Seale Anna N, Ho Siew Y, Shinebourne Elliot A, Carvalho Julene S
Brompton Fetal and Paediatric Cardiology, Royal Brompton Hospital, London, UK.
Cardiol Young. 2009 Apr;19(2):185-91. doi: 10.1017/S104795110900362X. Epub 2009 Feb 19.
To define the patterns of flow of blood to the lungs in fetuses with tetralogy of Fallot and pulmonary atresia.
In this condition, supply of blood to the lungs is provided via an arterial duct or systemic-to-pulmonary collateral arteries, or very rarely through other conduits such as coronary arterial fistulas or an aortopulmonary window. The intrapericardial pulmonary arteries vary in size, and may be absent. These variables influence the prognosis and management.
We carried out a retrospective review of cases from a tertiary service for fetal cardiology, identifying all cases of tetralogy of Fallot with pulmonary atresia diagnosed antenatally between January, 1997, and April, 2006. We established pre- and postnatal outcomes, and compared the prenatal diagnosis with postnatal or autopsy findings.
Of 6587 fetuses scanned during this period, 11 were diagnosed as having tetralogy of Fallot with pulmonary atresia and no other cardiac defect. In 5, arterial flow to the lungs was via an arterial duct, and in the other 6, the main identified source of flow was systemic-to-pulmonary collateral arteries. Of the latter 6 pregnancies, 4 were terminated, along with 3 of the 5 with ductal supply. The presence of systemic-to-pulmonary collateral arteries was confirmed at postmortem examination in 3 instances, and in the two delivered neonates, in neither of whom was an infusion of prostaglandin commenced.
The patterns of pulmonary flow can be identified prenatally in the setting of tetralogy with pulmonary atresia. Supply through systemic-to-pulmonary collateral arteries impacts on counselling, introducing uncertainty regarding postnatal surgical management.
明确法洛四联症合并肺动脉闭锁胎儿的肺血流模式。
在此病症中，肺的血液供应通过动脉导管或体肺侧支动脉，或极罕见地通过其他管道，如冠状动脉瘘或主肺动脉窗。心包内肺动脉大小各异，甚至可能缺如。这些变量会影响预后和治疗管理。
我们对一家三级胎儿心脏病服务机构的病例进行了回顾性研究，确定1997年1月至2006年4月期间产前诊断为法洛四联症合并肺动脉闭锁的所有病例。我们确定了产前和产后的结局，并将产前诊断与产后或尸检结果进行了比较。
在此期间扫描的6587例胎儿中，11例被诊断为法洛四联症合并肺动脉闭锁且无其他心脏缺陷。5例中，肺的动脉血流通过动脉导管，另外6例中，主要确定的血流来源是体肺侧支动脉。在后6例妊娠中，4例终止妊娠，5例通过导管供血的妊娠中有3例终止。3例在尸检时证实存在体肺侧支动脉，在2例分娩的新生儿中，均未开始输注前列腺素。
在法洛四联症合并肺动脉闭锁的情况下，产前可识别肺血流模式。通过体肺侧支动脉供血会影响咨询，给产后手术管理带来不确定性。
