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Improvement in exercise capacity in asymptomatic and mildly symptomatic adults after atrial septal defect percutaneous closure.

BACKGROUND: Controversy exists as to whether secundum atrial septal defects (ASDs) in asymptomatic or mildly symptomatic New York Heart Association (NYHA) class I or II adult patients should be closed. METHODS AND RESULTS: Thirty-seven patients (24 females; mean age 49.4 years, range 19 to 76) with a mean pulmonary to systemic flow ratio (Qp:Qs) of 2.1 (1.2 to 3.4) had a maximal oxygen uptake (VO2max) determination and echocardiographic measurement of right ventricular dimensions before and 6 months after elective percutaneous closure of ASD. At baseline, mean VO2max was 23.5+/-6.4 mL/kg per minute and was higher in the 15 NYHA I patients than in the 22 NYHA II patients (27+/-6.9 versus 20.8+/-4.6 mL/kg per minute; P=0.0015). VO2max increased significantly at 6 months (23.5+/-6.4 to 26.9+/-6.9 mL/kg per minute; P<0.0001). Improvement was as marked in NYHA I (+22%; P<0.0001) as in NYHA II patients (+12%; P<0.0001), in patients with Qp:Qs 1.2 to 2.0 (+16%; P<0.0001) as in those with Qp:Qs >2 (+12%; P<0.0001), and in patients > or =40 years of age (+14%; P<0.0001) as in those <40 years of age (+16%; P<0.0001). Compared with 15 of 37 patients before closure, 35 of 37 patients were in NYHA I at 6 months. Right ventricular dimensions decreased significantly (P<0.0001). CONCLUSIONS: Adult ASD patients significantly increase their functional capacity after percutaneous defect closure. This is observed even in patients classified as asymptomatic, in those with lesser shunts, and in older patients. These findings suggest that ASD closure in an adult population should be considered even in the absence of symptoms.

Adult↗

Intracardiac conduction intervals in children with congenital heart disease: comparison of His bundle studies in 41 normal children and 307 patients with congenital cardiac defects.

His bundle electrograms were recorded in 348 patients aged 2 months to 24 years following routine diagnostic cardiac catheterization. Among 41 children found to be free of anatomic or hemodynamic abnormality the following mean values were obtained: P-H interval, 93.4 +/- 15.3 msec; P-A time, 21.2 +/- 7.1 msec; A-H interval, 72.2 +/- 15.9 msec; and H-V interval, 39.8 +/- 5.2 msec. The remaining 307 patients were analyzed by diagnosis of congenital heart disease. The mean P-H interval was found to be significantly increased in both ostium primum and secundum atrial septal defect (ASD) with A-H prolongation in primum ASD and P-A prolongation in secundum ASD. The mean H-V interval was significantly prolonged in ostium primum ASD and in patients with severe aortic stenosis, aortic insufficiency. and mitral regurgitation. Grouping of the patients physiologically revealed that patients with moderate-to-severe right ventricular volume overload had P-H prolongation, and patients with severe left ventricular volume or pressure overload had H-V prolongation. The clinical implications of these findings are discussed.

Adolescent↗

Noninvasive evaluation of the ratio of pulmonary to systemic flow in atrial septal defect by duplex Doppler echocardiography.

The ratio of pulmonary to systemic flow (Qp/Qs) was noninvasively evaluated by duplex Doppler echocardiography in 22 patients with atrial septal defects (ASDs). Right and left ventricular stroke volumes (RSV, LSV) were determined from the recordings of ejection blood flow velocity and diameter at the level of the pulmonary and aortic orifices in each ventricular outflow tract. The ratio RSV/LSV, determined by the duplex Doppler echocardiography, was compared with Qp/Qs by oximetry. The RSV/LSV for 10 normal subjects was 0.99 +/- 0.05 (mean +/- SD), whereas the RSV/LSV for patients with ASD, 2.26 +/- 0.63, was significantly higher than that for normal subjects (p less than .01). In patients with ASD, a fairly good correlation was found between RSV/LSV and Qp/Qs (r = .92, p less than .01; y = 1.11x - 0.30), and this high correlation was found even in patients with complications such as pulmonary hypertension, mitral and tricuspid regurgitation, Eisenmenger complex, and ventricular septal defect. We also found that semilunar valve regurgitation modified the value of RSV/LSV in accordance with the degree of regurgitation. These findings indicate that, with a few limitations, the Doppler index RSV/LSV is clinically useful in the estimation of the magnitude of the shunt flow in patients with ASD and that the limitations could be overcome by additional Doppler examination.

Adolescent↗

The detection of atrial and ventricular septal defects with electrocardiographically synchronized magnetic resonance imaging.

To evaluate the detectability of cardiac septal defects by electrocardiographically synchronized (ECG-gated) magnetic resonance imaging (MRI), 48 subjects were imaged, including 18 normal and 30 abnormal subjects in whom 22 ventricular septal defects (VSDs) and nine atrial septal defects (ASDs) had been diagnosed angiographically. Two radiologists with ECG-gated cardiac MRI experience read the scans in a blinded fashion, and the results were evaluated by receiver operator characteristic curve analysis. The detectability of VSDs appeared greater than that of ASDs, although statistical significance at the .05 level was not achieved. The reported sensitivity and specificity of echocardiography in the detection of VSDs is comparable to MRI, whereas echocardiography probably is superior to MRI for detection of ASDs. Although MRI is potentially valuable in the diagnosis of various complex congenital cardiac defects, echocardiography is probably superior in the detection of VSDs and ASDs.

Adolescent↗

Acute stress disorder among parents of infants in the neonatal intensive care nursery.

The authors examined the prevalence of acute stress disorder (ASD) in parents of infants hospitalized in the neonatal intensive care unit (NICU). Forty parents were assessed after the birth of their infants. Parents completed self-report measures of ASD, parental stress, family environment, and coping style: 28% of parents developed symptoms of ASD. ASD was associated with female gender, alteration in parental role, family cohesiveness, and emotional restraint. Family environment and parental coping style are significantly associated with the development of trauma symptoms. Results from this study suggest potential interventions to help minimize psychological distress in parents.

Adaptation, Psychological↗

Selective laryngeal adductor denervation-reinnervation: a new surgical treatment for adductor spasmodic dysphonia.

During the past decade, botulinum toxin (Botox) has emerged as the accepted treatment for adductor spasmodic dysphonia (ASD). This therapy, which produces bilateral weakness of the thyroarytenoid muscle, undoubtedly produces physiologic effects that are beneficial to patients with ASD. However, it also has important limitations, including the need for repeated injections, the unpredictable relationship between dosage and response, and the possibility of short-term swallowing and voice problems. In this study, we will report our preliminary experience with a new surgical treatment for ASD. In this new procedure, the adductor branch of the recurrent laryngeal nerve is selectively denervated bilaterally, and its distal nerve stumps are reinnervated with branches of the ansa cervicalis nerve. Each of the patients was followed for at least 12 months; the median follow-up is 36 months. The outcome of the operation in 21 consecutive patients is reported. Nineteen of the 21 patients were judged to have an overall severity of dysphonia that was "absent to mild" following the procedure. Only 1 patient underwent further treatment with Botox postoperatively. The implications of this new procedure for ASD are discussed.

Female↗

Application of three scattering models to characterization of solid tumors in mice.

Two mouse models of mammary cancer (a carcinoma and sarcoma) were examined using quantitative ultrasound (QUS) and three models for ultrasound backscattering. The first model that was examined was the spherical Gaussian model (SGM). The second model was the fluid-filled sphere model (FFSM) and was hypothesized to model scattering from cell nuclei with the cell cytoplasm acting as background. The third model, called the new cell model (NCM), was constructed to model backscattering from cell nuclei and cytoskeleton. The average scatterer diameters (ASDs) were estimated from regions-of-interest (ROIs) inside the tumors using all three models and then compared. The ultrasound analysis bandwidth used in the study was 16 to 27 MHz. QUS images of the tumors utilizing the ASD estimates from the three models were constructed. The ASDs were 30.3 +/- 3.06 microm and 25.2 +/- 4.01 microm with the SGM, 47.3 +/- 7.99 microm and 47.7 +/- 7.01 microm with the FFSM and 41.2 +/- 1.39 microm and 34.4 +/- 5.95 microm with the NCM for the carcinoma and sarcoma, respectively, statistically significant differences between the ASD estimates from the carcinomas and sarcomas were observed using the SGM and NCM but not with the FFSM.

Acoustics↗

Social play and autistic spectrum disorders: a perspective on theory, implications and educational approaches.

The article considers the nature of the presumed social play deficit in autistic spectrum disorders (ASDs). The nature of play and its typical development is outlined and discussed in relation to play development in ASDs. It is suggested that social play is a confluence of two strands of development that are affected in autism: social and emotional development, and the cognitive development of play. It is shown that social play develops in a transactional way and in ASDs initial social difficulties prevent the development of social interaction, with its role in eliciting and enriching spontaneous play. At the same time, cognitive and affective difficulties prevent the play of children with autism developing to the extent of attracting other children and being of a complexity from which social play might develop. This cycle of impoverished play opportunities for children with ASDs may be broken through direct teaching and there are encouraging models of teaching social play with some success.

Autistic Disorder↗

Perceptions of social challenges of adults with autism spectrum disorder.

This study examines perceptions of social challenges by adults with autism spectrum disorder (ASD). The investigators analyzed three separate, regularly scheduled social group meetings attended by a total of 18 adults with ASD where the activity was a discussion of social issues. Participants generated social questions and challenges they had encountered as a result of having autism. The questions were presented to the group for a discussion of potential solutions. Written and audio data were collected and a member check was completed. The data were plumbed for key words and emergent themes to identify major social challenges as viewed by adults with ASD. The emergent themes included relationships at work, developing and maintaining personal relationships, appropriate behaviors around members of the opposite sex, and personal perspectives on having ASD.

Adult↗

Quality of life in high-functioning adults with autism spectrum disorder: The predictive value of disability and support characteristics.

Although the concept of quality of life has increasingly been used in the field of intellectual disabilities over the past three decades, the factors contributing to quality of life of persons with autism spectrum disorder (ASD) have received relatively little attention. In this study, disability and support characteristics associated with variations in the level of quality of life among adults with ASD are identified, using self-report measures. Fifty-eight high-functioning adults with ASD participated in the study. The results of a multiple linear regression analysis reveal that support characteristics are related to quality of life in adults with ASD, whereas disability characteristics are not. The R2 effect size (0.620) is large and significant. The results reinforce the significance of an available supportive social network, the importance of a substantial needs assessment and effective professional support.

Adolescent↗

Prevalence of overweight in children and adolescents with attention deficit hyperactivity disorder and autism spectrum disorders: a chart review.

BACKGROUND: The condition of obesity has become a significant public health problem in the United States. In children and adolescents, the prevalence of overweight has tripled in the last 20 years, with approximately 16.0% of children ages 6-19, and 10.3% of 2-5 year olds being considered overweight. Considerable research is underway to understand obesity in the general pediatric population, however little research is available on the prevalence of obesity in children with developmental disorders. The purpose of our study was to determine the prevalence of overweight among a clinical population of children diagnosed with attention deficit hyperactivity disorder (ADHD) and autism spectrum disorders (ASD). METHODS: Retrospective chart review of 140 charts of children ages 3-18 years seen between 1992 and 2003 at a tertiary care clinic that specializes in the evaluation and treatment of children with developmental, behavioral, and cognitive disorders. Diagnostic, medical, and demographic information was extracted from the charts. Primary diagnoses of either ADHD or ASD were recorded, as was information on race/ethnicity, age, gender, height, and weight. Information was also collected on medications that the child was taking. Body mass index (BMI) was calculated from measures of height and weight recorded in the child's chart. The Center for Disease Control's BMI growth reference was used to determine an age- and gender-specific BMI z-score for the children. RESULTS: The prevalence of at-risk-for-overweight (BMI > 85th%ile) and overweight (BMI > 95th%ile) was 29% and 17.3% respectively in children with ADHD. Although the prevalence appeared highest in the 2-5 year old group (42.9%ile), differences among age groups were not statistically significant. Prevalence did not differ between boys and girls or across age groups (all p > 0.05). For children with ASD, the overall prevalence of at-risk-for-overweight was 35.7% and prevalence of overweight was 19%. CONCLUSION: When compared to an age-matched reference population (NHANES 1999-2002), our estimates indicate that children with ADHD and with ASD have a prevalence of overweight that is similar to children in the general population.

Adolescent↗

Left atrial thrombus causing pulmonary embolism by passing through an atrial septal defect.

A 66-year-old woman admitted with dyspnea on exertion had atrial fibrillation and left ventricular dysfunction. Echocardiography revealed an atrial septal defect (ASD) and a soft, easily deformable thrombus in the dilated left atrium. The atrial mass suddenly disappeared on the 10th day after admission, and contrast-enhanced chest computed tomography and pulmonary blood flow scintigraphy showed that the thrombus had detached from the left atrium, floated into the right atrium through the ASD and caused pulmonary embolism. This is the first documented case of a left atrial thrombus causing pulmonary embolism by passing through an ASD. When an ASD is present, it is important to consider not only paradoxical thromboembolism (from the right to the left atrium), but also pulmonary embolism caused by thromboembolism from the left to the right atrium.

Aged↗

Transcatheter closure of atrial septal defects with the Amplatzer septal occluder--a Japanese clinical trial.

This study reports the results of a Japanese clinical trial of transcatheter closure of atrial septal defects (ASD) using the Amplatzer septal occluder (ASO). Thirty-five patients with secundum ASD underwent transcatheter closure using the ASO at a median age of 12.9 years (range, 3.2-29.2 years) and a median weight of 39.2kg (range, 11.6-65.1 kg). The ASO was successfully implanted in 34 patients. The mean ASD diameter of the 34 patients measured by transesophageal echocardiography was 11.7 +/- 4.2mm (range, 5.0-20.8mm) and the mean balloon stretched diameter was 16.8 +/- 4.2 mm (range, 9-25 mm). The mean ASO size was 16.9 +/- 4.3 mm (range, 9-26mm). Complete closure rate at 1 day and 1 year after closure was 91% and 97%, respectively. One patient developed a transient second-degree atrioventricular block during the implantation procedure. No other complications occurred. Transcatheter closure of ASD using the ASO is effective and safe.

Adolescent↗

Incidence and clinical features of asymptomatic atrial septal defect in school children diagnosed by heart disease screening.

The purpose of this study was to investigate the incidence and clinical features of atrial septal defect (ASD) in school children in Japan who were diagnosed by heart disease screening. From 1989 to 1998, a questionnaire, electrocardiography (ECG) and phonocardiogram were obtained from school children when they entered their first year of elementary school (n=86,142) or junior high school (n=80,632). In this program, 33 asymptomatic ASD patients were newly diagnosed (0.020%). The ECG findings showed incomplete right bundle-branch block (79%), right axis deviation (55%), and right ventricular hypertrophy (9%). An ejection systolic murmur was audible in 30 patients (94%) and mid-diastolic murmur in 10 patients (30%). Thirty patients (90%) showed fixed split of second heart sound. Using echocardiography or catheter observation, 31 patients (94%) were judged to require closure of the ASD. Although the medical care is widely available in Japan, undetected ASD patients were not rare and importantly, most of them required closure of the defect even if they were asymptomatic.

Adolescent↗

Cross-sectional echocardiographic study on the mitral valve prolapse associated with secundum atrial septal defect: pre- and post-operative comparison.

Cross-sectional echocardiography was performed on 77 patients with secundum atrial septal defect (ASD). Mitral valve prolapse (MVP) was recognized in 41 out of the 77 (53.2%); anterior MVP was found in 36 patients, three cases revealed posterior MVP, and two were diagnosed as having both anterior and posterior MVP. Pre- and post-operative study was carried out on 53 cases; anterior MVP decreased in incidence from 28 patients to 17 following closure of ASD, whereas posterior MVP remained unchanged, as did both anterior and posterior MVP. The left ventricular distortion ratio (LVDR) was measured in the short axis view in 45 cases. After the repair of ASD, the LVDR decreased both in end-systole and end-diastole (p less than 0.01), that is, the left ventricle became more circular in configuration. Pre- and post-operatively, MVP patients revealed a significantly greater LVDR than those without it in end-systole, and post-operatively in end-diastole as well (p less than 0.01). Prior to operation, the short axis area of the left ventricle (LVSAA) was statistically larger in patients with MVP than without it in end-diastole (p less than 0.05). However, there existed no relationship between the presence of MVP and the pulmonic to systemic flow ratio, right ventricular dimension index, right ventricular systolic pressure and size of ASD at the time of operation.

Adolescent↗

Long-term management of bradyarrhythmias following open heart surgery: surgical A-V block and sick sinus syndrome after surgery for secundum atrial septal defects treated with permanent cardiac pacing.

Postoperative clinical findings from 25 patients with surgical A-V block and 12 with SSS following surgery for ASD who received permanent pacemakers were analyzed in order to consider the long-term management of postoperative bradyarrhythmias. Surgical A-V block: Episodes of Adams-Stokes were observed in 11/25 patients before pacemaker implantation, and in 6 of these (55%) the onset of episodes occurred more than a year after open heart surgery. Of 8 cases for which ECG's during Adams-Stokes were available, 2 had bifascicular or trifascicular block which progressed to complete A-V block below the His bundle. The 6 others (75%) had ventricular tachycardia or ventricular flutter-fibrillation during Adams-Stokes. 2 of these had blocks above the His bundle. 4/25 had improvement of complete A-V block within a month. Following pacemaker implantation, 3 died of heart failure and 3 of sudden death. 5 year survival was 70%. Therefore, surgical A-V block requires careful long-term management, and the presence of ventricular tachyarrhythmias as well as the location of block are important predictors of patient risk. SSS after surgery for ASD: 10/12 patients had Adams-Stokes, of which 6 (60%) had initial onset over 5 years after surgery, and 9 had paroxysmal atrial flutter and fibrillation which coincided with the onset of Adams-Stokes. 3 of 7 patients (42%) for whom preoperative ECG's were available had sinus bradycardia. Thus, SSS after ASD surgery may be preceded by preoperative deterioration of sinus node function and succeeded by late onset of Adams-Stokes, necessitating pre- and postoperative assessment of sinus node function. The presence of atrial tachyarrhythmias also serves as an important indicator of the severity of SSS after ASD surgery. The onset of Adams-Stokes varied by patient over a wide range of time, emphasizing the need for careful long-term follow-up. Clinical symptoms and prognosis were affected by tachyarrhythmias as well as the severity of the bradycardia. Therefore, the presence of ventricular/atrial tachyarrhythmias is an important factor in the long-term management of postoperative bradyarrhythmias.

Adams-Stokes Syndrome↗

Estimation of the right ventricular volume overload in children with atrial septal defect by body surface mapping.

To investigate the possibility of estimating the right ventricular volume overload in atrial septal defect (ASD) by body surface mapping (BSM), 45 children with ASD were included in the present study. The breakthrough minimum (BTM) appeared later and more leftward in ASD subjects than in the normal controls. Neither the length of the delay in the appearance of the BTM nor the extent of its leftward deviation was significantly correlated with the pulmonary and systemic flow volume ratio (Qp/Qs), obtained by cardiac catheterization. Therefore neither could be used as the parameter of the right ventricular volume overload. sigma R, Rmax (D3 + D4) + magnitude of S max (H4 + H5) and "T dep", the sum of the absolute values of the departure indices (DIs) in the G4, G5, H4 and H5 leads, and the DIs in the J5, J6, K5, and K6 leads in T departure Map, correlated with the Qp/Qs significantly (r = 0.62, 0.68, 0.69, respectively). Accordingly, they might be useful for estimating the right ventricular volume overload. The subjects were divided into 3 groups according to their Qp/Qs. Discriminant analysis between these 3 groups was then performed, using Rmax (D3), magnitude of S max (H4), and T dep as the variables. The accuracy of this discrimination was 82.2%. The results suggest the possibility of estimating the severity of ASD by BSM.

Adolescent↗

Familial atrial septal defect and atrioventricular conduction disturbance associated with a point mutation in the cardiac homeobox gene CSX/NKX2-5 in a Japanese patient.

Atrial septal defect (ASD) is the most common form of congenital cardiac defect in humans. Recently, point mutations in the cardiac homeobox gene CSX/NKX2-5 have been reported to cause the autosomal dominant form of familial ASD. Notably, all the affected patients exhibit atrioventricular conduction disturbance and some of them died suddenly. The first case of familial ASD with a mutation of the CSX/NKX2-5 gene in a Japanese patient is reported here. Identification of CSX/NKX2-5 mutations in ASD patients would be very important because the existence of such mutations may predict sudden cardiac death.

Heart Block↗