Horigome H, Sakakibara Y, Atsumi N, Miyamoto T, Sato H
Department of Pediatrics, Institute of Clinical Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba 305, Japan.
Pediatr Cardiol. 1997 Mar-Apr;18(2):136-8. doi: 10.1007/s002469900132.
Absent pulmonary valve with intact ventricular septum is a rare congenital anomaly. Most cases are not diagnosed until years after birth, although several cases have been reported in the literature in which cardiorespiratory distress developed during the neonatal period. We present a patient who showed improvement after fairly conservative therapy to alleviate the pulmonary vascular resistance, with ultimate palliative surgery. The aneurysmal dilatation of the pulmonary artery was for the most part confined to the proximal portion of the trunk, possibly because the ductus arteriosus was patent. The literature is reviewed.
室间隔完整的肺动脉瓣缺如是一种罕见的先天性异常。大多数病例直到出生后数年才被诊断出来，尽管文献中已有数例报道在新生儿期出现心肺窘迫。我们报告一例患者，在采取相当保守的治疗以减轻肺血管阻力后病情有所改善，并最终接受了姑息性手术。肺动脉的瘤样扩张大部分局限于主干近端，可能是因为动脉导管未闭。本文对相关文献进行了综述。
