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PubMed2006

Assessment of atrial septal defect area changes during cardiac cycle by live three-dimensional echocardiography.

OBJECTIVES: To investigate the accuracy of measurement of the atrial septal defect (ASD)area and dynamic change by live three-dimensional echocardiography (L3DE). METHODS: L3DE was performed in patients with ASD using a three-dimensional workstation to obtain the en face view of the ASD and measure its area at the peak of P-wave, the peak of R-wave, the initial and the destination point of T-wave, and the period of P-T. Parameters derived from L3DE were compared with intraoperative measurements. RESULTS: The ASD area changed significantly during cardiac cycles (mean change 46.1%, p < 0.0001; range 15.2-72.5%), with the maximal area at endsystole and the minimal area at enddiastole. There was excellent correlation between L3DE and intraoperative measurements for the area of ASD at the peak of P-wave (r = 0.92). There were good correlations between the two methods during the other phases of cardiac cycle (r = 0.81-0.86). CONCLUSIONS: L3DE provides accurate and feasible measurements of the ASD area. Investigation of the dynamic changes during the cardiac cycle may lead to an improved understanding of the hemodynamics of ASD.

Adolescent↗
PubMed2006

Atrial septal defect in adults: the influence of age and haemodynamic parameters on the results of surgical repair.

INTRODUCTION: Indications for closure of atrial septal defect (ASD) are well established in children but still unclear in adult patients. There is also a discrepancy regarding the natural history of the defect and benefits of ASD closure and long-term prognosis. AIM: The aim of the study was to evaluate the outcome of surgical closure of atrial septal defect (ASD) with respect to age and selected haemodynamic parameters. METHODS: The study involved 52 patients with ASD, aged 38.6+/-15 years. All patients had a clinical examination, echocardiography, Holter ECG monitoring and cardiopulmonary exercise test (CPX) prior to and at least one year after the surgery. The results were analysed for the entire group of patients and then for the following subgroups of pts.: aged <40 and ł40 years; with right ventricular systolic blood pressure (RVSP) < or =30 and >30 mmHg; and with shunt volume ratio (Qp/Qs) <2.5 and > or =2.5. RESULTS: After repair of ASD, a significant improvement of the clinical status, as defined by NYHA class, was observed. At the same time, a constant or even increased incidence of arrhythmias was found. Repair of the defect resulted in a significant reduction of the right ventricular diameters in all studied subgroups. Among all evaluated parameters, only age at time of the operation was significantly associated with right ventricular diameter after surgery (b=0.598; p <0.001). Exercise capacity was found to have significantly increased after surgery, regardless of age, RVSP and Qp/Qs, but it remained significantly reduced in patients with pulmonary arterial hypertension compared to subjects with normal RVSP before ASD repair. A significant correlation was shown between peak oxygen consumption and RVSP both before (r=-0.68; p <0.001) and after (r=-0.38; p=0.01) surgery. CONCLUSIONS: Patients benefit from surgical closure of ASD regardless of age and previous RVSP and Qp/Qs. Age at the time of surgery and pulmonary arterial pressure determine long-term results and improvement of echocardiographic and ETT parameters.

Adult↗
PubMed2006

[Simultaneous transcatheter therapy for ventricular septal defect combined with atrial septal defect].

OBJECTIVE: To access the possibility, methods and efficacy of simultaneous transcatheter therapy for ventricular septal defect ( VSD ) combined with atrial septal defect (ASD). METHODS: In 68 patients with VSD, four patients ranging from 3 to 24 years old were combined with ASD. The diameters of perimembranous VSD were 2 approximately 10.5 mm, and the diameters of secundum ASD were 4.6 approximately 7 mm under the echocardiography before the operation. Another 4 patients with VSD occluded by left ventriculography: 3 patients were occluded by VSD occluder first, and then occluded by ASD occuder. The other was only occluded by VSD occluder. RESULTS: All VSD was treated successfully at one time in 4 patients. The diameters of VSD occluder were 4, 8, 10, and 16 mm. ASD was occluded successfully at one time in 3 patients. The diameters of ASD occluder were 8, 10, and 10 mm. The successful rate of the operation was 100%. No complication occurred in the operation and follow-up. CONCLUSION: Simultaneous transcatheter closure for VSD combined with ASD is a safe, feasible and effective therapy.

Abnormalities, Multiple↗
PubMed1990

[Characteristic difference of functional tricuspid regurgitation between acquired valvular heart disease and atrial septal defect].

The characteristic difference in functional tricuspid regurgitation (TR) was studied between acquired valvular heart disease (VHD) and adult atrial septal defect (ASD). Fifty patients of VHD and 20 patients of ASD were subjected to this study. In all patients, the TR volume per beat (VTR) was calculated with our original method using 2-dimensional color Doppler (2-DD) and continuous-wave Doppler (CW) echocardiographies. VTR is obtained by the product of the cross-sectional areas (S) of the base of regurgitant jet in 2-DD and the regurgitant volume of one unit area (Vu) in CW (VTR = 1/3.S.Vu). The preoperative evaluation of TR according to VTR showed a good relationship with the operative findings, and the patients with preoperative VTR greater than or equal to 10 cc were candidates for tricuspid valve repair. In all patients with preoperative VTR less than 10 cc (18 patients of VHD and 10 patients of ASD), VTR decreased postoperatively without any tricuspid valve repair. In patients with VTR = 10-20 cc (19 patients of VHD and 5 of ASD), VTR showed a decrease to below 10 cc after tricuspid annuloplasty (TAP) by Kay's method in 18 patients of VHD and 5 of ASD, and with no tricuspid valve repair in one patient of VHD. In patients with VTR greater than or equal to 20 cc (13 patients of VHD and 5 of ASD), the postoperative VTR diminished to below 10 cc after Kay's TAP in 8 patients of VHD and 5 of ASD, and VTR disappeared after valve replacement (TVR) in 2 patients of VHD.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1990

[Secundum atrial septal defect in two families].

Two families having secundum atrial septal defect (ASD) were reported. In one family, a 31-year-old male and his 60-year-old mother had secundum ASD without PR prolongation. His 38-year-old brother was diagnosed as having ASD. His grandmother, who had died at the age of 51, was suspected of having congenital heart disease. From early childhood she was noticed to have heart murmur. It was suspected that this was a case of familial ASD without PR prolongation, because it was consistent with the dominant trait of the defect. In the other family, a 16-year-old female had secundum ASD and her 18-year-old brother was also suspected of having ASD. Her 25-year-old brother had been operated on for tetralogy of Fallot, and her sister had died of an unknown congenital heart disease. All of these family members had mental retardation. Her 22-year-old brother was suspected of having a congenital heart disease, because of heart murmur from his early childhood. The parents, who were blood relations (cousins), had neither heart disease nor mental retardation. The children of this family were considered to be cases of congenital heart disease with ASD, associated with mental retardation. It was also suspected that the cause of the defect was a deleterious autosomal recessive gene.

Adolescent↗
PubMed1996

Risk factors for adult Still's disease.

OBJECTIVE: To assess risk factors for adult Still's disease (ASD). METHODS: A matched case-control study of 60 patients with ASD and 60 same sex siblings closest in age was conducted. Subjects were recruited from cohorts in Eastern Canada, Pittsburgh, and the Arthritis, Rheumatism, and Aging, Medical Information Systems (ARAMIS). A questionnaire was used to obtain data on demographic characteristics, education, income, occupation, exposure to toxic substances, stress, and medical history. RESULTS: 116 patients with ASD were identified, of which 104 participated. 86 identified same sex siblings, of which 60 replied. When compared to same sex siblings, ASD patients were similar with respect to education and occupation but had a trend to higher median income. There were no significant associations of ASD with smoking, alcohol consumption, individual toxic substances, vaccination, blood transfusion, minor or major surgery, pregnancy, or diet in the year preceding disease onset. There were no significant associations with tonsillectomy or adenoidectomy, appendectomy, asthma, hay fever, allergy shots, or pregnancy at any time preceding the onset of disease. There was a statistically nonsignificant increase in a history of exposure to coal dust [odds ratio (OR) 3.0; 95% confidence interval (CI) 0.30 to 28.84], in allergy preceding the onset of disease (OR 2.67; 95% CI 0.71 to 10.05), and in oral contraceptive use in the year preceding onset (OR 2.00; 95% CI 0.18 to 22.06). Stressful life events (OR 2.56; 95% CI 1.18 to 5.52) in the year preceding onset was significantly associated with increased risk for ASD. This positive association should be treated with caution unless confirmed by a separate study. CONCLUSION: This exploratory study of risk factors for ASD draws attention to stress as a potentially important risk factor, while likely excluding a considerable number of others.

Adult↗
PubMed1997

Transcatheter closure of secundum atrial septal defects using the new self-centering amplatzer septal occluder: initial human experience.

Transcatheter closure of secundum atrial septal defect (ASD) using clamshell or buttoned devices is accompanied by a high incidence of residual shunt. Recently, a new self-centering device, the Amplatzer septal occluder (ASO), has been evaluated in an animal model with very good results. Therefore, our purpose is to report on our initial clinical experience with this device. Thirty patients underwent an attempt at catheter closure of their ASDs at a median age of 6.1 yr (range, 2.9-62.4 yr) and median weight of 22 kg (range, 13-69 kg) using the ASO. The median ASD diameter measured by transesophageal echocardiography (TEE) was 12.5 mm (range, 5-21 mm), and the median ASD balloon stretched diameter was 14 mm (range, 7-19 mm). All patients had right atrial and ventricular volume overload with a mean +/- SD Qp/Qs of 2.3 +/- 0.6. A 7F catheter was used for delivery of the device in all patients. The device was placed correctly in all patients. There was immediate and complete closure (C) in 17/30 patients, 10 patients had trivial residual shunt (TS), and 3 had moderate residual shunt (MS). The median fluoroscopy time was 15 min (range, 8-35 min), and the median total procedure time was 92.5 min (range, 40-135 min). There was no episode of device embolization or any other complication. Follow-up was performed using transthoracic echocardiography (TTE) 1 day, 1 mo, 3 mo, and yearly thereafter. At 1 day, there was C of the ASD in 24/30 patients, 3 had TS, 1 had small shunt (SS), and 2 had MS. At a median follow-up interval of 6 mo, there have been no episodes of endocarditis, thromboembolism, or wire fracture. We conclude that the use of the new ASO is safe and effective in complete closure of secundum ASDs up to a diameter of 21 mm in the majority of patients. Further clinical trials are underway.

Adolescent↗
PubMed2000

Transcatheter occlusion of complex atrial septal defects.

Percutaneous device occlusion of secundum atrial septal defects (ASDs) is becoming an accepted alternative to surgical closure. This method allows us to evaluate patients with complex conditions for treatment. From a total of 70 patients with ASD evaluated for percutaneous closure, we selected for analysis 28 who had complex conditions. The mean age was 36+/-23 yr (range, 4-72). Six had heart failure, and of these six, three had atrial fibrillation. At cardiac catheterization, the pulmonary pressure was 47+/-24 mm Hg, and the QP/QS was 1.7+/-0.4; two patients had bidirectional shunt and systemic pulmonary pressure. Two patients received a buttoned device and 26 an Amplatzer septal occluder. The groups of patients with complex conditions were separated into the following groups. Group I (n = 4) underwent combined treatment of associated anomalies. Two patients had pulmonary stenosis, one had mitral stenosis, and one had an aortic root-left atrium fistula. They were treated in or during with the same procedure by combined transcatheter techniques (balloon valvuloplasty and fistula occlusion) before ASD occlusion. Group II (n = 9) had multiple defects (cribiform or two separate holes). They were treated with a single device in five instances and with two separate devices in four cases. Group III (n = 14) had large (32+/-3 mm) single defects. Nine of them underwent successful implantation using a device 33+/-3 mm in diameter; in the remaining five patients the device was removed because of instability. Group IV (n = 3) had residual defects after previous partial device occlusion. All three defects were successfully occluded with a second device. No movement or interference with the first device was observed. Group V (n = 6) had severe pulmonary hypertension (86+/-16 mm Hg). Immediately after ASD occlusion we observed significant relief in these patients (67+/-14 mm Hg; P<0.01). There were no major complications; all 23 patients with successful implants were discharged without symptoms 2-7 days later; one patient with atrial fibrillation recovered sinus rhythm. The follow-up (8+/-5 mo) Doppler echo study showed complete ASD occlusion in 22 patients and a peak pulmonary pressure of 30+/-14 mm Hg. We conclude that transcatheter occlusion of ASDs is an effective and safe treatment for patients with complex anatomic or physiopathologic conditions, as evaluated by short-term follow-up.

Adolescent↗
PubMed2005

Down syndrome and comorbid autism-spectrum disorder: characterization using the aberrant behavior checklist.

To report on the cognitive and behavioral attributes of 61 children with Down syndrome (DS) and autistic-spectrum disorder (ASD) according to DSM-IV criteria; to determine the utility of the aberrant behavior checklist (ABC) to characterize these subjects for research purposes; and to test the hypothesis that subjects with DS + ASD could be distinguished from their typical DS peers using the ABC. Cross-sectional design. Cases with DS + ASD (N = 61), comparison group of DS + stereotypy movement disorder (SMD) (N = 26) and typical DS controls without behavior problems (N = 44) were ascertained and enrolled sequentially upon presentation to a DS clinic at an academic medical center over a 10-year period from 1991 to 2001. All subjects underwent neurodevelopmental and medical evaluation, and standardized cognitive testing. The parents provided responses to standardized behavioral questionnaires. Cognitive function (IQ) differed markedly across the three groups. The Lethary and Stereotypy subscales of the ABC were highly significant (P < 0.001) in distinguishing the three groups from one another. Within the ASD group differences were apparent by DSM-IV type on the Lethargy subscale, which reached significance, ANOVA (F = 0.002) and t-test (Autism > PDD, P = 0.005; PDD < CDD, P = 0.002). Using a multivariate regression model, the ABC scales alone explained 62% of variance of ASD outcome; addition of demographic variables explained up to 68% of the variance. There is good correlation between DSM-IV criteria for autism and subscales scores on the ABC in subjects with DS. This study demonstrates the feasibility of using the ABC to characterize the neurobehavioral phenotype of a cohort of children with trisomy 21 and ASD for ongoing research purposes.

Adolescent↗
PubMed2005

New technique for device closure of large atrial septal defects.

The objective of this study was to describe a new technique for transcatheter device closure of large atrial septal defects (ASDs) using the Amplatzer septal occluder and our experience with this technique in 14 patients. Transcatheter closure of large (> 25 mm) ASDs is challenging. We have developed a balloon-assisted technique (BAT) to facilitate device closure of large ASDs. The BAT consists of using a balloon catheter to support the left atrial (LA) disk of the Amplatzer septal occluder during device deployment. The balloon support prevents prolapse of the LA disk into the right atrium. Between April 2003 and February 2004, 14 patients with large ASDs (mean age, 25.71 +/- 15.71 years; mean weight, 51.21 +/- 23.78 kg) underwent device closure with the Amplatzer septal occluder using the BAT. The median balloon-stretched diameter of the ASD was 32 (range, 26-40) mm. The median device size used was 33 mm (range, 26-40 mm). All 14 patients had successful deployment of the device using the BAT. The mean follow-up period was 16.5 +/- 11.95 weeks. No major complications were noted during the procedure or on short-term follow-up. The BAT enables predictably successful closure of large ASDs using the Amplatzer septal occluder.

Adolescent↗
PubMed2007

Transcatheter closure of secundum atrial defect in small children.

OBJECTIVE: To assess safety and efficacy of transcatheter atrial septal defect (ASD) closure in small children. BACKGROUND: Percutaneous closure of ASD is a well accepted alternative to surgery. Reported experience in small children remains, however, scarce. METHODS: Fifty-two children underwent percutaneous ASD closure at a weight < or =15 kg, in four Belgian tertiary referral paediatric cardiology centers. Indication for treatment, device implantation rate, procedural details, complication rate, residual shunt on echocardiography, and impact of procedure on symptoms were retrospectively analyzed. RESULTS: Mean age at procedure was 36 months (7-60), mean weight 13 kg (4.7-15). Associated cardiac lesions were seen in 21% of the patients and noncardiac in 32.7%. Most patients (69.2%) were symptomatic. Mean ASD size was 12 mm (range 5-20 mm). A device could be inserted in 49 patients (94%). No major complications occurred. Minor complications occurred in 8 patients (15.4%) including device embolization in 2, successfully treated with transcatheter retrieval and second device insertion. Mean follow-up reached 27 months (0.03-61.2). Residual shunt was absent, trivial, or small in 93% (n = 42) at latest follow-up. Clinical improvement was noted in 91.7% of the symptomatic patients. Minor complications were more frequent in the presence of large ASDs (>15 mm) but not in smaller babies (<10 kg). CONCLUSION: Patients < or =15 kg requiring ASD closure form a special population with high incidence of associated anomalies and medical conditions. For this particular set of patients, percutaneous closure is a valid alternative. Asymptomatic or mildly symptomatic children should be treated at a later age.

Belgium↗
PubMed2005

Balloon sizing and transcatheter closure of acute atrial septal defects guided by magnetic resonance fluoroscopy: assessment and validation in a large animal model.

PURPOSE: To quantitatively assess atrial septal defects (ASDs) with small shunts using MRI followed by transcatheter closure monitored by MR fluoroscopy. MATERIALS AND METHODS: Acute ASDs were created in 14 pigs under x-ray fluoroscopy. Six animals were studied in order to select MR-compatible delivery systems and imaging strategies. ASDs in eight animals were examined with balloon sizing under MR fluoroscopy, flow measurements, and contrast media injections, after which transcatheter closure was performed under MR fluoroscopy. The delivery system was assembled from commercially available materials. RESULTS: The ratio of pulmonary to systemic flow (Qp/Qs) was reduced from 1.23 +/- 0.15 before ASD closure to 1.07 +/- 0.11 after ASD closure (P < 0.001). In two out of eight animals Qp/Qs was close to 1.0 before closure despite the presence of defects >15 mm. The ASDs were measurable with MR balloon sizing in all of the animals. Balloon sizing was identical with MR (16.9 +/- 2.3 mm) and x-ray fluoroscopy (17.1 +/- 1.3 mm). The in-house-assembled delivery system allowed successful placement of closure devices under MR guidance. CONCLUSION: Assessment and closure of small shunts with MR fluoroscopy is feasible. A barrier to the rapid implementation of transcatheter closure in patients is uncertainty about the MR safety of guidewires and device delivery systems.

Acute Disease↗
PubMed2005

An investigation of retrieval inhibition in acute stress disorder.

Acute stress disorder is characterized by dissociative responses that are theorized to result in deficient encoding and retrieval of trauma-related material. This study examined retrieval inhibition using the list method of the directed forgetting paradigm in traumatized individuals with acute stress disorder (ASD; n = 14), no ASD (n = 14), and a nontraumatized control group (n = 15). Participants were presented with a list of intermixed positive, neutral, and trauma-related words. Instructions to forget that list and instead remember a second list were then given, and a new list presented. ASD participants exhibited poorer recall of to-be-forgotten trauma words than the non-ASD and control groups. The ASD group also demonstrated deficient recognition of to-be-remembered and to-be-forgotten positive words. Severity of acute posttraumatic stress response was associated with retrieval impairments. The cumulative findings suggest that ASD is associated with impoverished memory for trauma-related and positive material.

Accidents, Traffic↗
PubMed1989

The incidence and management of the slit ventricle syndrome.

The incidence of slit ventricles of shunt treated hydrocephalic children was evaluated in a follow up study of 141 patients. Slit like ventricles on computer tomography was seen in 75 patients (53%). 52 patients (37%) suffered from clinical symptoms corresponding with overdrainage of cerebrospinal fluid. Those 52 patients with the "Slit Ventricle Syndrome" (SLVS) were treated by changing the valve to one with a higher opening pressure and/or adding an antisiphon device (ASD) to the shunt. 22 patients, initially treated by changing the opening pressure of the valve, needed the ASD later. Altogether 74 episodes of the SLVS were treated. The ASD proved reliable for the management of the SLVS. Normalization of ventricular size occurred in 54% of patients. whereas only in 15% treated without the ASD. Reduction of paroxysmal activity on EEG was seen in 70% of patients treated with the ASD, whereas only in 23% of patients treated without the ASD. Clinical relief of symptoms occurred in every patient, and ventricular catheter obstructions could be avoided, even if the ventricular size remained slit-like on CT.

Adolescent↗
PubMed2004

Bipolar disorder, schizophrenia, and other psychotic disorders in adults with childhood onset AD/HD and/or autism spectrum disorders.

Individuals with attention-deficit/hyperactivity disorder (AD/HD) and autism spectrum disorders (ASD) often display symptoms from other diagnostic categories. Exclusion criteria in the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) and the International Statistical Classification of Diseases and Related Health Problems (ICD-10) impede the use of categorical diagnoses to describe the particular problem constellation in a patient. In this study, we describe the prevalence and patterns of comorbid bipolar and psychotic disorders in 241 consecutively referred adult patients with AD/HD and/or ASD. Thirty per cent of patients with AD/HD had comorbid ASD and 38% of patients with ASD had comorbid AD/HD. Of the subjects with ASD, 7% had bipolar disorder with psychotic features, and 7.8% had schizophrenia or another psychotic disorder. The corresponding figures for the patients with AD/HD were 5.0% and 5.0%, respectively. Current diagnostic criteria have to be revised to acknowledge the comorbidity of bipolar and/or psychotic disorders in AD/HD and ASD.

Adult↗
PubMed2006

Proton magnetic resonance spectroscopy in developmentally delayed young boys with or without autism.

OBJECTIVE: The aim of the present study is to investigate whether brain metabolism of boys with autism spectrum disorder (ASD) is altered compared to boys with a developmental delay without autism if corrected for patient age and developmental level. STUDY DESIGN: 25 boys with ASD (with or without concurrent mental retardation) and 12 boys without ASD with mental retardation or language disorder underwent proton magnetic resonance spectroscopy. All analyses were performed with chronological age and developmental level as independent variables. RESULTS: No metabolic differences were found between boys with ASD and without ASD. CONCLUSIONS: Our findings do not replicate previous reports of differences in NAA, Cho and Cr levels in ASD.

Age Factors↗
PubMed2002

The prevalence of autism spectrum disorders. Recent evidence and future challenges.

BACKGROUND: Until recently best estimate prevalence rates for autism spectrum disorders (ASD) were 0.5/1,000 for autism and 2.0/1,000 for the broader spectrum. Three recent studies have suggested a significantly higher prevalence rate for ASD of 6.0/1,000 (mean 95 % CI = 4.8-8.0). METHOD: Possible determinants of the apparent increase in the prevalence of ASD are outlined. Methodological aspects of the three recent studies are examined. FINDINGS: Increased recognition, the broadening of the diagnostic concept over time and methodological differences across studies may account for most or all of the apparent increase in prevalence, although this cannot be quantified. CONCLUSIONS: Findings from ongoing studies should help confirm or disconfirm the putative rate of 6.0/1,000 for all ASD. The possibility that autism has been over-diagnosed in recent studies needs to be ruled out. Notwithstanding these outstanding questions, it appears likely that the current true prevalence of ASD is considerably greater than previously recognised. This has significant implications for our scientific understanding of ASD and for families and services. Future directions for epidemiological research are outlined.

Adolescent↗
PubMed2004

Early indicators of autism spectrum disorders in the second year of life.

Three groups of 18 children were selected for this study, one group with autism spectrum disorders (ASD), one group with developmental delays in which ASD was ruled out (DD), and one group with typical development (TD), from a pool of 3026 children who were screened with the Communication and Symbolic Behavior Scales Developmental Profile (CSBS DP, Wetherby & Prizant. 2002) Infant-Toddler Checklist under 24 months of age. The CSBS DP Behavior Sample was videotaped on selected children as a second-level evaluation during the second year of life. The Infant-Toddler Checklist had a sensitivity and specificity of 88.9% for this sample of children. Significant group differences were found on the Infant-Toddler Checklist and the Behavior Sample, however, these differences did not distinguish children with ASD and DD with high accuracy. The videotapes of the Behavior Sample were reanalyzed to identify red flags of ASD. Nine red flags differentiated children in the ASD group from both the DD and TD groups and four red flags differentiated children in the ASD Group from the TD group but not the DD group. These 13 red flags were found to discriminate the three groups with a correct classification rate of 94.4%.

Age Factors↗