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Persistent Pulmonary Hypertension in a Neonate with Vein of Galen Arteriovenous Malformation

新生兒因Galen氏動靜脈瘤造成之持續性肺高壓

並列摘要


Veom of Galen aneurismal malformation (VGAM) often leads to death in the neonatal period, mainly due to congestive heart failure. Chronic and excessive pulmonary flow in utero and postnatally is attributed to large VGAM and right ventricular overload. We report a male neonate with VGAM complicated by severe heart failure and persistent pulmonary hypertension. Endovascular coil embolization of VGAM was performed, resulting in improvement of congestive heart failure; however, severe persistent pulmonary hypertensionled to death 2 days after the embolizaton. Postmortem examination showed marked right and left ventricular hypertrophy and impressive muscular thickening of intra-alveolar arteriosle. Neonatal embolization seems to be beneficial only in babies without suprasystemic pulmonary hypertension. Therefore, early delivery and repair of VGAM should be considered before the onset of persistent pulmonary hypertension.

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