Department of Obstetrics and Prenatal Medicine, University of Bonn, Bonn, Germany.
Ultrasound Obstet Gynecol. 2010 Feb;35(2):243-5. doi: 10.1002/uog.7500.
The extremely rare syndrome including absent pulmonary valve associated with membranous tricuspid atresia or severe tricuspid stenosis, intact ventricular septum and patent ductus arteriosus has been reported sporadically in the postnatal literature. This cardiac defect is characterized by right ventricular dysplasia with asymmetrical ventricular septal hypertrophy, ventricular septum bulging into the left ventricle, small right ventricular cavity, membranous tricuspid atresia or severe stenosis with abnormal papillary muscles and leaflets and absence of the pulmonary valve leaflets. The only prenatal case reported so far was diagnosed at 33 weeks of gestation and terminated shortly thereafter; the natural history of prenatally diagnosed cases is therefore unknown. We report on the intrauterine course of a case diagnosed at 17 weeks of gestation that had a favorable postnatal outcome after palliation.
已在新生儿文献中零星报道过一种极其罕见的综合征，包括肺动脉瓣缺如合并膜性三尖瓣闭锁或重度三尖瓣狭窄、室间隔完整和动脉导管未闭。这种心脏缺陷的特点是右心室发育不良伴不对称性室间隔肥厚、室间隔膨入左心室、右心室腔小、膜性三尖瓣闭锁或重度狭窄伴异常乳头肌和瓣叶以及肺动脉瓣瓣叶缺如。迄今为止，唯一报道的产前病例是在妊娠 33 周时诊断的，随后不久即终止妊娠；因此，尚不清楚产前诊断病例的自然病程。我们报告了一例在妊娠 17 周时诊断的病例的宫内病程，该病例在姑息治疗后有良好的预后。
