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PubMed2003

Experience with transcatheter closure of secundum atrial septal defects using the Amplatzer septal occluder: a single centre study in 236 consecutive patients.

AIM: To evaluate the safety and efficacy of transcatheter closure of secundum atrial septal defects (ASD) with the Amplatzer septal occluder. METHODS: 236 consecutive patients with a significant ASD (age 6 months to 46 years, median 5 years; body weight 6.5-79 kg, median 18 kg) were considered for transcatheter closure with the Amplatzer septal occluder; 18 patients with defects that were too large or with a deficient inferior margin were excluded from attempted transcatheter closure after initial transthoracic (4) or transoesophageal echocardiography (14). RESULTS: At cardiac catheterisation, devices were not implanted in 18 patients because the stretched diameter of the ASD was too large (4), the device was unstable (4), compromised the mitral valve (1), or obstructed the upper right pulmonary vein (1); eight patients with additional systemic or pulmonary vein anomalies (5) or a Qp:Qs less than 1.5 (3) were excluded after angiographic and haemodynamic assessment. Thus ASD closure was done successfully in 200 patients (procedure time 25-210 minutes, median 66 minutes; fluoroscopy time 2.5-60 minutes, median 12 minutes), among whom 22 had multiple ASDs (14) or a septal aneurysm (8). The diameter of the devices ranged between 6-34 mm. Severe procedure related complications (retroperitoneal bleeding, air embolism) occurred in two cases. At follow up (33 days to 4.3 years, median 2.3 years) complete closure was documented in 94%, with a trivial residual shunt in 12 patients. CONCLUSIONS: The Amplatzer septal occluder is very efficient and offered interventional ASD closure in 84.7% of our group of consecutive patients, with excellent intermediate results.

Adolescent↗
PubMed2006

Array-based comparative genomic hybridisation identifies high frequency of cryptic chromosomal rearrangements in patients with syndromic autism spectrum disorders.

BACKGROUND: Autism spectrum disorders (ASD) refer to a broader group of neurobiological conditions, pervasive developmental disorders. They are characterised by a symptomatic triad associated with qualitative changes in social interactions, defect in communication abilities, and repetitive and stereotyped interests and activities. ASD is prevalent in 1 to 3 per 1000 people. Despite several arguments for a strong genetic contribution, the molecular basis of a most cases remains unexplained. About 5% of patients with autism have a chromosome abnormality visible with cytogenetic methods. The most frequent are 15q11-q13 duplication, 2q37 and 22q13.3 deletions. Many other chromosomal imbalances have been described. However, most of them remain undetectable using routine karyotype analysis, thus impeding diagnosis and genetic counselling. METHODS AND RESULTS: 29 patients presenting with syndromic ASD were investigated using a DNA microarray constructed from large insert clones spaced at approximately 1 Mb intervals across the genome. Eight clinically relevant rearrangements were identified in 8 (27.5%) patients: six deletions and two duplications. Altered segments ranged in size from 1.4 to 16 Mb (2-19 clones). No recurrent abnormality was identified. CONCLUSION: These results clearly show that array comparative genomic hybridisation should be considered to be an essential aspect of the genetic analysis of patients with syndromic ASD. Moreover, besides their importance for diagnosis and genetic counselling, they may allow the delineation of new contiguous gene syndromes associated with ASD. Finally, the detailed molecular analysis of the rearranged regions may pave the way for the identification of new ASD genes.

Adolescent↗
PubMed2003

Atrial septal defects in pediatric patients: noninvasive sizing with cardiovascular MR imaging.

PURPOSE: To evaluate phase-contrast magnetic resonance (MR) imaging for sizing of secundum atrial septal defects (ASDs) and inflow MR angiography for detection of associated venous anomalies in pediatric patients with inconclusive transthoracic echocardiographic (TTE) results. MATERIALS AND METHODS: Sixty-five children (mean age, 5.4 years +/- 2.7 [SD]) with ASD and inconclusive TTE results underwent phase-contrast MR imaging. Defect size and rim distances measured on MR imaging sections obtained in the ASD plane and from the defect to the venae cavae, aortic root, and atrioventricular valves were compared with transesophageal echocardiographic (TEE) findings (n = 30) during transcatheter closure or surgical measurements (n = 40) by using Bland-Altman analysis. Inflow MR angiography was compared with invasive cine angiocardiography for detection of associated venous anomalies. RESULTS: For ASD size, mean differences were less than 1 mm between MR imaging and TEE measurements (with upper and lower limits of agreement between 2.3 and -3.3 mm) and were between 1.2 and -1.6 mm between MR imaging and surgical measurements (with upper and lower limits of agreement between 4.7 and -5.2 mm). Septal rim measurements at MR imaging agreed fairly well with TEE and surgical results. Septal length was overestimated at MR imaging versus TEE (mean difference, 3.0 mm; upper and lower limits of agreement, between 8.0 and -2.8 mm), but MR imaging septal length measurements agreed with surgical results. Rim distance to coronary sinus was difficult to assess. MR imaging enabled referral of 25 of 30 patients for successful transcatheter closure; five patients were found to have too large defects after balloon sizing. Multiple ASDs and/or associated vascular anomalies in 17 of 65 patients were clearly identified at MR imaging, compared with results of TEE, surgery, and cardiac catheterization. CONCLUSION: In children with ASD and inconclusive TTE results, MR imaging can enable determination of defect size, rim distances to adjacent structures, and venous connections.

Adolescent↗
PubMed1994

Atrial septal defect blunts the impairment of left ventricular function during the Mueller maneuver.

To investigate whether atrial septal defect (ASD) modifies the left ventricular (LV) hemodynamic response to a fall of intrathoracic pressure (Mueller maneuver), we studied 15 patients with an uncomplicated ASD and 16 healthy control subjects. LV function was measured by M-mode and Doppler echocardiography at rest and during the maneuver. Indicator-dilution technique was used to quantify the pulmonary-to-systemic flow ratio. During comparable changes (means +/- SE) of intrathoracic pressure (-33 +/- 2 mmHg in persons with ASD vs. -34 +/- 2 mmHg in those without), LV systolic function and filling diminished in both groups but patients with ASD showed smaller reductions in LV stroke dimension (-0.9 +/- 0.5 vs. -2.5 +/- 0.4 mm; P = 0.016), peak diameter shortening rate (-4 +/- 2 vs. -12 +/- 2 mm/s; P = 0.007), transmitral velocity-time integral (-1.0 +/- 0.3 vs. -2.2 +/- 0.4 cm; P = 0.022), and cardiac output (-6 +/- 3 vs. -18 +/- 3%; P = 0.029). The pulmonary-to-systemic flow ratio increased from 2.1 +/- 0.1 to 2.6 +/- 0.2 in the ASD group (P = 0.014). In conclusion, LV function diminishes significantly in healthy persons during the Mueller maneuver. In patients with ASD, the changes are directionally similar but quantitatively smaller. An interatrial communication mitigates the impairment of LV function after an acute and sustained drop of intrathoracic pressure.

Adult↗
PubMed2007

Association of GABRB3 polymorphisms with autism spectrum disorders in Korean trios.

BACKGROUND/AIMS: Autism spectrum disorders (ASD) are complex neuropsychiatric disorders having a genetic risk factor. The association and linkage study for the gamma-aminobutyric acid type A receptor beta3 subunit gene (GABRB3), located within the chromosome 15q11-q13 autism candidate region, and ASD have been evaluated. The aim of this study was to investigate the association between GABRB3 and ASD in the Korean population. METHODS: The present study was conducted with the detection of four single-nucleotide polymorphisms (rs1426217, rs2081648, rs890317, rs981778) in GABRB3 and association analysis in 104 Korean ASD trios using the transmission disequilibrium test. RESULTS: The transmission disequilibrium test demonstrated that an allele at rs2081648 showed preferential transmission (p = 0.027). One haplotype, including all examined markers in GABRB3, demonstrated significant association (p = 0.043), but the global chi2 test for haplotype transmission did not reveal an association between GABRB3 and ASD (chi2 = 15.516, d.f. = 15). CONCLUSION: Our finding suggested that single-nucleotide polymorphisms in GABRB3 may play a significant role in the genetic predisposition to ASD in the Korean population.

Adolescent↗
PubMed2003

Effect of chronic right atrial stretch on atrial electrical remodeling in patients with an atrial septal defect.

BACKGROUND: Adults with an atrial septal defect (ASD) frequently develop late atrial arrhythmias. We sought to characterize the pattern and persistence of atrial electrical remodeling caused by chronic right atrial (RA) stretch in this group. METHODS AND RESULTS: Thirteen ASD patients without atrial arrhythmia (42+/-10 years old; RA volume, 65+/-16 mL) and 17 normal control subjects (44+/-11 years old; RA volume, 38+/-8 mL) had electrophysiological study to measure (1) atrial effective refractory period (AERP) from the low lateral/high lateral/high septal RA and distal coronary sinus (CS), (2) dispersion of AERP, (3) lateral-RA and CS conduction time during constant pacing, (4) conduction delay across the crista terminalis measuring the number of crista catheter bipoles (0-10) recording discrete double potentials during pacing, (5) corrected sinus node recovery time, and (6) P-wave duration. After ASD closure (8.3+/-5.6 months), follow-up echo studies (n=12) and electrophysiological study (n=4) were performed. The low-lateral AERP, P-wave duration, sinus node recovery time, and extent of conduction delay across the crista terminalis were significantly greater in ASD patients. No differences were found for other measured electrophysiological study parameters. At follow-up, there was incomplete resolution of RA volume (47+/-12 mL; P<0.01 versus before surgery), a trend toward shortening of the AERP at the lateral RA and an increase at the distal CS and high septal RA, but persisting extensive, widely split crista double potentials. CONCLUSIONS: Chronic RA stretch because of ASD causes electrical remodeling with modest increases in RA ERP, conduction delay at the crista terminalis, and sinus node dysfunction. Conduction delay at the crista terminalis persists beyond ASD closure and may contribute to the long-term atrial arrhythmia substrate in this condition.

Adult↗
PubMed1995

Modification of the Fontan procedure. Superior vena cava to left pulmonary artery connection and inferior vena cava to right pulmonary artery connection with adjustable atrial septal defect.

BACKGROUND: A modification of the Fontan procedure with unidirectional cavopulmonary connection is described in which the superior vena cava (SVC) is connected to the left pulmonary artery (PA) and the inferior vena cava (IVC) is connected to the right PA via a lateral tunnel with a snare-controlled, adjustable atrial septal defect (ASD). This allows matching of the SVC and IVC flows with the lung of appropriate size. The obligatory left Glenn shunt provides an adequate arterial oxygen saturation, and the elevation in SVC pressure is well tolerated. The adjustable ASD allows selective decompression of the IVC that maintains cardiac output and reduces fluid accumulation in the serous cavities. METHODS AND RESULTS: Since March 1992, we have performed this procedure in 18 patients. There were 17 children and 1 adult. Median age was 3 years and 9 months (range, 13 months to 36 years). Six patients had been staged with a previous bidirectional Glenn shunt. Preoperative cardiac catheterization revealed a PA pressure of 13 +/- 2 mm Hg and a transpulmonary gradient of 5 +/- 3 mm Hg. Ventricular function was satisfactory in all patients. At the completion of bypass, the pressures in the SVC and IVC were 16 +/- 4 mm Hg and 10 +/- 3 mm Hg, respectively (P < .01). The left atrial pressure was 6.0 +/- 3.0 mm Hg and the arterial O2 saturation on 100% oxygen was 93 +/- 3%. There was one death as a result of intractable atrial arrhythmias. The remaining 17 patients had a mean hospital stay of 9.7 days (6 to 18 days). The length of pleural drainage was 7 +/- 3 days. The ASD was adjusted in 11 patients before discharge. Oxygen saturation at discharge was 85.4 +/- 4%. Nine patients had repeat catheterization. The ASD was completely closed in 6 patients, an average of 2.5 months after surgery (range, 3 weeks to 5 months). After ASD closure, the arterial oxygen saturation was 96 +/- 3%, and the SVC and IVC pressures were both 13 +/- 3 mm Hg. CONCLUSIONS: The Fontan procedure with unidirectional cavopulmonary connection and adjustable ASD has several advantages that may reduce mortality and morbidity for the high-risk Fontan candidate.

Adolescent↗
PubMed1993

Atrial septal defect after balloon mitral valvuloplasty: a transesophageal echocardiographic study.

Fifty patients with rheumatic mitral stenosis aged twelve to thirty-six (twenty +/- six) years were studied by two-dimensional, pulsed and color Doppler echocardiography during, seventy-two hours after, and biweekly for three months after balloon mitral valvuloplasty (BMV). Transesophageal echocardiography (TEE) done immediately after BMV (in the catheterization laboratory) detected a new atrial septal defect (ASD) in 46 (92%) patients. These measured 1 to 2 (mean 1.2 +/- 0.3) mm in diameter. Doppler color flow mapping guided the location of the ASD in most of the cases. A narrow jet of left-to-right shunt could be evaluated by pulsed Doppler studies. Velocity time integral (VTI) of the jet across one cardiac cycle and the diameter of the ASD were used to calculate the left-to-right shunt (shunt = VTI x pi (D/2)2 x heart rate). The estimated shunt was 0.04-0.39 (mean 0.20 +/- 0.10) L/minute. A repeat study at seventy-two hours revealed the defect in 40 (80%) patients. At three months, the defect persisted in only 5 (10%) cases. The mean interval of closure of ASD was 4.6 +/- 2.2 weeks. The authors conclude: (1) ASD occurs commonly after BMV, (2) the septal defect and the resultant left-to-right shunt are insignificant, and (3) ASD disappears in the majority of cases by three months after BMV.

Adolescent↗
PubMed2005

Factors associated with functioning style and coping strategies of families with a child with an autism spectrum disorder.

A survey of parents/caregivers of a child with an autism spectrum disorder (ASD) was conducted to examine the relationship between ASD characteristics, family functioning and coping strategies. Having a child with ASD places considerable stress on the family. Primary caregivers of a child with ASD from a regional and rural area in Victoria, Australia (N = 53) were surveyed concerning their child with ASD, family functioning (adaptability and cohesion), marital satisfaction, self-esteem and coping strategies. Results suggest that these caregivers had healthy self-esteem, although they reported somewhat lower marital happiness, family cohesion and family adaptability than did norm groups. Coping strategies were not significant predictors of these outcome variables. Results highlight the need for support programmes to target family and relationship variables as well as ASD children and their behaviours, in order to sustain the family unit and improve quality of life for parents and caregivers as well as those children.

Adaptation, Psychological↗
PubMed2007

Tics and Tourette syndrome in autism spectrum disorders.

Autism spectrum disorders (ASDs) are more frequently associated with tic disorders than expected by chance. Variable rates of comorbidity have been reported and common genetic and neurobiological factors are probably involved. The aim of this study was to determine the rate of tic disorders in a clinical sample (n = 105) of children and adolescents with ASDs and to describe the clinical characteristics of a group with comorbid ASDs and tics (n = 24). The overlap between tics and other repetitive movements and behaviors in ASDs was carefully assessed. Among individuals with ASDs, 22 percent presented tic disorders: 11 percent with Tourette disorder (TD), and 11 percent with chronic motor tics. All had various degrees of cognitive impairment. An association between the level of mental retardation and tic severity was found. It is concluded that the occurrence of tics in ASDs should not be overlooked and should be carefully evaluated.

Adolescent↗
PubMed2026

Prenatal BPA exposure perturbs RNA-binding protein-mediated splicing regulation and synaptogenesis in the developing cerebellum in a sex-dependent manner.

BACKGROUND: Autism spectrum disorder (ASD) is a pervasive neurodevelopmental condition characterized by social communication deficits, exhibiting a male bias in prevalence. Emerging evidence suggests that prenatal exposure to bisphenol A (BPA) may perturb neurodevelopmental trajectories relevant to ASD. While the cerebellum is increasingly recognized as a brain region implicated in ASD pathophysiology, the impact of gestational BPA exposure on its post-transcriptional alternative splicing machinery remains fundamentally undefined. METHODS: Here, we investigated sex-dependent effects of prenatal BPA exposure on the alternative splicing landscape of the neonatal rat cerebellum. We utilized RNA-seq to profile differential alternative splicing (DAS) events. Ingenuity Pathway Analysis (IPA) was used to predict biological functions and canonical pathways, and to construct the interactome network of DAS genes. To explore candidate upstream regulatory mechanisms, we performed in silico molecular docking and used high-resolution melting (HRM) qRT-PCR to validate selected splicing events. Furthermore, we assessed in vitro cellular phenotypes in primary cerebellar neurons by measuring MTS-based viability and Syn1/Psd95 puncta colocalization. RESULTS: Prenatal BPA exposure was associated with widespread DAS in genes enriched for ASD-relevant pathways in the neonatal rat cerebellum. To our knowledge, this study is the first to report molecular docking analyses predicting favorable interactions between BPA and several candidate RNA-binding proteins (RBPs), including CPEB1, RALYL, HNRNPDL, and ACO1. Our findings support a model in which BPA may perturb RBP-associated splicing regulation, including altered splicing of chromatin regulators such as Ccar1 in males. These molecular and cellular findings were accompanied by sex-stratified differences in neuronal viability and synaptic puncta measurements. BPA exposure was associated with an increased MTS viability signal in male primary cerebellar neurons, together with significant reductions in Psd95 and Syn1 puncta density, whereas female neurons showed significantly increased synaptic puncta colocalization together with reduced viability. CONCLUSIONS: In this study, we propose that prenatal BPA may be relevant to ASD-related neurodevelopmental pathways through sex-dependent changes in RBP-associated alternative splicing, including altered splicing of Ccar1 in males, together with distinct cellular outcomes. Together, these findings identify the developing cerebellum as a sensitive target of prenatal BPA exposure and highlight alternative splicing as a candidate pathway relevant to ASD biology.

Animals↗
PubMed2006

The immune response in autism: a new frontier for autism research.

Autism spectrum disorders (ASD) are part of a broad spectrum of neurodevelopmental disorders known as pervasive developmental disorders, which occur in childhood. They are characterized by impairments in social interaction, verbal and nonverbal communication and the presence of restricted and repetitive stereotyped behaviors. At the present time, the etiology of ASD is largely unknown, but genetic, environmental, immunological, and neurological factors are thought to play a role in the development of ASD. Recently, increasing research has focused on the connections between the immune system and the nervous system, including its possible role in the development of ASD. These neuroimmune interactions begin early during embryogenesis and persist throughout an individual's lifetime, with successful neurodevelopment contingent upon a normal balanced immune response. Immune aberrations consistent with a dysregulated immune response, which so far, have been reported in autistic children, include abnormal or skewed T helper cell type 1 (T(H)1)/T(H)2 cytokine profiles, decreased lymphocyte numbers, decreased T cell mitogen response, and the imbalance of serum immunoglobulin levels. In addition, autism has been linked with autoimmunity and an association with immune-based genes including human leukocyte antigen (HLA)-DRB1 and complement C4 alleles described. There is potential that such aberrant immune activity during vulnerable and critical periods of neurodevelopment could participate in the generation of neurological dysfunction characteristic of ASD. This review will examine the status of the research linking the immune response with ASD.

Animals↗
PubMed2003

Regional brain chemical alterations in young children with autism spectrum disorder.

OBJECTIVE: The authors evaluated regional brain chemistry for evidence of increased neuronal packing density in autism. METHODS: Forty-five 3- to 4-year-old children with autism spectrum disorder (ASD), 13 children with typical development (TD), and 15 children with delayed development (DD) were studied using dual-echo proton echoplanar spectroscopic imaging (32 x 32 matrix-1 cm(3) voxels) to measure brain chemical concentrations and relaxation times. Chemical quantification was corrected for tissue partial volume and relative measures of chemical relaxation (T(2r)) were calculated from the paired echoes. Measures from averaged and individual regions were compared using analysis of variance corrected for multiple comparisons. RESULTS: ASD subjects demonstrated reduced N-acetylaspartate (NAA) (-10%), creatine (Cre) (-8%), and myo-inositol (-13%) concentrations compared to TD controls and prolonged NAA T(2r) relative to TD (7%) and DD (9%) groups. Compared to DD subjects, children with ASD also demonstrated prolonged T(2r) for choline (10%) and Cre (9%). Regional analyses demonstrated subtle patterns of chemical alterations in ASD compared to the TD and DD groups. CONCLUSIONS: Brain chemical abnormalities are present in ASD at 3 to 4 years of age. However, the direction and widespread distribution of these abnormalities do not support hypothesis of diffuse increased neuronal packing density in ASD.

Age Distribution↗
PubMed1985

Effects of pulmonary hemodynamics on lung function in adult patients with atrial septal defect.

To clarify the relationship of pulmonary function with differing severity of atrial septal defect (ASD) and to assess the effects of pulmonary hemodynamics on these functions, we classified 101 patients with adult ASD by their pulmonary arterial mean pressure (PAm) and pulmonary blood flow (Qp), and compared details of pulmonary functions between pairs of groups. Classification was by drawing lines at the PAm of 20 mmHg and the Qp of 10 L/min. Group I was the low pressure-low flow group, Group II the low pressure-high flow group, Group III the high pressure-low flow group, and Group IV the high pressure-high flow group. Vital capacity as the percentage of predicted (%VC) was low in Groups III and IV. There were no significant differences in the ratio of forced expiratory volume in one second to forced vital capacity (FEV1%), flow at 25% forced vital capacity and closing volume as the percentage of predicted (%V25, %CV/VC) between each group. Both maximum midexpiratory flow rate and flow rate at 50% forced vital capacity as the percentage of predicted (%MMEF, %V50) in Group III with the most severe ASD, were significantly low. Pulmonary diffusing capacities for carbon monoxide as the percentage of predicted (%DLco) exceeded 100% in all groups, and tended to decrease with increases of PAm without relation to Qp. Pulmonary dysfunctions in adult ASD were mainly due to restrictive changes accompanied by obstructive change in proportion to the decrease of VC. Peripheral airway obstructions are mild in ASD and these might be caused by lower pressure of left atrium and no existence of pulmonary venous congestion in ASD, even in the most severe group.

Adolescent↗
PubMed2006

Intrusive experiences and hyperarousal in acute stress disorder.

OBJECTIVE: This study investigated the relationship between hyperarousal, intrusions, and dissociative experiences in acute stress disorder (ASD). METHOD: Trauma survivors with ASD (n=30) and without ASD (n=30) completed either a hyperventilation provocation test (HVPT) or a non-hyperventilating control procedure whilst monitoring intrusive experiences. Participants then completed the Physical Reactions Scale and the Peritraumatic Dissociative Experiences Questionnaire. RESULTS: Whereas the hyperventilation procedure resulted in an increase in the number of intrusions for ASD participants, the hyperventilation procedure resulted in a decrease in the number of intrusions for non-ASD participants. Contrary to expectations, there was no differences in dissociative reactions across group or condition. CONCLUSIONS: These findings provide evidence that intrusive phenomenon are directly associated with elevated states of arousal for individuals with ASD.

Adult↗
PubMed1993

Unusual clinical presentations of secundum atrial septal defect.

Atrial septal defect (ASD) is one of the most common congenital cardiac anomalies found in the adult population. Although usually asymptomatic in childhood, ASD will be symptomatic in approximately 75 percent of adults. The most common symptoms include fatigue, dyspnea on exertion, and palpitations. However, the presentation of ASD can be protean. We present four patients with secundum ASD with unusual clinical manifestations. Patient 1 had moderately severe mitral regurgitation. Patient 2 had pulmonary edema with generalized left ventricular impairment. Patient 3 had chest pain typical of angina pectoris. Patient 4 had right-to-left shunt following an orthopedic surgical procedure. These patients had chest radiographs and electrocardiograms typical of secundum ASD, but their presentations were uncommon. In three of four of these patients, dramatic resolution of symptoms followed surgical repair of their ASD.

Adult↗
PubMed2006

The genetics of autism spectrum disorders.

Epidemiological twin studies demonstrate that autism spectrum disorders (ASDs) represent genetic disorders. Subsequent analyses indicate that the causes of ASDs include less common single-gene mutations and chromosomal abnormalities, as well as ASDs caused by multiple interacting genes of weak effect. Genome-wide linkage analysis has identified several susceptibility loci for the ASDs, and positional and functional candidate genes have been identified that appear to represent susceptibility genes for the ASDs. Analysis of additional larger samples and the use of genome-wide association and high-throughput variant detection will lead to the identification of further genes for ASDs.

Autistic Disorder↗
PubMed2000

Factors associated with age at operation for children with congenital heart disease.

OBJECTIVE: Previous studies have shown that children with congenital heart disease (CHD) who live in nonurban areas or who do not have private insurance are at risk for delayed referral to a pediatric cardiologist. However, the effect of these factors on the age at which cardiac surgery is performed has not been evaluated. This study is designed to evaluate the factors that influence the age at which definitive surgical repair is performed. METHODS: Data on hospital discharges for 1995 and 1996 in California were obtained from the Office of Statewide Health Planning and Development database. Children <18 years who underwent surgical repair for atrial septal defect (ASD), ventricular septal defect (VSD), tetralogy of Fallot (TOF), or atrioventricular canal (AVC) were included in the study. Age at surgery was evaluated using type of CHD, gender, race, type of insurance, surgical centers, urban or rural home location, and distance between home and surgical center as independent variables. RESULTS: In 1995-1996, 666 children underwent ASD closure (mean age: 5.1 years; median: 4.0 years), 582 VSD closure (mean age: 2.8; median: 1.1 years), 394 TOF repair (mean age: 1.7; median:.9 years), and 177 AVC repair (mean age: 1.1; median:.6 years). Comparing median and mean age at surgery, we found: AVC<TOF<VSD<ASD (< indicates younger than). A consistent trend for all 4 types of CHD was seen indicating that for median age at operation: private insurance<managed care<Medicaid. Gender or race had no effect on age at operation, although Asians tended to be older at surgery for all 4 types of CHD. There is a significant negative correlation between the case volume of surgical centers and median age at operation for ASD (r = -.37), VSD (r = -.49), TOF (r = -.63), and AVC (r = -.17). In addition, significant positive correlation was found between degree of urbanization of home locations (measured by population density) and median age at operation for ASD (r =.50), VSD (r =.77), and TOF (r =.18). No significant correlation was found between distance to surgical center and age at operation. CONCLUSIONS: Many medical and nonmedical variables play important roles in determining age for definitive repair of CHD in children. Type of insurance, a recognized surrogate for access to care, may play an important role. In addition, centers with higher surgical case volume were more likely to operate at a younger age. Finally, children in urban areas tend to be older at the time of surgery for ASD, VSD, and TOF.

Age Distribution↗