Cook A C, Fagg N L, Ho S Y, Groves A M, Sharland G K, Anderson R H, Allan L D
Department of Fetal Cardiology, Guy's Hospital, London.
Br Heart J. 1995 Oct;74(4):443-8. doi: 10.1136/hrt.74.4.443.
To investigate the echocardiographic, morphological, and histological appearances of aorto-left ventricular tunnel observed in four fetal hearts and compare the findings with those reported in older patients with the malformation.
Previous studies have concentrated on clinical features of the malformation from birth to adult life and have speculated on either its embryological formation or its acquisition during late intrauterine life. The presentation of a large series of cases in fetal life is a unique opportunity to study the malformation at an early stage in its natural course.
A retrospective study was performed of four cases of aorto-left ventricular tunnel discovered among 872 cases of congenital abnormalities diagnosed at a tertiary centre for fetal echocardiography. Detailed echocardiographic and anatomical observations were made of the malformation as identified during fetal life. The precise anatomical arrangement was determined and compared with previous descriptions found in journals published in English.
In fetal life, as after birth, the malformation is characterised by enlargement and hypertrophy of the left ventricle, enlargement of the aortic root, and free regurgitation at the level of the aortic valve. Anatomical abnormalities are found at the aortic ventriculoarterial and sinutubular junctions as well as in the intervening aortic wall. These are unrelated to necrosis, ischaemia, or the presence of mucopolysaccharides.
The lesion is a developmental abnormality that should be reliably diagnosed by fetal echocardiography combined with colour flow Doppler echocardiography during the mid-trimester. The exact anatomical relations clarified by this study are pertinent to diagnosis and subsequent surgical correction.
研究在4例胎儿心脏中观察到的主动脉-左心室通道的超声心动图、形态学及组织学表现，并将结果与年长畸形患者的报告结果进行比较。
既往研究集中于该畸形从出生到成年期的临床特征，并推测其胚胎学形成或在子宫内晚期获得的过程。在胎儿期呈现大量病例是在其自然病程早期研究该畸形的独特机会。
对在一家三级胎儿超声心动图中心诊断的872例先天性异常病例中发现的4例主动脉-左心室通道病例进行回顾性研究。对胎儿期发现的畸形进行详细的超声心动图和解剖学观察。确定精确的解剖结构，并与英文期刊上先前的描述进行比较。
在胎儿期，与出生后一样，该畸形的特征是左心室扩大和肥厚、主动脉根部扩大以及主动脉瓣水平的自由反流。在主动脉心室动脉和窦管交界处以及其间的主动脉壁发现解剖学异常。这些与坏死、缺血或粘多糖的存在无关。
该病变是一种发育异常，在孕中期应通过胎儿超声心动图结合彩色多普勒超声心动图进行可靠诊断。本研究阐明的确切解剖关系与诊断及后续手术矫正相关。
