Echocardiographic Guidance of Catheter-Based Treatments of Atrial Septal Defect

The availability of echocardiographic imaging to the catheterization laboratory has been recognized as an essential component of every congenital heart program for almost 30 years. The imaging data that are uniquely available using this technique may be used to plan the catheterization procedure; to add insights regarding valve anatomy and chordal insertions and myocardial performance; to assist in the identification of pericardial effusions; and to provide additional guidance for the performance of pericardiocentesis.     

Repetitive Delay in Diagnosis of Ventricular Septal Defect

Objective

Although ventricular septal defect (VSD) is the most common congenital heart disease, it is usually diagnosed late. The presentation of the disease is variable; sometimes it is so quiet and silent that might even improve and heal spontaneously, and in some certain cases if the appropriate, on time and early treatment is not done, this would lead to irreparable complications and mortality even in the early life period. This study reviews the diagnostic process, treatment and follow-up of the patients. It is hoped that the results of the present study be used to improve the patients'' condition.

Methods

This was a cross-sectional study done on 145 patients with VSD during 54 months in Isfahan. The disease was identified through color Doppler echocardiogram, cardiac catheterization and angiography if necessary. The required data were collected at the time of definite diagnosis.

Findings

Mean age at initial and definite diagnosis of the disease was 17 months and 44 months, respectively. Heart murmur led to initial diagnosis in 85% of the cases. In 27.5% VSD was associated with other cardiac anomalies. Pulmonary artery hypertension existed in 16.5% of the cases. Fifty nine surgeries were performed on 40 patients.

Conclusion

In routine physical examination of the infants, the probability of heart disease should be considered; conducting echocardiogram in suspected cases would lead to early diagnosis and eventually timely treatment. Appropriate follow-up of the patients will provide optimal care and treatment at proper time.     

Simultaneous Transcatheter Closure of Ventricular Septal Defect and Pulmonary Valvuloplasty

Background

Transcatheter balloon valvuloplasty has been the accepted first line treatment for congenital pulmonic stenosis (PS) in children. Transcatheter closure of perimembranous ventricular septal defect (VSD) with Amplatzer VSD occluder is an alternative to surgical repair.

Case Presentation

A 12 year old boy presented with history of exertional dyspnea and atypical chest pain. Physical findings were suggestive of severe pulmonic stenosis. Transthoracic echocardiography, right and left ventricular angiography showed medium to large sized perimembranous VSD and severe valvar pulmonary stenosis. Transcatheter closure of VSD was done first so as to avoid large left to right shunt across VSD after balloon pulmonary valvuplasty.

Conclusion

We demonstrated the feasibility and success in treating combined ventricular septal defect and severe pulmonary valve stenosis with transcatheter interventional procedure in the same session.     

Regression of Right Ventricular Pressure in Ventricular Septal Defect in Infancy: A Longitudinal Color-Flow Doppler Echocardiographic Study

To determine the course of right ventricular pressure (RVP) in patients with isolated ventricular septal defect (VSD) and factors influencing it, unselected 148 infants were followed-up longitudinally with color-Doppler echocardiography from a median age of 1 month for 201 patient-years. The patients were divided into three groups by absolute echographic size of VSD: group I, ≤4.0 mm; group II, >4 to ≤7 mm; group III, >7 mm. Sixty percent belonged to group I. Muscular defects dominated in group I, perimembranous defects dominated in group II, and those with outlet extensions dominated in group III. Peak systolic RVP was obtained by Doppler-estimated difference between systolic brachial artery and peak gradient across the VSD. Initial RVP ranged between 15 and 95 mmHg and increased in parallel to the size of defect. According to the regression equations RVP decreased in general by 0.17 mmHg per month. This correlated significantly with the size of the defect. In group I, the rate of decrease was very fast and is best expressed by a log function of time (r=−0.67, r ²= 0.45). In groups II and III the rate of decrease was less steep and had a greater variability. RVP normalized in 100% in those of group I and in 90% of group II, at median ages of 0.17 and 0.33 years, respectively. Median Q _p:Q _s values were 1.5, 2.2, and 3.0 in groups I–III, respectively. The outcome depended on the size of VSD. Spontaneous closure was observed in 51% of group I, 10% of group II, and none of group III. The rate was higher in muscular defects. Congestive heart failure was present in 53% and 100% in groups II and III, respectively. Death rate was 2.03%, all in patients with large defects. It is concluded that the temporal course of RVP with time can be estimated fairly well by the regression equation presented in relation to the initial size of the VSD.     

Transcatheter Atrial Septal Defect Closure under Transthorasic Echocardiography in Children

Objective

Atrial septal defect (ASD) device closure is routinely done under the guide of transesophageal or intracardiac echocardiography which are expensive techniques and not easily affordable in developing countries.

Methods

Using metallic devices, we attempted 32 ASD device closures under transthoracic echocardiography.

Findings

Of those, 30 procedures were successful (94 %). In two patients with relatively large ASD we encountered difficulty in positioning the device. These patients were referred for surgical closure.

Conclusion

ASD device closure can be carried out successfully in most patients under transthoracic echocardiography in situations where transesophageal or intravenous echocardiographies are not available or affordable.     

Evaluation of Atrial, Ventricular and Atrioventricular Septal Defects by Cine Magnetic Resonance Imaging

Cine magnetic resonance imaging (MRI) was performed on 20 patients (mean age: 5.3 ± 4.4 years) with atrial, ventricular, or atrioventricular septal defects for evaluation of cardiac structure and blood flow. Prior to cine MRI, electrocardiographically gated MRI using multislice acquisition was performed on all patients to localize optimal slice location. Cine-MRI was obtained with a 30° flip angle, 15 msec echo time, and 30 msec pulse repetition time, on a 256×256 or 128×128 acquisition matrix. Abnormalities of cardiac structure were well defined in all patients by gated cardiac imaging. In 18 of the 20 patients, cine-MRI was able to detect shunt flow, visualized as a low intensity signal in comparison with the surrounding blood flow. Cine-MRI can provide not only accurate anatomy of cardiac structures but functional assessment of the cardiac chamber, wall topology and flow relations. Cine-MRI will become an important noninvasive technique for assessment of anatomy and physiology in congenital heart disease.     

Sudden Death Due to Ventricular Septal Defect

Ventricular septal defects (VSD) are usually considered non-life-threatening, usually closing spontaneously or causing symptoms of congestive heart failure, which can be surgically treated in time to save the patient's life. Despite the usually benign clinical course of VSD, serious arrhythmias occur in 16–31% of patients. Sudden death accounted for one-third of all deaths in a series of medically managed patients and occurred in 4.2% of patients in a study of VSD and arrhythmias. Cardiac hypertrophy is the common denominator in all cases reported in detail of VSD-associated sudden death. We have encountered four cases of sudden unexpected death from VSD in infants ranging in age from 1 week to 3 and ½ months. In each case there was cardiomegaly and in one case there was pulmonary arteriolar medial thickening, with extension of smooth muscle into small intralobular vessels. In half of our cases the attending physician was sued for malpractice. We believe that VSD in infants and young children are potentially life-threatening malformations which warrant careful clinical follow-up.     

Atrial Septal Defect: Spectrum of Care

Atrial septal defect (ASD) is a common congenital heart defect. Variability in management of this lesion exists among clinicians. A review of the literature reveals that there is lack of standard guidelines for the evaluation and management of patients with different types of ASDs. This survey-based study was conducted to test the uniformity of diagnostic and therapeutic approach to management of children with secundum, sinus venosus, and primum ASDs. Survey questionnaires were prepared to include questions regarding follow-up, diagnosis, and therapeutic intervention of different types and sizes of ASDs. Questions addressed follow-up visitations, type and frequency of investigative studies, pharmacological therapy, and choice of repair method. Surveys were sent out to all pediatric cardiology academic programs in the United States (n=48) and randomly selected international programs from Europe, Asia, and Australia (n=19). A total of 23 programs (34%) responded to the survey (15 from the United States and 8 internationally). A separate questionnaire was prepared for secundum, primum, and sinus venosus ASD. In each questionnaire, lesion types were subdivided into small, moderate, and large defect sizes to address differences of management approaches to each defect type and size. Results indicate that in secundum ASD, most participants use size of the defect and/or evidence of right-sided volume overload as criteria for defining small, moderate, and large defects. Frequency of follow-up does not vary with the type of lesion but is more frequent with larger defects. Most participants see patients with small defects at intervals of 6 months to 1 year and those with large defects at 3- to 6-month intervals. Age of patient and presence of symptoms determined the frequency of follow-up across all defects. Echocardiography was the most frequently used investigative modality in all defect sizes and types during follow-up visits (used by >80% for follow-up), followed by electrocardiography (ECG). There is a striking preference for the use of pharmacological therapy in primum ASD compared with secundum and sinus venosus ASD. The timing of repair was mainly dependent on patient age and symptomatology in different defects, with the presence of associated anomalies contributing to that in primum and sinus venosus ASD. Most participants use percutaneous approach to close secundum ASD (either as a first choice or as one of two choices depending on the presence of certain features). Before repair, participants use MRI or cardiac catheterization to fully evaluate a secundum ASD if it is large. These investigative modalities are not commonly used in primum and sinus venosus ASD. There is agreement on postoperative follow-up in different types of defects, with most participants continuing follow-up indefinitely, especially in larger defects.     

二维应变和应变率评估房间隔缺损患儿右心室功能的价值

目的 探讨超声右心室长轴整体收缩期应变(GLS)和应变率(GLSR)及右心室游离壁基底段应变(Sb)和中段应变(Sm)在房间隔缺损(ASD)患儿中的变化,评估二维应变和应变率对ASD患儿右心室功能的诊断价值.方法　选择ASD患儿32例.分为单纯ASD组(n=20),ASD+肺动脉高压(PAH)组(n=12).同时选择20例健康体检者作为健康对照组.保存二维超声心动图图像,导入GE VIVID 7 Echopac工作站进行脱机分析;应用二维应变分析软件,追踪右心室各节段的运动,得出右心室各节段的整体收缩期应变和应变率曲线.应用SPSS 13.0软件进行统计学分析.结果 单纯ASD组患儿GLS、GLSR、Sb、Sm水平较健康对照组显著增加(Pa＜0.05);ASD+PAH组GLS、GLSR、Sb及Sm水平均显著低于单纯ASD组(Pa＜0.05).ASD+PAH组GLS、Sb、GLSR均明显低于健康对照组,差异均有统计学意义(Pa＜0.05),Sm在健康对照组与ASD+PAH组间差异无统计学意义(P＞0.05).结论　应变和应变率可以很好地评估ASD患儿右心室功能.右心室心肌的应变和应变率受容量和压力负荷的双重影响.     

Progression to Late Complete Atrioventricular Block Following Amplatzer Device Closure of Atrial Septal Defect in a Child

Atrial septal defect (ASD) closure by interventional catheterization techniques has been widely accepted and is considered a standard treatment for this congenital heart defect. We report the case of a 6-year-old girl who presented with progression of first-degree atrio-ventricular (AV) block to symptomatic, complete heart block after ASD closure with an Amplatzer septal occluder (ASO). Although she received steroids immediately after the procedure when second-degree AV block was seen, her AV conduction slowly deteriorated over 4 years, requiring permanent pacemaker implantation. We can only speculate that the injury to the conduction system was the result of persistent trauma, ischemia, or progressive scarring caused by the ASO on the AV nodal region. It is uncertain if early device removal would have prevented this complication. This case highlights the importance of cardiac rhythm monitoring following ASO implantation for ASD closure on long-term follow-up.     

Flow Velocity Patterns across Atrial Septal Defects Recorded with Doppler Echocardiography

ABSTRACT. During a two-year period pulsed Doppler combined with two-dimensional echocardiography was used in evaluating patients for atrial septal defects. Blood flow velocities were recorded with pulsed Doppler in the right atrium along the atrial septum, across the atrioventricular and semilunar valves and when a shunt was detected also on the left side of the phial septum. The typical flow velocity pattern most often seen is described and is related to the differences in pressure between the two atria during the cardiac cycle. Deviations from this velocity pattern are described and their hemodynamic significances discussed. Diagnosis and assessment of pulmonary hypertension from tricuspid and pulmonary flow velocities are described.     

Two-Dimensional Pulsed Doppler Echocardiographic Technique for Estimating Pulmonary to Systemic Blood Flow Ratio in Children with Atrial Septal Defect and Patent Ductus Arteriosus

A two-dimensional pulsed Doppler echocardiographic method was evaluated for quantifying the pulmonary (Qp) to systemic (Qs) blood flow ratio. Twenty six patients were studied, 15 with atrial septal defect (ASD) and 11 with patent ductus arteriosus (PDA). An apical fourchamber view was used to measure the maximal Doppler flow velocities in the right and left ventricular inflow regions (RVIR and LVIR) and the maximal diameters of the tricuspid and mitral anuli at diastole. The two-dimensional pulsed Doppler pulmonary to systemic blood flow ratio (Qp/Qs) estimation involved calculating the area of the Doppler flow velocity signal along the time scale multiplied by the diameter of either the tricuspid or mitral anulus as measured on the apical fourchamber view. In ASD patients, Qp was measured at RVIR and Qs at LVIR. In PDA patients, Qp was calculated at LVIR and Qs at RVIR. Twenty-six healthy children comprised the control group. The areas of the Doppler flow velocity signals along the time scales of RVIR and LVIR were also quantified and the diameters of the tricuspid and mitral anuli were measured. In the control group, the linear correlation of the trans-mitral M(d)*M(a) to trans-tricuspid T(d)*T(a) value was r=0.909, n=26. The correlations between Doppler Qp/Qs ratio estimates and those obtained at cardiac catheterization were r=0.902, n=15 in ASD and r=0.967, n=l l in PDA. These results suggest a clinical application of this Doppler echocardiographic method in the noninvasive estimation of the cardiac shunt in ASD and PDA.     

Glucose Metabolism and Insulin Secretion in Infants with Symptomatic Ventricular Septal Defect

ABSTRACT. Nineteen infants with symptomatic ventricular septal defect (VSD) were examined on, altogether, 26 occasions, when each was given an intravenous glucose tolerance test (IVGTT); concentrations of insulin- and C-peptide in plasma were determined. Comparisons were made with 14 healthy infants of the same age. The VSD infants were growth retarded with lower weight/age and length/age ratios. Glucose tolerance as indicated by glucose fasting levels and response to intravenous glucose tolerance test, including glucose disappearance rate, did not differ between the two groups. In response to the glucose load, insulin in plasma was significantly less in VSD infants. In response to the IVGTT, insulin secretion rate calculated from C-peptide levels in plasma was significantly elevated in the VSD group. We conclude that compared to healthy infants, those with symptomatic VSD have normal glucose tolerance, increased secretion rate of insulin, but decreased levels of circulating insulin in response to an intravenous glucose load. We suggest this is so because binding of insulin to peripheral receptors and/or insulin extraction in the liver somehow increases.     

The Natural Course of Isolated Ventricular Septal Defect During Adolescence

Serial changes in patients with isolated VSD during adolescence have not previously been investigated. Hemodynamic status, diameter of the defect, and growth were studied yearly in 106 children with VSD. The mean duration of the follow-up was 13.16 years and ranged in 80% of subjects from 7 to 19 years (1395 patient years). The mean ages at pre- and postpuberty were 8.62 and 16.67, respectively. The presented longitudinal study, in which losses due to death and operation were minimal (4%), ideally reflected the natural history of VSD. Although weight showed retardation during prepuberty, this lag was caught up by the end of adolescence. Stature showed no retardation in pre- and postpuberty. Cardiothoracic ratio decreased significantly from a mean of 0.48 to 0.44 and showed normal variation. Although the mean defect diameter at prepuberty was 5.33 mm, this decreased to 2.7 postpubertally. The individual decrease (1.7 ± 2.34 mm) was significant (t= 5.349, p < 1/10⁵). The defect closed spontaneously in 24 (22.6%). In the 75 patients without pulmonary hypertension and with mild left-to-right shunting, 52 remained in the same class and spontaneous closure was observed in 23. In the 24 patients with moderate to severe left-to-right shunt, this decreased in 23 and only one remained stable. The 2 patients (1.9%) with Eisenmenger syndrome remained stable and 1 died. Aortic regurgitution developed in 10 patients (9.4%); however, this was of mild degree in most of them. No infective endocarditis was observed. It is concluded that patients with VSD should be followed closely through adolescence, because the diameter of the defect, as well as left-to-right shunting, can decrease, and it is concluded that the spontaneous closure of the defect is to be expected in a considerable 23%, and aortic prolapse or mild regurgitation may develop in approximately 10%.     

Histopathological Workup of an Amplatzer Atrial Septal Defect Occluder After Surgical Removal

We present results of the histopathological work-up of an atrial septal defect occluder that was explanted 15 months after interventional implantation due to a significant residual shunt. Complete endothelialization of the surface and a mild inflammatory reaction was demonstrated.     

Rapid Evolution of Right Ventricular Cardiomyopathy After Surgical Closure of a Secundum Atrial Septal Defect

Rapidly developing diffuse right ventricular cardiomyopathy presenting with right heart failure in a 9-year-old boy within 2 months after surgical closure of a secundum atrial septal defect is reported. The child was treated by palliative surgery with partial exclusion of the right ventricle by bidirectional Glenn shunt.     

Community Use of the Amplatzer Atrial Septal Defect Occluder: Results of the Multicenter MAGIC Atrial Septal Defect Study

The objective of this study was to determine the initial safety and results of unrestricted multi-institution routine community use of the Amplatzer Septal Occluder (ASO) for atrial septal defect (ASD) closure. A multicenter, nonrandomized prospective study was performed in 13 pediatric cardiology centers from November 2004 to September 2007. Data were collected at the time of cardiac catheterization and 1 day postimplant. Four hundred seventy-eight patients underwent cardiac catheterization for ASO device closure of an ASD. The median age was 6 years (range, <1–83 years), and the mean weight was 37.2 kg (range, 2.6–148 kg). Procedural success was 96% (458/478 patients), with deficient rims being the major single reason for failed implantation (9/20). Major and minor complication rates were 1.1% and 4.8%, respectively, and were not different between simple and complex ASD groups. Success at 24 h was 99.4% (333/335) in the simple ASD group and 100% (120/120) in the complex ASD group. The presence of large defects, the presence of multiple defects, the use of multiple devices, and a weight <8 kg were significantly associated with a residual shunt (small to moderate) at 24 h. In conclusion, the ASO device in routine clinical practice for simple and complex ASD closure has an immediate safety and effectiveness profile equal to that reported in the initial pivotal FDA trial for simple ASDs. Based on the evolution in care posed by the ASO and the lack of consensus on patient selection in complex ASDs, this study points out the need to redefine the optimal patient and possibly broaden the indications for device closure of ASDs.     

经食管超声心动图对儿童室间隔缺损的围术期评价

目的探讨运用经食管超声心动图(TEE)对儿童室间隔缺损(VSD)进行围术期的作用。方法 VSD患儿205例,年龄3个月～17岁(平均3.4岁),术前均由经胸超声心动图(TTE)作出诊断。体外循环前后分别行TEE探查,比较TFE及TTE 变化。结果 1．术前TEE和TTE对VSD位置的诊断准确率分别为99.0％和97．6％,膜部瘤检出率分别为93．8％和43．8％, VSD直径测量与手术探查结果相关系数分别为0．958和0．898;2．术后TFE发现残余分流38例(18．5％),其中9例(4.4％)立即再次修补。结论术前TEE可准确诊断VSD;术后TEE可发现残余分流,立即干预,以免再次开胸。     

A Rare Case of Aortic Coarctation and Ventricular Septal Defect Combined with Alveolar Capillary Dysplasia

We report a rare case of alveolar capillary dysplasia (ACD) associated with severe aortic coarctation, hypoplastic aortic arch, and mild hypoplasia of the left ventricle. After successful coarctation repair, pulmonary hypertension persisted, and despite only minor anomalies on the x-ray, chest computed tomography (CT) revealed the presence of primary lung disease. Review of the literature suggests that ACD may be associated with left heart obstructions. Chest CT is applicable to diagnose ACD precociously.