产前超声心动图诊断胎儿左心发育不良综合征

目的探讨产前超声心动图诊断胎儿左心发育不良综合征（HLHS）的技术要点。方法对产前超声检出的25例HLHS胎儿的临床及超声资料进行回顾性分析，重点探讨合并房间隔异常的HLHS胎儿超声声像图改变及血流动力学变化特点，并与尸检和出生后超声检查结果进行对比分析。结果25例HLHS胎儿中7例为二尖瓣及主动脉瓣闭锁，左心室重度发育不良；16例为二尖瓣闭锁合并不同程度主动脉瓣狭窄及主动脉缩窄，其中2例主动脉瓣狭窄伴主动脉缩窄随孕周进展其发育不良的左心室心内膜回声增强及左心室收缩力明显降低；另2例二尖瓣增厚和瓣膜开启运动受限伴主动脉瓣狭窄及主动脉弓缩窄。25例中7例伴房间隔完整，超声示房间隔增厚及卯园孔不开放，多普勒超声示房水平无分流信号，另外18例有房水平左向右少量分流；25例均见动脉导管逆向血流信号。结论产前超声心动图可早期诊断胎儿HLHS，除检出导致左心室发育不良系列左心系统病变外，房间隔完整或卵园孔径小及房水平的左向右分流也是评价HLHS预后及出生后手术治疗的重要指征。     

产前超声诊断胎儿主动脉闭锁

目的 探讨产前超声诊断胎儿主动脉闭锁的临床价值.方法 回顾性分析9例经引产后尸检证实的主动脉闭锁胎儿的产前超声心动图表现,总结其声像图特征.结果 9例主动脉闭锁胎儿声像图显示升主动脉及主动脉弓细窄7例,升主动脉及主动脉弓显示不清2例,彩色多普勒血流显像9例胎儿均显示主动脉与左心室无血流连接,主动脉弓显示反向血流.9例胎儿中6例伴发左心发育不良,2例伴发室间隔缺损,左心室发育良好,1例伴发完全型房室间隔缺损.9例胎儿检出时平均孕龄(26.0±2.6)周,孕龄最小者仅为孕16周.结论 产前超声诊断胎儿主动脉闭锁具有重要临床价值;升主动脉、主动脉弓细窄或显示不清,主动脉与左心室无血流连接及主动脉弓内反向血流是主要诊断依据;彩色多普勒血流显像有助于早期诊断.     

胎儿超声心动图对卵圆孔相关参数与左心发育不良关系的定量研究

目的：探讨超声测定卵圆孔相关参数在胎儿左心发育不良产前诊断中的临床意义。方法：测量188例胎儿的卵圆孔相关参数,分析其随孕周变化的规律,比较其与左心发育不良的相关性。结果：188例胎儿中正常胎儿165例,左心发育不良胎儿23例（左心发育不良综合征12例、主动脉瓣狭窄8例、主动脉弓缩窄3例）。正常胎儿的卵圆孔与主动脉内径相近,卵圆孔瓣开放径与主动脉内径的比值低于左心发育不良胎儿（P<0.01）,而卵圆孔瓣开放径与房间隔总长度的比值高于伴有左心发育不良的胎儿（P<0.01）。结论：超声心动图测定卵圆孔相关参数在胎儿左心发育不良的产前诊断中具有重要的辅助诊断价值。     

产前超声心动图诊断胎儿左心室发育不良综合征

目的 探讨胎儿超声心动图诊断左心室发育不良综合征(HLHS)的临床价值。方法 对18胎HLHS胎儿的临床资料进行回顾性研究,分析其超声心动图特征。结果 18胎HLHS胎儿的超声心动图结果:①心尖四腔心切面:左心室小、心室发育不良。8胎二尖瓣及主动脉瓣闭锁,且左心室重度发育不良;5胎二尖瓣闭锁合并主动脉瓣狭窄及主动脉缩窄;3胎二尖瓣狭窄伴主动脉瓣狭窄及主动脉弓缩窄;2胎二尖瓣狭窄伴主动脉瓣闭锁及主动脉弓缩窄。②三血管切面:主动脉内径明显小于肺动脉,主动脉与肺动脉内径比值为0.25~0.40,平均0.38±0.18,主动脉横弓与动脉导管、肺动脉血流方向相反。③胸骨旁四腔心切面:6胎房间隔增厚,未显示房间隔穿隔血流。12胎胎儿卵圆孔径偏小、显示房间隔左向右分流。结论 胎儿超声心动图可早期诊断胎儿HLHS,结合染色体检查对围生期的监测和临床处理有重要意义。     

胎儿心室发育不良的产前超声心动图诊断价值

目的　探讨产前超声心动图对胎儿心室发育不良的诊断价值。方法　回顾性分析我院 2 6 4 8例行中孕超声筛查和高度怀疑胎儿心脏畸形的晚孕孕妇。首先确定胎儿心脏的位置及心轴 ,然后心脏三节段分析法进行分析。二维超声显示四腔心、左室流出道、右室流出道、心底短轴、主动脉弓、动脉导管弓及三血管切面 ,重点观察二尖瓣、三尖瓣、主动脉瓣和肺动脉瓣的启闭情况 ,再用多普勒超声和 M-型超声观察胎儿心脏各瓣膜的血流情况。结果　 2 6 4 8例行常规超声筛查孕妇中检出先天性心脏畸形 2 1例 ,其中心室发育不良 6例 (3例左心发育不良综合征和 3例右心发育不良 )。 6例心室发育不良均已引产并有 3例尸检 ,尸检结论与超声完全一致。结论　产前超声心动图对检出胎儿心室发育不良及其分型具有极重要的价值。二维超声心动图是根本 ,而多普勒超声和 M-型超声心动图也不可或缺。     

多普勒超声心动图对胎儿瓣膜性心脏病的诊断价值

目的：探讨多普勒超声心动图对胎儿瓣膜性心脏病的诊断价值。方法：对高危致畸因素的1100例胎儿进行多普勒超声心动图检查，并对患瓣膜性心脏病的胎儿生后进行复杂。结果：先天性瓣膜性心脏病16例，其中肺动脉瓣狭窄2例，肺动脉瓣闭锁1例，肺动脉瓣返流2例，主动脉瓣狭窄2例，主动脉返流1例，三尖瓣闭锁1例，三尖瓣下移1例，三尖瓣返流5例，二尖瓣返流1例，结论：多普勒超声心动图对胎儿瓣膜性心脏病具有特异性诊断价值。     

胎儿左心室发育不良综合征并肺静脉共同腔闭锁尸检1例CSCD

目的探讨1例左心发育不良综合征并肺静脉共同腔闭锁胎儿的临床资料、超声分析及尸检解剖学特点。方法分析超声心动检查发现的复杂胎儿先天性心脏畸形病例,引产后进行尸体病理解剖,心、血管畸形按心脏节段分析方法进行分析和总结。结果胎儿左心发育不良综合征为复杂的先天性心脏畸形,同时合并肺静脉共同腔闭锁的病例更罕见,母体超声心动图可描述比较显著的解剖及生理特征,引产后详细的尸体解剖可进一步明确诊断。本例可见房间隔缺如、单心房、左心室腔小壁厚,二尖瓣闭锁及肺静脉共同腔闭锁,而且胎儿与婴幼儿的肺静脉共同腔闭锁不同,组织学不伴有肺淋巴管扩张。结论胎儿左心发育不良综合征合并肺静脉共同腔闭锁为少见的先天性心血管畸形,诊断更加困难。     

胎儿左心室发育不良综合征并肺静脉共同腔闭锁尸检1例

目的探讨1例左心发育不良综合征并肺静脉共同腔闭锁胎儿的临床资料、超声分析及尸检解剖学特点。方法分析超声心动检查发现的复杂胎儿先天性心脏畸形病例,引产后进行尸体病理解剖,心、血管畸形按心脏节段分析方法进行分析和总结。结果胎儿左心发育不良综合征为复杂的先天性心脏畸形,同时合并肺静脉共同腔闭锁的病例更罕见,母体超声心动图可描述比较显著的解剖及生理特征,引产后详细的尸体解剖可进一步明确诊断。本例可见房间隔缺如、单心房、左心室腔小壁厚,二尖瓣闭锁及肺静脉共同腔闭锁,而且胎儿与婴幼儿的肺静脉共同腔闭锁不同,组织学不伴有肺淋巴管扩张。结论胎儿左心发育不良综合征合并肺静脉共同腔闭锁为少见的先天性心血管畸形,诊断更加困难。     

彩色多普勒超声心动图对钙化性心脏瓣膜病变的诊断价值

目的回顾分析钙化性心脏瓣膜病的超声心动图特点,评价彩色多普勒超声心动图对钙化性心脏瓣膜病的诊断价值。方法应用彩色多普勒超声诊断仪进行超声心动图检测,观察瓣膜形态、厚度与狭窄、反流的部位及程度,以及房室大小、心功能等变化。结果单纯主动脉瓣受累71例,二尖瓣受累13例,主动脉瓣和二尖瓣联合受累16例,同时出现相应的瓣膜功能受损,以及心脏结构和功能的改变。结论应用彩色多普勒超声心动图可以直观地发现瓣膜钙化部位,评价瓣膜功能,并且可以检测到心脏结构及心功能的改变,对钙化性心脏瓣膜病的诊断及鉴别诊断有很高的临床应用价值。     

彩色多普勒超声与DSA检测颅外段椎动脉狭窄和发育不良的临床对比研究

目的：与数字减影血管造影（DSA）比较,探讨彩色多普勒超声在颅外段椎动脉狭窄和发育不良诊断中的价值。方法：回顾性分析68例椎动脉狭窄或发育不良患者的彩色多普勒超声和DSA检测结果,进行彩色多普勒超声与DSA两种方法检测椎动脉狭窄或椎动脉发育不良的Kappa一致性检验。结果：彩色多普勒超声检测椎动脉狭窄与DSA检查比较,两种方法一致性较好,然而在检测椎动脉发育不良时,两种方法一致性差。结论：虽然DSA是诊断椎动脉疾病的金标准,但彩色多普勒超声在椎动脉狭窄疾病诊断方面已显示出极大的优越性,推荐彩色多普勒超声作为椎动脉狭窄疾病的初次筛查方法。     

Prenatal sonographic features of isolated hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) is a spectrum of fetal conditions associated with severe hypoplasia of the left ventricle and left ventricular outflow tract. The purpose of this series was to focus on the sonographic prenatal features of isolated HLHS. Based on the 5 cases presented here, the prenatal sonographic features of HLHS include small-sized left ventricle, atretic or hypoplastic mitral valves with restricted motion and a small amount of antegrade flow or regurgitation, and hypoplastic ascending aorta. Retrograde flow in the transverse aortic arch strongly suggests inadequate systemic output from the left heart. Tricuspid regurgitation is common and increases the chance for the development of hydrops. Other findings may be helpful, including left ventricular hyperechoic endocardium and increased nuchal translucency at 11-14 weeks. With careful fetal echocardiography, HLHS may be readily observed on a 4-chamber view as either a small or even absent left ventricle.     

Prenatal diagnosis of ventriculocoronary arterial communication in fetuses with hypoplastic left heart syndrome.

OBJECTIVE: The purpose of this series was to describe the fetal echocardiographic findings in hypoplastic left heart syndrome with aortic atresia and ventriculocoronary arterial communication and implications of these findings. METHODS: We describe 2 fetuses with hypoplastic left heart syndrome with ventriculocoronary arterial communication diagnosed at 29 and 20 weeks' gestation, respectively. The underlying cardiac anatomy consisted of a hypoplastic left heart and mitral stenosis with aortic atresia. We used color Doppler and pulsed Doppler sonography on the surface of the myocardium to specifically look for coronary arterial flow. RESULTS: By color Doppler sonography, ventriculocoronary arterial communication was shown between the left ventricular cavity and the left coronary artery with characteristic bidirectional flow on pulsed Doppler examination. There was no mitral regurgitation. The left ventricular myocardium was substantially hypertrophied. The first patient underwent surgical Norwood palliation and died after a prolonged postoperative course. The second patient underwent stenting of the arterial duct and bilateral pulmonary artery banding in the catheterization laboratory but died after a few weeks. Implications of ventriculocoronary arterial communication in association with hypoplastic left heart syndrome are discussed. CONCLUSIONS: It is possible to accurately diagnose ventriculocoronary arterial communication on fetal echocardiography. The presence of ventriculocoronary arterial communication is seen exclusively in a subgroup of patients with an aortic atresia and mitral stenosis variant of hypoplastic left heart syndrome. The prognosis is poor in this subgroup of patients.     

Changes in left heart hemodynamics after technically successful in-utero aortic valvuloplasty.

OBJECTIVE: Severe aortic stenosis in the mid-gestation fetus can progress to hypoplastic left heart syndrome (HLHS). @ In-utero aortic valvuloplasty is an innovative therapy to promote left ventricular growth and function and potentially to prevent HLHS. This study evaluated the effects of mid-gestation fetal balloon aortic valvuloplasty on subsequent fetal left ventricular function and left heart Doppler characteristics. METHODS: We reviewed fetuses with aortic stenosis that underwent attempted in-utero aortic valvuloplasty between 2000 and 2006. Pre-intervention and the latest post-intervention fetal echocardiograms were analyzed to characterize changes in left heart function and Doppler characteristics in utero. RESULTS: Forty-two fetuses underwent attempted aortic valvuloplasty during the study period, 12 of which were excluded from analysis secondary to inadequate follow-up data, termination or fetal demise. Study fetuses (n = 30) underwent pre-intervention echocardiography at a median gestational age of 23 weeks, and were followed for a median of 66 +/- 23 days post-intervention. In 26 fetuses, aortic valvuloplasty was technically successful. Among these 26, left heart physiology was abnormal pre-intervention and improved or normalized after intervention in most cases: biphasic mitral inflow was present in 5/25 (20%) cases pre-intervention and in 21/23 (91%) post-intervention (P < 0.001); moderate or severe mitral regurgitation was present in 14/26 (54%) cases pre-intervention and in 5/23 (22%) post-intervention (P = 0.02); bidirectional flow across the patent foramen ovale was present in 0/26 cases pre-intervention and in 6/25 (24%) post-intervention (P = 0.01); antegrade flow in the transverse arch was present in 0/25 cases pre-intervention and in 17/26 (65%) post-intervention (P < 0.001). The left ventricular ejection fraction increased from 19 +/- 10% pre-intervention to 39 +/- 14% post-intervention (P < 0.001). These changes were not observed in control fetuses (n = 18). CONCLUSION: Fetal aortic valvuloplasty, when technically successful, improves left ventricular systolic function and left heart Doppler characteristics.     

Hemodynamic evaluation of normally functioning Sulzer Carbomedics prosthetic valves

The Sulzer Carbomedics prosthetic heart valve (CP) is a commonly used mechanical valve in clinical practice. In the present study, we used conventional and color Doppler echocardiography to assess the hemodynamics of normally functioning CP in the aortic (n = 73) and mitral (n = 127) positions. Our findings demonstrate no significant correlation of Doppler-measured peak and mean pressure gradients and effective orifice area with implanted valve size and actual orifice areas, measured directly by the manufacturer for CPs in both the mitral and aortic positions. However, it is still useful to measure effective orifice area by Doppler because a value in the normal or nonstenotic range points to an unobstructed prosthesis in the aortic or mitral position, in the absence of poor left ventricular ejection fraction. A value in the stenotic range could mean a normally functioning or obstructed prosthesis and, therefore, may need further investigation, such as assessment of valve leaflet motion by transthoracic or transesophageal echocardiography or fluoroscopy. Valve regurgitation as evaluated by color Doppler flow mapping was mild in practically all CPs in the aortic position, and in the majority of CPs in the mitral position.     

室间隔完整的肺动脉闭锁的产前超声诊断

目的探讨室间隔完整的肺动脉闭锁的胎儿期超声诊断声像特征、检测技巧及其鉴别诊断,提高此类畸形的产前检出率。 方法对33200例胎儿均采取胎儿心脏四腔心切面加胎儿头侧偏转法获得四腔心切面及左右心室流出道及主、肺动脉长轴切面快速筛查胎儿心脏畸形,对疑有胎儿心脏畸形者则更行详细的胎儿彩色多普勒超声心脏检查。分析8例产前及产后诊断的室间隔完整的肺动脉闭锁的声像特征和病理特征。 结果产前共诊断室间隔完整的肺动脉闭锁8例,其主要声像特征是8例均有肺动脉内径细小,彩色多普勒于三血管气管平面显示7例动脉导管内血流反向,7例右室右房扩大,6例右室壁厚,7例中重度三尖瓣反流,1例三尖瓣下移畸形,1例三尖瓣狭窄并右室小。 结论肺动脉内径细小、不同程度的右房右室扩大、三尖瓣畸形、右室发育不良、动脉导管内血流反向是肺动脉闭锁的主要声像特征,三血管气管平面是产前超声诊断室间隔完整的肺动脉闭锁的关键切面,需注意与主动脉闭锁、永存动脉干进行鉴别。     

多普勒超声对正常胎儿心脏机械性PR间期的临床研究

目的探讨多普勒超声对正常胎儿心脏机械性运动PR间期的检测方法。方法将受检胎儿300例分为早、中、晚孕3组,在标准胎儿左心室流人道及流出道切面上,运用彩色多普勒引导脉冲多普勒取样容积记录左心室流人道及流出道血流频谱,测量从舒张期二尖瓣A峰起始至收缩期主动脉射血起始的心房剑心窀的传导时间（PR问期）,并同时测量收缩期主动脉射血起始至二尖瓣A峰开始的时间（VA间期）。结果胎儿孕周、心率、PR间期、VA间期的数值均呈正态分布,300例胎儿的平均孕周为（21．33±9．55）周,平均心率（152．34±10．83）次／min,平均PR间期（105．63±13．35）ms,平均VA间期（283．294±23．57）ms。不同孕期组间的孕周、胎儿心率、PR间期、VA间期的差异均有统计学意义（P=0．000）,经SNK—q检验显示不同孕期组间两两差异均有统计学意义（P〈0．01）。结论应用多普勒超卢测量PR间期和VA间期方法简便,图像清晰且重复性好,该方法可对胎儿I度房室传导阻滞捉供诊断信息,并可对胎儿房室传导阻滞的防治和预后提供依据。     

Prenatal diagnosis of bilateral ventriculocoronary fistulas with hypoplasia of the left ventricle.

Fetal echocardiography and color Doppler sonography have shown that most heart defects can be reliably diagnosed in utero by associated hemodynamic changes. Congenital ventriculocoronary fistulas associated with hypoplastic right heart syndrome (HRHS) and hypoplastic left heart syndrome (HLHS) have been described in the prenatal and neonatal periods, but little attention has been paid to their incidence and hemodynamic changes. There have been only a few reports of prenatal diagnosis of ventriculocoronary communications in HRHS and HLHS. Here we report prenatal diagnosis of HLHS and fistulas between the left ventricle and both the right and left coronary arteries to the pulmonary artery with a muscular ventricular septal defect (VSD) in a fetus at 23 weeks' gestation.     

产前超声诊断胎儿左心发育不良综合征

目的探讨胎儿左心发育不良综合征产前超声诊断方法 ,提高诊断准确性。方法常规产前超声检查方法 ,应用四腔心切面和三血管气管平面进行胎儿心脏畸形筛查。结果 2006年1月2008年12月发现4例左心发育不良综合征,3例并发心内畸形（2例右室双出口及1例室缺）,1例并发心外畸形（Dandy-Walker畸形及单脐动脉）。结论应用四腔心切面和三血管气管平面筛查心室发育不良简单易行,准确性高。     

Change in amplitude distributions of Doppler spectrograms recorded below the aortic valve in patients with a valvular aortic stenosis.

Amplitude distributions of Doppler spectrograms were characterized in a group of 22 patients having no aortic pressure gradient and another group of 26 patients having a stenotic aortic valve. Specifically, for each patient, the ratios of the mean amplitude in three normalized frequency bands (low, middle and high) to the mean amplitude of the Doppler spectrogram computed in selected portions of the systolic period were considered. Pulsed-wave Doppler spectrograms were recorded by positioning the sample volume in the left ventricular outflow tract, approximately 1 cm below the aortic valve. Statistically significant differences were found between the middle (p = 0.041) and high (p = 0.028) frequency bands of Doppler signals recorded from the two groups of patients. The differences observed are believed to be attributed to blood flow eddies generated below the stenotic aortic heart valve and to changes in blood flow orientation.