Sun Heather Y, Boe Justin, Rubesova Erika, Barth Richard A, Tacy Theresa A
Pediatric Cardiology, Lucile Packard Children's Hospital, Stanford University, Palo Alto, Calif, USA.
Congenit Heart Dis. 2014 Jul-Aug;9(4):E105-9. doi: 10.1111/chd.12091. Epub 2013 May 22.
In tetralogy of Fallot with absent pulmonary valve, pulmonary stenosis and regurgitation results in significant pulmonary artery dilatation. Branch pulmonary artery dilatation often compresses the tracheobronchial tree, causing fluid trapping in fetal life and air trapping and/or atelectasis after birth. Prenatal diagnosis predicts poor prognosis, which depends on the degree of respiratory insufficiency from airway compromise and lung parenchymal disease after birth. Fetal magnetic resonance imaging (MRI) has been useful in evaluating the effects of congenital lung lesions on lung development and indicating severity of pulmonary hypoplasia. This report is the first demonstrating the utility of fetal MRI in tetralogy of Fallot/absent pulmonary valve patients, which predicted postnatal pulmonary artery size and visualized airway compression and lung parenchymal lesions. The distribution of lobar fluid trapping on fetal MRI correlated with air trapping on postnatal computed tomography angiogram.
在法洛四联症合并肺动脉瓣缺如时，肺动脉狭窄和反流会导致显著的肺动脉扩张。肺分支动脉扩张常压迫气管支气管树，在胎儿期导致液体潴留，出生后导致气体潴留和/或肺不张。产前诊断提示预后不良，这取决于出生后气道受压和肺实质疾病导致的呼吸功能不全程度。胎儿磁共振成像（MRI）有助于评估先天性肺部病变对肺发育的影响，并提示肺发育不全的严重程度。本报告首次证明胎儿MRI在法洛四联症/肺动脉瓣缺如患者中的应用价值，其可预测出生后肺动脉大小，并显示气道受压和肺实质病变。胎儿MRI上叶性液体潴留的分布与出生后计算机断层血管造影上的气体潴留相关。
