Monacci Francesca, Bondi Tommaso, Canessa Clementina, Chiappa Enrico
Department of Experimental and Clinical Medicine, Division of Obstetrics and Gynecology, University of Pisa, Pisa, Italy.
Department of Neonatology, Nuovo Ospedale Santo Stefano, Prato, Italy.
J Obstet Gynaecol Res. 2019 Mar;45(3):714-718. doi: 10.1111/jog.13878. Epub 2019 Jan 17.
Absent pulmonary valve syndrome is a rare congenital heart disease characterized by absent or rudimentary pulmonary valve leaflets and hypoplastic pulmonary annulus. The most common variant, associated with tetralogy of Fallot, implies dilatation of pulmonary branches and usually absent ductus arteriosus. Rarely, absent pulmonary valve occurs with intact ventricular septum: pulmonary branches are normally sized or mildly dilated and ductus arteriosus is usually patent. The rarest type is associated with intact ventricular septum and tricuspid atresia. A close connection has been raised between pulmonary regurgitation and development of tricuspid valve. We describe a case of prenatal diagnosed absent pulmonary valve with intact ventricular septum and patent ductus arteriosus in which severe pulmonary regurgitation caused reversible functional atresia of tricuspid valve. Postnatally, satisfactory biventricular circulation was obtained with inotropic support for a few days while ductus arteriosus closed spontaneously. At a 3-year follow up the child is asymptomatic without therapy.
肺动脉瓣缺如综合征是一种罕见的先天性心脏病，其特征为肺动脉瓣叶缺如或发育不全以及肺动脉瓣环发育不良。最常见的变异型与法洛四联症相关，表现为肺动脉分支扩张且动脉导管通常缺如。罕见情况下，肺动脉瓣缺如合并室间隔完整：肺动脉分支大小正常或轻度扩张，动脉导管通常开放。最罕见的类型与室间隔完整和三尖瓣闭锁相关。肺动脉反流与三尖瓣发育之间的密切联系已被提出。我们描述了一例产前诊断为室间隔完整且动脉导管开放的肺动脉瓣缺如病例，其中严重的肺动脉反流导致三尖瓣可逆性功能闭锁。出生后，在给予几天的正性肌力支持以使动脉导管自然闭合的同时，获得了满意的双心室循环。在3年的随访中，该患儿未经治疗无症状。
