Transesophageal echocardiography in children with congenital heart disease: an initial experience

Transesophageal echocardiography with a single plane (transverse axis), dedicated pediatric probe was performed prospectively in 25 anesthetized children undergoing routine cardiac catheterization or intracardiac surgery, to assess the potential role of this technique in the initial diagnosis, perioperative management and postoperative follow-up of children with congenital heart disease. The group ranged in age from 1 year to 14.8 years (mean 6.1) and weight from 6.5 to 52 kg (mean 22.4). Studies were successful in all patients and no complications were encountered. The results of the transesophageal studies (combined imaging, color flow mapping and pulsed wave Doppler sampling) were correlated both with the results of prior precordial studies and the information obtained at cardiac catheterization. Transesophageal echocardiography provided a more detailed evaluation of the morphology and function of systemic and pulmonary venous return, the atria, interatrial baffles, atrioventricular valves and the left ventricular outflow tract. Additional information was obtained in 15 patients (60%). Problem areas for single plane transesophageal imaging were the apical interventricular septum, the right ventricular outflow tract and the left pulmonary artery. The intraoperative use of transesophageal echocardiography allowed assessment of the surgical repair and monitoring of ventricular function and volume status while the patient was weaned from cardiopulmonary bypass. Transesophageal echocardiography in pediatric patients is of additional value in three main areas: 1) the precise morphologic diagnosis of congenital heart disease, 2) perioperative monitoring, and 3) postsurgical follow-up.     

Morphologic features of carcinoid heart disease as assessed by three-dimensional transesophageal echocardiography

Background: Carcinoid heart disease (CHD) is a rare cause of valvular heart disease and carries a poor prognosis. CHD has a unique morphology and echocardiographic features that predominantly involve right‐sided valvular structures. The diagnosis of CHD is usually made by two‐dimensional transthoracic echocardiography (TTE). With the superior spatial resolution of real time three‐dimensional transesophageal echocardiography (3DTEE), structural changes that occur in patients with CHD‐associated valvular heart disease can be examined in greater detail. We undertook this study to examine the incremental value of 3DTEE in the diagnosis of CHD. Methods: A total of four patients with CHD underwent TTE, transesophageal echocardiography (TEE), and 3DTEE as part of their routine clinical evaluation. Results: TTE and TEE for all four patients revealed thickened, fibrosed, retracted, and malcoapted tricuspid leaflets with wide‐open tricuspid valve regurgitation. 3DTEE en face imaging of the tricuspid valve demonstrated the characteristic morphologic features of CHD more clearly in all four patients. Conclusions: 3DTEE provides substantial incremental value over TTE in the assessment of characteristic CHD pathology and thus enhances the echocardiographic diagnosis of CHD. (Echocardiography 2010;27:1098‐1105)     

Transesophageal real-time echocardiography in infants and children with congenital heart disease.

Transesophageal echocardiography using Doppler color flow mapping with both single plane transverse and longitudinal pediatric probes was performed in 127 infants and children both intraoperatively and postoperatively. The 127 patients ranged in age from 1 day to 18 years (mean 37 months) and in weight from 2.4 to 45 kg (mean 13.2); 157 studies were successfully performed (100% of attempted) with no complications. Transesophageal two-dimensional echocardiography combined with pulsed wave Doppler ultrasound and color flow mapping provided detailed morphologic as well as physiologic information, with additional information provided overall in 56% of the cases. Typical limitations of single-plane transverse imaging were overcome using a prototype longitudinal-axis pediatric probe. Both left and right ventricular outflow tracts, distal pulmonary arteries and all of the interventricular and atrial septa were visualized. Assessment of surgical repair intraoperatively as well as assessment postoperatively for cardiac function, residual intracardiac shunts, residual valvular sequelae and other hemodynamic events was important and easily performed in an otherwise inaccessible patient. Transesophageal echocardiography in infants and in small children is a valuable "noninvasive" imaging technique that offers important additional and complementary information.     

Applications of three-dimensional transesophageal echocardiography in congenital heart disease

Three-dimensional echocardiography allows for presurgical planning for congenital heart disease, reduces radiation using fusion imaging in catheter interventions, and provides guidance during catheter interventions and lead placements or extractions. The purpose of this review is to detail applications of three-dimensional transesophageal echocardiography in presurgical planning of congenital heart disease, guidance of catheter interventions such as fusion imaging, and guidance in electrophysiology lead extractions or placements.     

Real time three-dimensional transthoracic echocardiography in congenital heart disease

Diagnosing congenital heart disease (CHD) depends heavily on imaging. Traditionally, two-dimensional (2D) echocardiography has been the most widely used but since this imaging modality utilizes a 2D technique to evaluate three-dimensional (3D) structures it suffers from inherent limitations. The more recently developed 3D echocardiography is poised to be superior in providing comprehensive evaluation prior to intervention on such complex conditions. In this review, we summarize the applications of 3D echocardiography in evaluating patients with CHD.     

Partial atrioventricular septal defect assessed by real-time three-dimensional echocardiography: a case report

A 48-year-old woman was admitted with a heart murmur and increased difficulty in breathing. Two-dimensional echocardiography revealed a defect in the lower part of the atrial septum [(primum atrial septal defect (ASD)] and a pouch at the interventricular septum. Color Doppler echocardiography detected grade 3/4 mitral regurgitation. Real-time three-dimensional echocardiography (RT-3DE) revealed a cleft in the anterior leaflet of the mitral valve toward the tricuspid valve, and the ASD located near the atrioventricular valves with 14mm in minor axis. Color Doppler three-dimensional echocardiography disclosed left-to-right ASD shunt toward the atrial posterior wall. No shunt through the pouch at the membranous part, left ventricular outflow obstruction, or partial anomalous pulmonary venous connection was observed. RT-3DE is quite useful to describe complicated congenital heart disease.     

900例胎儿超声心动图诊断胎儿先天性心脏病的体会

目的　探讨胎儿超声心动图对胎儿期先天性心脏病的诊断方法及应用价值。方法　 1989年 11月～ 2 0 0 0年 11月对 90 0名高危孕妇行胎儿超声心动图检查。孕妇年龄 2 2～ 39岁 (2 7 9岁± 3 9岁 ) ,孕期 2 0～ 42周 (33 4周± 4 8周 )。首先应用二维超声心动图 (2DE)探察 ,横切胎儿胸部取标准四腔切面作为基础切面 ,旋转探头方向获取五腔心、大动脉短轴、左室长轴、主动脉弓长轴、下腔静脉长轴等切面 ,在清晰的切面图像上叠加彩色多普勒信号 ,观察心内有无异常血流 ;以脉冲多普勒记录主、肺动脉瓣及二、三尖瓣血流频谱或异常湍流频谱。结果　胎儿期先天性心脏病发病率3 78% (34 / 90 0 ) ,胎儿超声心动图诊断胎儿先天性心脏病的敏感性 92 %、特异性 95 %、阳性预测值 91%、阴性预测值96 %。结论　胎儿超声心动图诊断胎儿先天性心脏病的敏感性、特异性高 ,为妇产科及儿科医生提供有价值的资料 ,应作为高危孕妇的常规检查     

Dynamic three-dimensional echocardiography with a computed tomography imaging probe: initial clinical experience with transthoracic application in infants and children with congenital heart defects.

OBJECTIVE--To assess the clinical applicability of a prototype computed tomographic echocardiographic imaging probe in paediatric patients with congenital heart disease. DESIGN--A phased array echocardiographic transducer (64 elements, 5 MHz) mounted on a sliding carriage was used transthoracically in various positions on the chest. The transducer moves from the outflow tract to the apex of the heart in 0.5 to 1.3 mm increments and records a tomographic slice of the heart at each increment level. Parallel images are recorded at a frame rate of 25-30 images/s. At each level a complete cardiac cycle is recorded. The images are digitised and stored in the image processing computer, which reconstructs the anatomical structures of the heart in a three-dimensional format by means of different grey scales. PATIENTS--45 paediatric patients (age range 3 days to 17 years) with various congenital heart defects who had been admitted to hospital for diagnostic or therapeutic cardiac catheterisation or surgery. RESULTS--Good quality echocardiographic pictures were obtained in all but two of the 45 patients. Three-dimensional reconstructions of the heart were possible from transthoracic echocardiograms. The recorded cardiac chambers and valves were displayed in three-dimensions in real time (four-dimensionally). The heart was also displayed in real time in any desired plane and in up to five planes simultaneously without having to change the position of the transducer on the chest. Different parts of the heart were displayed in a view similar to that seen by a surgeon during an operation. Image acquisition took 3-5 minutes and three-dimensional reconstruction of various cardiac structures 20-90 minutes. CONCLUSIONS--The computed tomographic imaging probe facilitates acquisition of echocardiographic data as multiple planes can be obtained from one transducer position. Display of three-dimensional structures of the heart may enhance the understanding of cardiac anatomy.     

Cor triatriatum dexter assessed by three-dimensional echocardiography reconstruction in two adult patients

Cor triatriatum dexter is a rare congenital anomaly caused by persistence of the right valve of the embryonic sinus venosus, and this results in septation of the right atrium. Three-dimensional echocardiography is a novel technique that can be used to identify and characterize this entity. Here, we report two women with cor triatriatum dexter.     

Initial clinical experience of real-time three-dimensional echocardiography in neonates with isolated congenital ductus arteriosus aneurysm

BACKGROUND: Congenital ductus arteriosus aneurysm (DAA) was considered rare but potentially fatal abnormality, often followed by surgical intervention after careful evaluation. This prospective study used real-time three-dimensional echocardiography (RT3DE) to assist in evaluation of neonatal DAA. METHODS: A total of 1390 full-term neonates were enrolled in this study between 2002 and 2003. They received two-dimensional echocardiographic (2DE) screening and periodic follow-up. RT3DE was performed selectively for newborns with DAA. RESULTS: DAA were detected in 116 (8.34%) newborns using 2DE. Maximum diameter of the DAAs ranged from 6.8 to 14.0 mm (8.2+/-1.1 mm). None of the cases were symptomatic or had complications related to DAA. There were no significant differences in sex and gestational age between the newborns with and without DAA. Neonates with DAA had a higher birth body weight and a higher incidence of large-for-gestational-age (P<0.05). RT3DE provided instant, consistent and reliable 3D images of DAA and its related structures and allowed for more rapid examination times and reduction of baby wait times. CONCLUSIONS: Congenital DAA is as common as has been previously reported. RT3DE is useful in assisting evaluation of DAA. Preferred images of DAA were typically visualized in the high parasternal short-axis view before the third day of life. Routine use of RT3DE is suggested to enhance assessment of neonates with DAA detected by 2DE.     

Transcatheter closure of perimembranous ventricular septal defects with Amplatzer occluder assessed by real-time three-dimensional echocardiography.

We report our initial experience in two patients using real-time three-dimensional echocardiography to assess perimembranous ventricular septal defect and device morphology and their relation with contiguous cardiac structure. Defect size and rims as well as device position and profile were displayed from the three-dimensional "en face" views. We think that real-time three-dimensional echocardiography could be a complementary approach to angiography and transesophageal echocardiography in performing transcatheter closure of perimembranous ventricular septal defect.     

Real-time three-dimensional echocardiography of congenital heart disease using a high frequency paediatric matrix transducer.

New matrix transducers are now available for three-dimensional echocardiography which have a higher frequency and smaller footprint than previous matrix probes. This has resulted in better image resolution in infants and children. Current applications include assessment of cardiac morphology and function. Intraoperative epicardial techniques may be used in addition to a conventional transthoracic approach.     

The progression of fenfluramine-associated valvular heart disease assessed by echocardiography

BACKGROUND: An association between the dietary suppressants fenfluramine and dexfenfluramine and valvular heart disease was first described in patients from North Dakota and Minnesota in 1997. Limited data are available on the natural history of this valvulopathy after discontinuation of drug therapy. OBJECTIVE: To follow the progression of fenfluramine-associated valvular heart disease after discontinuation of therapy by using serial echocardiography. DESIGN: Retrospective cohort study. SETTING: Regional medical center in Fargo, North Dakota. PATIENTS: 50 patients with previous exposure to fenfluramines who had at least mild mitral regurgitation or aortic regurgitation after exposure to fenfluramines on serial echocardiography between December 1994 and February 1999 (96% were female, mean body mass index was 36.6 kg/m(2), and mean duration of drug exposure was 447 days). MEASUREMENTS: Serial echocardiograms were reviewed by two echocardiographers who were blinded to the order of image acquisition. The severity of valvular regurgitation and presence or absence of valve leaflet restriction were assessed. RESULTS: As described in the initial report, significant valvular disease on initial postexposure echocardiography was common in this cohort; 38 patients (76%) had at least mild mitral regurgitation and 43 patients (86%) had at least mild aortic regurgitation. On serial echocardiograms obtained an average of 356 days apart, mitral regurgitation improved by at least one grade in 17 patients (P = 0.001) and aortic regurgitation improved by at least one grade in 19 patients (P = 0.004). Nineteen and 22 patients, respectively, experienced no change in severity of mitral and aortic regurgitation. Two patients in each group experienced worsening of regurgitation by at least one grade. Results were similar for tricuspid (P = 0.002) and pulmonic (P = 0.012) regurgitation. CONCLUSION: On serial echocardiography, fenfluramine-associated valvular regurgitation improved or remained stable in most patients after therapy ended. Worsening of valvular regurgitation was uncommon. The potential for stabilization or regression of valvular regurgitation should be taken into account when counseling patients and considering the need for and timing of valve surgery.     

Transesophageal echocardiography for congenital heart disease

Surface echocardiographic imaging of small children is routinely successful in defining anatomical details and Doppler flow patterns with even the most complex congenital cardiac malformations. However, in larger children or adults, imaging is frequently limited. A recent expansion of the role of echocardiography is intraoperative epicardial imaging. Epicardial and postoperative imaging, however, have significant limitations. To avoid some of these limitations, transesophageal echocardiography has increasingly been used in the arena of congenital heart disease. The more recent development of small sized gastroscopic probes has allowed transesophageal echocardiographic assessment of congenital heart disease in children down to newborn size. As detailed studies of individual lesions are reported, it has become clear that the mere presence of a congenital heart defect is not an indication for transesophageal echocardiography in most children if imaging can be accomplished by surface examination. However, transesophageal echocardiography may be indicated for the intraoperative or postoperative assessment of that defect, particularly when repair has been difficult or is known to be associated with significant residual abnormalities. Cardiac structures encountered with horizontal and vertical imaging plane transducers have been described and should be completely familiar to the examining echocardiographer.     

Adult cyanotic congenital heart disease: surgical experience.

Cyanotic congenital heart diseases constitute about 10 percent of total congenital heart disease cases in adults in the developing world. Prolonged cyanosis and old age adversely affect the outcome of surgery, thus posing a challenge to the cardiac surgeons. This study was conducted to assess the feasibility, safety and outcome of surgery in this group of patients. From January 1991 to December 1997, a total of 303 patients, aged 14 to 54 years (mean 19.8 +/- 1.5 years) with diagnosis of various cyanotic congenital heart diseases were operated at our institute. There were 210 males (69.3%). Two hundred and forty-seven patients (81.5%) had tetralogy of Fallot's physiology, 51 patients (16.8%) had single ventricle physiology and five (1.6%) had other lesions. Sixty-six patients (21.7%) had pre-operative complications such as haemoptysis, epistaxis, cerebrovascular accidents, brain abscess and infective endocarditis. Sixty patients (19.8%) had previous palliative shunts and 26 patients (8.5%) had coil embolisation of major aortopulmonary collaterals prior to surgery; 229 patients (75.5%) underwent biventricular repair, 52 (17.1%) had univentricular repair, 22 (7.7%) had palliative shunts and one patient had open ligation of a major aortopulmonary collateral in addition. In-hospital mortality was 3.3 percent. Follow-up period ranged from five months to seven years (mean 4.2 +/- 1.8 years). There were two late deaths. Of the 291 survivors, 11 were lost to follow-up. Two hundred and fifty-eight patients (92.1%) are in New York Heart Association class I. Significant residual defects warranting reoperation were present in four patients (1.3%). It is concluded that congenital heart surgery in older cyanotic patients can be performed safely with satisfactory results.     

Three-dimensional echocardiography in congenital heart disease

Complex intracardiac anatomy and spatial relationships are inherent to congenital heart defects (CHDs). Recognition of the limitations of two-dimensional echocardiography has stimulated clinical interest in three-dimensional imaging. The current review examines contemporary studies in the following areas where three-dimensional echocardiography has provided additive value in CHD: (1) visualization of morphology, (2) quantitation of chamber sizes and ventricular function, and (3) image-guided interventions.     

Use of three-dimensional echocardiography for analysis of outflow obstruction in congenital heart disease

To evaluate the feasibility and accuracy of 3-dimensional (3D) echocardiography in analysis of left and right ventricular outflow tract (LVOT and RVOT) obstruction, 3D echocardiography was performed in 28 patients (age 4 months to 36 years) with outflow tract pathology. Type of lesion and relation to valves were assessed. Length and degree of obstruction were measured. Three-D data sets were adequate for reconstruction in 25 of 28 patients; 47 reconstructions were made. In 13 patients with LVOT obstruction, 3D echocardiography was used to study subvalvular details in 8, valvular in 13, and supravalvular in 1. Four of these 13 patients had complex subaortic obstruction. In 12 patients with RVOT lesions, 3D echocardiography was used to study subvalvular details in 11, valvular in 12, and supravalvular in 2. Three-dimensional reconstructions were suitable for analysis in 100% of subvalvular LVOT, 77% valvular LVOT, 100% supravalvular LVOT, 100% subvalvular RVOT, 50% valvular RVOT, and 50% supravalvular RVOT. Twenty patients underwent operation, and surgical findings served as morphologic control for thirty-four 3D reconstructions (LVOT 17, RVOT 17). Operative findings revealed an accuracy at subvalvular LVOT of 100%, valvular LVOT 90%, supravalvular LVOT 100%, subvalvular RVOT 100%, valvular RVOT 100%, and supravalvular RVOT 100%. Quantitative measurements could adequately be performed. Three-D echocardiography is feasible and accurate for analyzing both outflow tracts of the heart. Particularly, generation of nonconventional horizontal cross sections allows a good definition of extension and severity of lesions.     

Three-dimensional echocardiography in congenital heart disease

Matrix array technology has brought three-dimensional echocardiography into the clinical practice of cardiology. Arguably, this advancement is most notable in the field of pediatric cardiology. Full-volume acquisitions now can be undertaken in the youngest of infants with excellent image quality. This article illustrates the clinical application of three-dimensional echocardiography in congenital heart disease.