡病例。出院时，平均氧分压为42.2±0.85毫米汞柱，平均体循环氧饱和度为83.2%±1.94%。婴儿在随访期间生长情况良好。此后有3名婴儿返回接受进一步的手术干预。1名11个月大的婴儿因进行性氧饱和度下降接受了格林吻合术。2名分别为23个月和22个月大的婴儿返回接受了Fontan手术，这是他们的确定性手术治疗。我们认为这种新方法为危重新生儿的埃布斯坦畸形提供了出色的姑息治疗。两名婴儿接受Fontan手术取得的良好结果证明了后期进行确定性矫正的可行性。
