Persistent pulmonary hypertension of the newborn presenting as the primary manifestation of intracranial arteriovenous malformation of the Vein of Galen

Persistent pulmonary hypertension of the newborn presenting as the primary manifestation of intracranial arteriovenous malformation of the Vein of Galen
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DOI:
10.1055/s-2000-13456
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发表时间:
2000-01-01
影响因子:
2
通讯作者:
Lemke, RP
Lemke, RP
中科院分区:
医学4区
文献类型:
--
作者:
Hendson, L;Emery, DJ;Lemke, RP

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在新生儿期,Galen静脉的动静脉畸形仍然是诊断和治疗的难题。大约40%-50%的Galen静脉畸形出现在新生儿期,通常伴有充血性心力衰竭。这些新生儿是最严重的病例,也是最难管理的。我们报告一例患有Galen静脉畸形的新生儿,他表现为紫紫、心脏杂音和新生儿严重的持续性肺动脉高压。心力衰竭发生在患者病程较晚的阶段。超声心动图上的肺动脉高压程度用来确定Galen畸形静脉血管内栓塞的时间。栓塞术后,他的肺动脉高压急剧消退。我们推测,与Galen静脉畸形相关的肺动脉高压在这些新生儿的发病率和死亡率中被低估了,应该更积极地监测和治疗。
Arteriovenous malformations of the Vein of Galen continue to present diagnostic and therapeutic challenges in the neonatal period. Approximately 40-50% of all mal formations of the Vein of Galen present in the neonatal period, usual ly with congestive heart failure. These neonates represent the most severe cases and are also the most difficult to manage. We report a case of a neonate with a Vein of Galen Malformation who presented with cyanosis, a cardiac murmur, and severe persistent pulmonary hypertension of the newborn. Cardiac failure developed later in the patient's course. The degree of pulmonary hypertension on echocardiography was used to time endovascular embolization of the Vein of Galen Malformation. Following embolization, his pulmonary hypertension subsided dramatically. We speculate that pulmonary hypertension associated with Vein of Galen Malformations has been underestimated in the morbidity and demise of these neonates, and should be more aggressively monitored and treated.