Intraoperative Transesophageal Echocardiography in a 1.4-kg Infant with Complex Congenital Heart Disease

The lower weight limit for infants undergoing intraoperative transesophageal echocardiography (TEE) with current commercially available probes has not been determined. A review of the literature reveals that infants as small as 1.6 kg have been studied successfully. This report describes the first intraoperative TEE reported in a 1.4-kg infant during truncus arteriosus/interrupted aortic arch repair. Successful pre- and postoperative images of the cardiac abnormality were obtained. Probe insertion was performed in this small patient after predilating the esophagus with a 14-F suction catheter.     

Prolonged Electrical Quiescence After Direct Current Cardioversion for Atrial Flutter in Congenital Heart Disease

Elective direct current cardioversion is considered first-line treatment in many cases of atrial flutter and fibrillation. This also is true in the pediatric population. This report describes a case of successful cardioversion that resulted in a very prolonged electrical quiescence.     

超声心动图在新生儿先天性心脏病筛查中的应用

目的探讨超声心动图对新生儿先天性心脏病(CHD)临床筛查的应用价值。方法对本院2010年1月-2011年12月出生的330例疑似CHD新生儿的心脏超声心动图筛查结果进行分析,应用心脏彩超对新生儿经胸行多切面扫查,了解心脏及大血管位置关系、内径比例,测量房室大小、室壁厚度、运动幅度,测量心功能。观察瓣膜厚度、活动度、房室间隔是否完整。测量瓣口血流速度,观察瓣口有无返流,心腔及大血管间是否存在分流。结果 330例新生儿中单纯卵圆孔未闭96例(29.0%),单纯PDA 45例(13.6%)。CHD 116例(35.1%),其中单纯VSD 16例(13.8%),VSD并卵圆孔未闭15例(12.9%),单纯ASD 13例(11.2%),ASD并卵圆孔未闭13例(11.2%),ASD并VSD 8例(6.9%),ASD并VSD并卵圆孔未闭7例(6.0%),ASD并PDA 17例(14.7%),法洛四联症14例(4.2%),完全性心内膜垫缺损6例(1.8%),右心室双出口3例(0.9%),大动脉转位、三尖瓣下移畸形各2例(各占0.6%)。结论新生儿期较为多见的心内分流为卵圆孔未闭,其与PDA在未来均有闭合的可能。目前绝大多数常见的CHD可通过超声检查得到确诊,可取代大部分心导管检查和心血管造影对于CHD的诊断。     

Creation and Enlargement of Atrial Defects in Congenital Heart Disease

Transcatheter creation and enlargement of interatrial defects (IAD) may improve hemodynamics; however, procedural outcomes have not been well defined. Hospital records were reviewed for children who underwent percutaneous procedures to create and enlarge an IAD and were grouped as follows: (1) right and (2) left heart obstructive lesions, (3) left atrial (LA) decompression during left heart assist, (4) failing Fontan circulation, and (5) miscellaneous. Forty-five children (mean age, 3.4 ± 4.7 years; 30 (67%) male) were identified. In group 1 (n = 6), all achieved endpoints of right atrial (RA) decompression (n = 2), improved left ventricular filling (n = 3), or improved arterial saturations (n = 1). In group 2 (n =18), mean LA pressure decreased (21 ± 6 to 13 ± 5 mmHg, p < 0.001) and arterial saturations increased (61 ± 13% to 78 ± 11%, p < 0.001). All except 2 patients achieved definitive repair, further palliation (n = 9), or heart transplantation (HTX) (n = 7). In group 3 (n = 5), the LA was decompressed (21 to 13 mmHg, p = 0.03) in all, and all except 1 patient survived to HTX (n = 2) or full recovery (n = 2). In group 4 (n = 11), of 7 patients with a low cardiac output syndrome after surgery, despite improved atrial shunting, 3 died and 1 required a HTX. In group 5 (n=5), RA decompression (n = 1) or improved arterial saturation (n = 4) was achieved in all. Overall, 5-year HTX free survival was 75%. Mechanical ventilation before the procedure (p < 0.001), the need for a blade septostomy (p = 0.002), and higher LA pressures after the procedure (p = 0.04) independently predicted mortality or the requirement for HTX. Transcatheter optimization of an atrial communication can help optimize treatment strategies and has a low procedural risk.     

Intraoperative Transesophageal Echocardiogram Using an Intracardiac Ultrasound Catheter in a Congenital Heart Surgery: A Case Report

Transesophageal echocardiography (TEE) during cardiac surgery is a routine procedure. The use of pediatric TEE probes is limited in small infants weighing less than 5 kg. Recent reports have shown the safety of monoplane intravascular ultrasound catheters in transesophageal echocardiograms. This report describes the case of a newborn with total anomalous pulmonary venous return who underwent cardiac surgery. A pre- and postbypass TEE examination was performed, with successful visualization of the cardiac anatomy and function and no complications.     

The Impact of Fetal Echocardiography on the Prevalence of Liveborn Congenital Heart Disease

Fetal echocardiography allows for early detection of congenital heart disease, and pregnancy termination may be an option in cases of complex defects. In the current study, the most important factors contributing to the diagnosis and termination of affected pregnancies are reviewed and their combined effect on the future prevalence of liveborn congenital heart disease is evaluated. The relative reduction of the prevalence of the most severe forms of congenital heart disease is estimated as the product of the probability that (1) a fetal cardiac screening is performed (p _evaluation), (2) an affected pregnancy is detected (P _detection), (3) pregnancy termination is decided following antenatal diagnosis (P _decision). In areas where termination of pregnancy is a realistic and supported option, a universal sonographic screening of all pregnancies (P _evaluation = 1), with an average reported sensitivity of 35% and a termination rate of 43% following antenatal diagnosis, would result in a 15% overall reduction of the prevalence of the most severe forms of congenital heart disease. However, wide variability exists regarding the defect-specific estimates (2–50% prevalence relative reduction) due to considerable differences in the reported diagnostic sensitivity and termination rates associated with each heart defect. If an earlier diagnosis could be achieved, which is reported to be associated with an average 1.4-fold increased probability of termination, the overall reduction of the prevalence of congenital heart disease could approach 21%. As the skills of obstetric and pediatric cardiology sonographers improve, fetal echocardiography is expected to have a substantial impact on the future epidemiology of liveborn congenital heart disease.     

超声心动图在先天性心脏病介入治疗中的应用进展

随着计算机和超声技术的迅速发展,超声心动图(ECHO)在指导先天性心脏病介入治疗术前合理筛选病例、监测介入方法及正确实施介入操作、评估介入术后的即刻和中远期疗效等方面起着越来越重要的作用。该文系统阐述了近年来ECHO在先天性心脏病介入治疗中的应用进展,着重介绍各种超声技术在介入术中的优缺点,以期有助于ECHO技术的合理选择和良好应用。     

Combining Pulse Oximetry and Clinical Examination in Screening for Congenital Heart Disease

The objective of this study was to evaluate combined pulse oximetry and clinical examination as a screening method for congenital heart disease (CHD) in asymptomatic newborns. Asymptomatic newborns were screened for CHD using pulse oximetry and clinical examination before their discharge from the nursery. Oxygen saturation ≥94% was considered normal. Echocardiography was done for newborns with abnormal readings and for those with significant murmurs. Data concerning undetected cases were collected from the pediatric referral hospital. A total of 5211 cases were screened. Echocardiographic evaluations were done based on low pulse oximetry in five cases and on murmur detection in ten others. The sensitivity of the combined method of screening was 77%, whereas it was 31% for oximetry alone and 46% for clinical examination alone. Specificity was ∼100% for all methods. The positive predictive value of the combined tool was 66.7%. We conclude that combining pulse oximetry and clinical examination can enhance the clinician’s ability to detect life-threatening CHD in a timely manner. This screening method should become a part of the discharge plan for every newborn.     

实时三维超声心动图在先天性心脏病诊疗中的应用

实时三维超声心动图(RT-3DE)可实时采集、同步显示心脏血管的立体动态影像,在心血管疾病诊疗工作中具有独特优势。近年来RT-3DE发展迅速,已由临床研究逐步进入应用阶段,也为小儿先天性心脏病(CHD)的诊治提供一新途径,本文就RT-3DE在小儿CHD诊断和治疗中的应用作一综述。     

Intracardiac Thrombus in Children: The Fine Equilibrium Between the Risk and the Benefit

The medical records of 16 patients diagnosed as intracardiac thrombus were searched. The size, location and outcome of thrombus together with demographic data of patients were assessed. The median age of the patients was 2.2 years. Six patients were newborn and two patients were infant. The median size of thrombus was 9 mm. The localization was right atrium in seven, right ventricle in five, left ventricle in one, pulmonary artery in one, and superior vena cava in two patients. There was prematurity in five, ciyanotic congenital heart disease in one, blood culture positivity in three, malignancy in four, nephrotic syndrome in one, indwelling catheters in 10, and acquired or genetic thrombophilia in six patients as risk factors. In the treatment, the first choice was tissue plasminogen activator in two patients, heparin infusion in one patient and low molecular weight heparin in remaining 12 patients. In nine patients, therapy included parenteral antimicrobials together with anticoagulants. The result was complete resolution in 15 patients and in one patient thrombus was surgically removed. The median time was 16 (2–70) days for 50% resolution and 26 (3–93) days for complete resolution. There was a statistically significant (P = .027 and r = 0.5) correlation between the size and the complete resolution time. There was no anticoagulant therapy related major complication. In patients with intracardiac thrombus, selection of anticoagulant therapy may decrease the risk of complications. Surgery is rarely required and thrombolytics are not usually necessary for resolution of thrombus.     

Impact of Three-Dimensional Echocardiography in Complex Congenital Heart Defect Cases: The Surgical View

Other authors have demonstrated the ability of three-dimensional (3D) echocardiography to produce “en face” views of anomalies such as atrioventricular valve disease and atrial and ventricular septal defects. Few data exist about the usefulness of 3D images for more complex congenital heart defects and the surgical impact of this relatively new technology. This study, covering a period of 8 months and including 43 young patients affected by complex congenital heart defects, demonstrated that the routine use of 3D echocardiography is feasible and valuable for some types of cardiac defects. In fact, 3D images have provided more detailed anatomic definition of interrelations between structures in about one-third (15/43) of our cases, yielding new insight into the anatomy analogous to what can be derived from examining a heart specimen. Our surgeons found the 3D images particularly helpful for providing a realistic and almost specimen-like preview of the surgical anatomy that facilitates planning of the surgical program. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Maternal Antibody-Associated Fetal Second-Degree Heart Block and Atrial Flutter: Case Report and Review

Exposure to maternal anti-Ro (SS-A) and anti-La (SS-B) antibodies is a well-described risk factor for the development of fetal atrioventricular (AV) block. The role of maternal fluorinated steroids in the treatment and prevention of antibody-mediated fetal AV block is controversial. Fetal atrial flutter has rarely been described in association with maternal antibodies. This report describes a case of fetal exposure to maternal anti-Ro antibodies with associated second-degree AV block and atrial flutter. Interestingly, the reported patient had 2:1 AV conduction during both normal atrial rates (consistent with AV node conduction disease) and episodes of flutter (consistent with physiologic AV node functionality). The fetus was treated with transplacental digoxin and dexamethasone, which resolved both rhythm disturbances. The case report is followed by a brief discussion of AV block and atrial flutter associated with maternal antibody exposure.     

Patient-Activated Antitachycardia Pacing to Terminate Atrial Tachycardias with 1:1 Atrioventricular Conduction in Congenital Heart Disease

A young girl with complex congenital heart disease and a Fontan procedure presented with frequent episodes of atrial tachycardias with 1:1 atrioventricular conduction. The patient underwent placement of a dual-chamber AT500 pacemaker with epicardial leads but because of the detection algorithm requiring > or = 2:1 AV relation, she received no antitachycardia therapy. Installation of special software AT500 TPARx v2.1 allowed use of patient-activated antitachycardia pacing for 1:1 atrial tachycardias. Since the installation of this application, the patient has successfully pace-terminated her tachycardia multiple times.     

Feasibility and Effectiveness of Three-Dimensional Echocardiography in Diagnosing Congenital Heart Diseases

Three-dimensional echocardiography (3DE), a novel approach employed in detecting congenital heart disease (CHD), has gained popularity since it was made commercially available in 2002. This modality is now accepted as an important diagnostic tool for diagnosing CHD. Advancement in transducer technologies and digital data processing allows the use of 3DE in daily clinical practice. In this review, modes of 3DE data acquisition and storage methods in the echocardiogram’s machine’s hard disk (data processing) are examined. Analysis of the acquired data (cropping or slicing the data set) and methods of illustrating the cropped data set for cardiologists and pediatric cardiovascular surgeons are also discussed. Published literature was searched in PubMed using the keywords “three-dimensional echocardiography”, “congenital heart disease”, “cropping”, and “echoangiogram”. This search produced 100 articles, which were further short-listed to 30 articles. Based on this algorithm, the final selected 30 articles were extensively examined in the current review. The clinical applications of real-time transthoracic 3DE, as well as novel transesophageal 3DE and color flow 3DE data set analyses (echoangiogram) in the routine practice of CHD assessment, are also reviewed. Finally, the limitations 3DE, together with the potential future developments required to improve various techniques of 3DE to make it more readily applicable, are examined.     

Congenital Heart Disease in India: A Status Report

Considering a birth prevalence of congenital heart disease as 9/1000, the estimated number of children born with congenital heart disease in India is more than 200,000 per year. Of these, about one-fifth are likely to have serious defect, requiring an intervention in the first year of life. Currently advanced cardiac care is available to only a minority of such children. A number of cardiac centers have been developed over the last 10 years. However, most are in the private sector, and are not geographically well-distributed. Challenges to pediatric cardiac care include financial constraints, health-seeking behavior of community, and lack of awareness. Government of India is taking a number of steps for improving health of children through its various program and schemes that are likely to benefit children with congenital heart disease, especially those who are vulnerable and marginalized.     

The Adult Patient with Congenital Heart Disease in the Emergency Department

It is well recognized that adult survivors with congenital heart disease are at risk for several cardiac and noncardiac complications that can impact their long-term outcomes. Emergency departments are increasingly being used for the evaluation and management of acute complications in these patients. However, there are several issues that are unique to adults with congenital heart disease related to the complexity of their cardiac anatomy, previous surgical intervention, and residual hemodynamic lesions that can complicate their presentation and management. This review examines the many challenges faced in caring for adults with congenital heart disease presenting to the emergency department, including the issues related to underlying cardiac lesion, access to care, and the evaluation and management of common clinical presentations.