Bhupali Ashok N, Patankar Kiran B, Prasad Sayi, Patil Jeetendra K, Tamhane Ajitey
Consultant Interventional Cardiologist and Head of Institute, Saraswati Hospital and Advanced Medical Care Center (Post Graduate Institute-DNB), Dasara Chowk, Kolhapur 416002, Maharashtra, India; Consultant Cardiologist, Apple Hospital and Research Institute, Diagnostic Center, Shahupuri, Kolhapur, Maharashtra, India.
Indian Heart J. 2013 Mar-Apr;65(2):191-3. doi: 10.1016/j.ihj.2012.10.010. Epub 2012 Oct 29.
Absent pulmonary valve syndrome (APVS) is a rare congenital anomaly, usually seen in association with a ventricular septal defect. It has been reported to occur in 3-6% of cases of tetralogy of Fallot (TOF). In this case report we discuss a case of absent pulmonary valve syndrome with tetralogy of Fallot that was detected in utero by fetal echocardiography at 27 weeks of gestation.
A 20-year-old pregnant woman at 27 weeks of gestation referred to our Institute. She has no consanguineous history. We diagnosed the case as tetralogy of Fallot with absent pulmonary valves in fetal echocardiographic study.
We conclude that when a paracardiac cystic, pulsatile lesion with dilated pulmonary arteries are seen in the fetus in utero then other features associated with the syndrome, such as TOF and the presence or absence of the ductus arteriosus should be looked for. In our case there was no ductus arteriosus.
肺动脉瓣缺如综合征（APVS）是一种罕见的先天性异常，通常与室间隔缺损相关。据报道，其在法洛四联症（TOF）病例中的发生率为3%至6%。在本病例报告中，我们讨论了一例在妊娠27周时通过胎儿超声心动图在子宫内检测到的法洛四联症合并肺动脉瓣缺如综合征病例。
一名妊娠27周的20岁孕妇转诊至我院。她无近亲结婚史。在胎儿超声心动图检查中，我们将该病例诊断为法洛四联症合并肺动脉瓣缺如。
我们得出结论，当在子宫内的胎儿中看到心旁囊性、搏动性病变且肺动脉扩张时，应寻找与该综合征相关的其他特征，如法洛四联症以及动脉导管的存在与否。在我们的病例中，不存在动脉导管。
