圆锥动脉干畸形的三维超声病理形态学诊断的研究

Pathological diagnosis of conotruncal defects by transthoracic three-dimensional echocardiography

目的探讨三维超声心动图(3DE)在小儿圆锥动脉干畸形(CTD)病理形态学诊断中的应用价值.方法用HP Sonos 5500型彩色超声心动图仪和Tom Tec Echo-view 4.2对210例0～15岁CTD 患儿进行二维超声心动图(2DE)和3DE检查,并以心导管造影和手术诊断为标准,用5剖切面10剖视面三维超声剖视诊断方案和Van Praagh先天性心脏病顺序分段诊断步骤对CTD作3DE剖视诊断,结果与2DE对比.结果房、室间隔左、右心观(L1a、L2a)、心脏四腔观(Ha、Hb)、瓣膜俯视及仰视观(S1a、S2a)等剖视面对CTD有诊断价值,在心室构型,心室大动脉连接,室间隔缺损具体位置、形态及其与大动脉相互关系等方面能较2DE提供更多空间信息.在已手术的147 例中,补充2DE诊断18例(12.2%),纠正2DE诊断10 例(6.8%).结论 3DE可对CTD作出较准确的三维病理形态学诊断,能基本满足CTD 3DE剖视诊断要求.

Objective Recently, it has been demonstrated in several studies that three-dimensional echocardiography （3DE） with any cut view interpretation of the cardiac morphological structures and its dynamic three-dimensional display can conform to the need of accurate anatomical diagnosis of complex congenital heart diseases including conotruncal defects （CTD）. 3DE can not only provide functional and morphological information of CTD to decide on surgical repair but also simulate the intra operative visualization of complex anatomical cardiac structures to improve the understanding of the anatomy of CTD, but there have been scarce studies to assess systematically the potential noninvasive diagnostic value of transthoracic 3DE for CTD in children. Thus, this study attempted to evaluate the diagnostic value of transthoracic 3DE for the pathological diagnosis of CTD in children. Methods HP Sonos 5500 echocardiographic system interfaced with Tomtec 3DE imaging system （echo-view 4. 2 software） was used to diagnose 210 patients （ 132 male, 78 female, aged 0-15 years, mean 3.2 ± 3.2 years ） with CTD in Shanghai Children＇s Medical Center from May 2000 to November 2003, including 62 patients with double outlet right ventricle, 48 patients with transposition of great arteries, 48 patients with tetralogy of Fallot, 38 patients with pulmonary atresia with interventricular septal defect and 14 patients with persistent truncus arteriosus. Diagnostic method consisting of ten designed 3DE sectional views and Van Praagh＇s sequential segmental approach in congenital heart diseases was used in this study. And the results were compared with those diagnosed by two-dimensional echocardiography （2DE） and angiocardiography. Surgical findings were considered as the “gold standard”. Results There were 526 image acquisitions of transthoracic 3DE in the 210 patients with CTD including 200 through subcostal window, 254 through parasternal window and 72 through apical window, among which 477 image acquisitions （93.2 % ） could demonstrate good spatial cardiovascular structures. Meanwhile, six 3DE sectional views of the diagnostic method of the ten designed 3DE sectional views were identified for the diagnosis of CTD as follows： en-face view of atrial and ventricular septa from left ventricle （Lla） ; en-face view of atrial and ventricular septa from right ventricle （L2a） ; fourchamber view of whole-heart oblique frontal plane （ Ha and Hb） ; en-face view of atrial-ventricular valve from atria （Sla） and en-face view of atrial-ventricular valve from ventricles （S2a）. The 3DE sectional diagnosis showed that 3DE could provide more information of inner cardiac structure than 2DE, such as ventricular configuration identification, atrial-ventricular and ventricular-arterial continuity, location and shape of ventricular septal defect （ VSD ） , and appreciation of the defects related with aorta and pulmonary arteries, etc. In 147 cases of surgical repair, both 3DE and 2DE were in accordance with the surgical findings, but 3DE had new findings in 18 （12. 2% ） cases and corrected the diagnoses in 10 （6. 8% ） cases as compared with 2DE. Coneltmion The 3DE sectional diagnostic method could make a successfully sectional diagnosis of pathological morphology in CTD by demonstrating more spatial information of inner cardiac structure than 2DE and angiocardiography.