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Atrial septal defect: a diagnostic approach.

A simple objective screening method for diagnosis of the atrial septal defect (ASD) is needed. Acoustic signals were collected from 61 children with ASD and 60 with a physiological murmur. The second heart sound (S2) and the spectrum of systolic murmur were analysed. A statistical model was designed using stepwise logistic regression analysis. Significant variables distinguishing pathological form normal findings were the interval between the first heart sound and the beginning of systolic murmur or the respiratory variation of S2, and the frequency of the murmur at its maximum intensity. The area under the ROC curve was 0.922; indicating very good fit of the model and the confidence interval was 0.872-0.971. The sensitivity of the model was 91% and the specificity 73%. The analysis of acoustic findings from the heart is a valuable tool in diagnosing ASD. The next step will be automating this process.

Adolescent↗

A Systematic Review of Help-Seeking Barriers for Racial-Ethnic Minority Caregivers Accessing Autism Diagnostic and Intervention Services.

Caregivers play an essential role in early help-seeking and intervention for children with Autism Spectrum Disorder (ASD). Caregivers, therefore, provide a crucial role in helping to address the racial and ethnic disparity identified in accessing ASD intervention and diagnostic services (Bejarano-Martín et al., Journal of Autism and Developmental Disorders 50(9), 3380-3394, 2020). Unfortunately, racial-ethnic minority caregivers of children with autism (CCA) are less likely to contact a physician or healthcare professionals about their concerns and more likely to delay their contact to have their child evaluated (Zeleke et al., Journal of Autism and Developmental Disorders 49(10), 4320-4331, 2019). However, little evidence exists to explain why such a gap exists in the help-seeking behaviors between White and racial-ethnic minority CCA. To address this knowledge gap, we conducted a systematic literature review to identify articles that have studied barriers in help-seeking for racial-ethnic minority CCA. A broad literature search across four databases was conducted (i.e., PubMed, PsycINFO, Education Resources Information Center, and Child Development and Adolescent Studies). The coding team identified 17 articles on help-seeking barriers for racial-ethnic minority CCA. A thematic analysis was used to narratively synthesize the help-seeking barriers identified across these 17 studies. Four themes emerged from our findings: logistical barriers, provider competence, ASD literacy, and cultural stigma. We also provided clinical recommendations for healthcare providers working with families with racial-ethnic minority CCA.

Humans↗

Value of respiratory variations of right ventricular dimension in the identification of small atrial septal defects (secundum type) not requiring surgery: an echocardiographic study.

In search of reliable criteria that could help differentiate insignificant atrial septal defects (ASDs) from those with a large shunt, M-mode echocardiograms of three groups of patients were studied retrospectively: group I = 10 normal children (mean age 5.7 years); group II = 10 patients (mean age 7 years) with small ASD in whom the decision was taken not to proceed to surgical closure, based on hemodynamic and angiographic criteria; and group III = 15 patients (mean age 7 years) with an "operable" shunt, who underwent corrective surgery. The results showed that right ventricular end-diastolic dimensions during expiration (RVDDE) were increased in all patients in group III but were normal in only 3 of the 10 patients in group II. A normal septal movement was found in all patients in groups I and II but also in five patients in group III. The variation in right ventricular diastolic dimension with respiration (RVDVR) was always normal in group II. However, in group III all patients but one had a small RVDVR (less than 6%). It was concluded that a normal RVDDE is very specific (100%) but not sensitive (30%), a normal septal movement is very sensitive (100%) and moderately specific (70.6%), and a normal RVDVR is both very sensitive (100%) and specific (94.4%) as a criterion for identification of small ASDs not requiring surgery.

Adolescent↗

Atrial septal defects in neonates with reference to spontaneous closure.

Early diagnosis of atrial septal defects (ASDs) by means of two-dimensional echocardiography has facilitated studies on the natural course of this lesion. Among 12 Japanese infants in whom a diagnosis of opening of the atrial septum was made in the neonatal period, eight had spontaneous closure at age 2 to 16 months, as shown by two-dimensional echocardiography. To study the exact incidence and natural course of atrial openings in the neonatal period, another 102 consecutive infants from two neonatal care units were followed prospectively with the use of two-dimensional echocardiography. Atrial openings were evident in 24 infants (24%) within the first week of life, in 13 (13%) older than 1 week, in seven (7%) older than 1 month, in five (5%) older than 6 months, and in two (2%) older than 1 year. Twenty-eight of the 30 infants with spontaneous closure in both groups had valvelike openings of the atrial septum that closed by fusion of the valves. ASDs that closed spontaneously in infants with clinical signs and symptoms of atrial shunt may be the same clinical entity as the valvelike openings of the atrial septum frequently observed in neonates with few clinical symptoms and may be differentiated morphologically from ASDs with openings without valve formation, which have little tendency to close.

Diagnosis, Differential↗

Arrhythmias after repair of secundum atrial septal defect: the influence of surgical modification.

A retrospective study performed in our institution showed a significant correlation between venous cannulation and the incidence of arrhythmias after atrial septal defect (ASD) repair. We now report the results of a prospective study in 50 children operated on for ASD with selective cannulation of the superior vena cava. ECGs and Holter recordings were made before and after surgery, with a mean follow-up of 2.6 years. We found a significant decrease in postoperative arrhythmias (p less than 0.05) after changing the cannulation technique. Severe arrhythmias, present in 10% of the children in the retrospective group, were not found in the prospective study. Six children had asymptomatic arrhythmias on the Holter recording 1 year after surgery. Three of them, however, already had arrhythmias before the operation. We conclude that the incidence and severity of arrhythmias after ASD repair can be reduced significantly by surgical modifications, at least for the follow-up period of this study. The long-term significance has to be awaited.

Adolescent↗

Masked subaortic stenosis in ostium primum atrial septal defect: recognition and treatment.

Five patients with ostium primum atrial septal defect (ASD) and a cleft mitral valve had no hemodynamic evidence of left ventricular (LV) outflow tract obstruction on preoperative cardiac catheterization. After surgical closure of the ASD and repair of the mitral cleft, all 5 patients manifested subaortic stenosis with pressure gradients ranging from 10 to 120 mm Hg. Postoperative LV angiograms revealed systolic narrowing of the outflow tract, and the same outflow tract dynamics were recognized on reviewing the preoperative angiograms and echocardiograms. Persistence or exaggeration of the characteristic diastolic "goose-neck" deformity during LV systole in atrioventricular canal defects is diagnostic of a potential or actual subaortic obstruction. This diagnostic sign is also readily recognizable by 2-dimensional echocardiography, and when present, the surgeon should be alerted to explore the LV outflow tract because the outflow tract anatomy is not readily apparent at operation aimed solely at closing the ASD and repairing the cleft mitral valve.

Adolescent↗

Diagnosis of increased pulmonary blood flow by suprasternal M-mode echocardiography in atrial septal defect.

The right pulmonary artery (PA) was quantitatively assessed by suprasternal M-mode echocardiography in 25 patients in whom an atrial septal defect (ASD) was suspected clinically. In 10 patients an ASD was excluded (Group 1) and in 15 it was confirmed (Group 2). The smallest diameter of the right PA at end-diastole in Group 1 was 8.8 +/- 1.5 mm/m2 body surface area and in Group 2 14.8 +/- 3.6 mm/m2 (p less than 0.001). The greatest diameter of the right PA during systole was also much smaller in Group 1 (11.3 +/- 1.2 mm/m2) than in Group 2 (17.7 +/- 3.5 mm/m2) (p less than 0.001). The absolute and percent systolic expansion of the right PA did not differ in the 2 groups (2.7 +/- 0.5 mm [29.1 +/- 10.8%] in Group 1 and 2.9 +/- 0.8 mm [20.8 +/- 9.8%] in Group 2). No correlation was found between measured and derived echocardiographic variables of the right PA and the magnitude of the left-to-right shunt. Patients in Group 2, who had an additional pressure elevation in the PA, showed, on average, a larger right PA and a smaller percent systolic expansion. The study demonstrates characteristic alterations in the wall motion pattern of the right PA in patients with ASD, indicating increased pulmonary blood flow.

Adolescent↗

Electrophysiologic abnormalities of children with ostium secundum atrial septal defect.

Sinus node (SN) and atrioventricular node (AVN) function were evaluated in 49 patients with secundum type atrial septal defect (ASD). Automaticity and conduction system function were assessed by intracardiac recording of the AH and HV intervals at rest, corrected SN recovery time, sinoatrial conduction time, AVN refractory period and the ability of the AVN to conduct rapidly paced atrial beats to the ventricles. Electrophysiologic abnormalities were found in 41% of the 34 patients who were studied before surgery. However, no preoperative abnormalities were present in children younger than 2.5 years. If only children older than 2.5 years were analyzed, the incidence of conduction abnormalities was similar for the patients studied before operation (62%) and those studied after operation (71%). The size and ejection fractions of the right and left ventricles, the magnitude of shunt flow and the size of the ASD did not differ between the patients with and those without electrophysiologic abnormalities. AVN dysfunction was present in 40% of the patients who were studied after surgical repair. While this frequency was more than twice the preoperative incidence of AVN dysfunction, it was not statistically significant. The data suggest that patient age is the major factor that influences the presence of conduction system dysfunction in patients with ASD.

Adolescent↗

Usefulness of echocardiographic assessment of right ventricular and pulmonary trunk size for estimating magnitude of left-to-right shunt in children with atrial septal defect.

M-mode and 2-dimensional echocardiographic studies were performed in 42 patients, aged 1 to 16 years (mean 6), with a secundum or sinus venosus type atrial septal defect (ASD) and normal pulmonary artery pressure. Twenty normal children served as a control group. In patients with ASD the echocardiographic variables were correlated with the magnitude of the left-to-right shunt (Qp/Qs) calculated by the Fick principle. Although M-mode echocardiograms showed increased right ventricular (RV) dimension in 69% of the patients, the correlation between RV dimension index (RV dimension/body surface area) and Qp/Qs was weak (r = 0.49). When RV dimension was related to left ventricular (LV) dimension and expressed by the RV/LV ratio, 90% of the patients were found to have an abnormally large right ventricle. The correlation between the RV/LV ratio and Qp/Qs was fairly good (r = 0.64). In 33 patients (78%), the pulmonary trunk (PT) was adequately visualized and measured on 2-dimensional echocardiograms. The dimension of the PT was related to the aortic root dimension and expressed by the PT dimension/aortic dimension ratio. This ratio was 0.99 +/- 0.06 in normal children and 1.35 +/- 0.23 in patients with ASD (p less than 0.001). The PT/aortic ratio exceeded the upper limit of normal (the normal mean value + 2 standard deviations) in each of the 27 patients with a Qp/Qs of 1.5 or greater. In 5 of the 6 patients with a Qp/Qs of less than 1.5 the PT/aortic ratio was close to 1 and within the normal range. An excellent correlation (r = 0.89) was found between the PT/aortic ratio and Qp/Qs.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Extravascular lung water in children immediately after operative closure of either isolated atrial septal defect or ventricular septal defect.

Extravascular lung water (EVLW) was measured in 16 patients with congenital heart disease by the cold green dye, double indicator dilution technique. Five patients with optimally corrected tetralogy of Fallot served as controls, and EVLW in this group was 4.7 +/- 0.5 ml/kg (111 +/- 13 ml/m2) (mean +/- standard deviation). In 5 asymptomatic patients with atrial septal defect (ASD), normal pulmonary artery (PA) pressure and increased pulmonary blood flow, EVLW was 5.7 +/- 2.8 ml/kg (132 +/- 63 ml/m2), which was not significantly different from the value of control patients. However, in 6 patients with ventricular septal defect, PA hypertension, normal left atrial pressure and an equivalent left-to-right shunt to ASD patients, EVLW was 15.9 +/- 3.8 ml/kg (270 +/- 60 ml/m2). This was significantly different from values in both control and ASD patients (p less than 0.01). It is concluded that in the face of normal pulmonary vascular resistance, PA pressure is transmitted to the microvasculature, causing hydrostatic pulmonary edema. Other factors that may be implicated in the pathogenesis of pulmonary edema, such as increased pulmonary blood flow and relative lymphatic insufficiency in infants, cannot be excluded.

Child↗

Pulmonary arterial thrombosis in secundum atrial septal defect.

Nineteen adolescent or adult patients with secundum atrial septal defect (ASD) underwent pulmonary arteriography to evaluate the presence of proximal pulmonary arterial (PA) thrombosis. This procedure demonstrated proximal PA thrombosis in 8 patients (group 2). These patients had a distinctive hemodynamic profile, consisting primarily of significant PA hypertension. None of the 11 patients with normal angiograms (group 1) had severe PA hypertension (p less than 0.0001). Proximal PA thrombosis appears to be the major factor in the development and progression of PA hypertension in adult patients with ostium secundum ASD. Pulmonary angiography should be undertaken in all adult patients with ostium secundum ASD who have at least moderate PA hypertension. Long-term anticoagulation is advocated for patients with PA thrombosis irrespective of a decision for surgical intervention.

Adolescent↗

Spontaneous closure of fenestrations in an interatrial Gore-Tex patch: application to the Fontan procedure.

The concept of the partial Fontan procedure, first described with the adjustable atrial septal defect (ASD) and more recently with the fenestrated technique, has become an accepted approach for the management of high-risk patients undergoing the Fontan procedure. Experience with both techniques has shown that a patent ASD placed in a prosthetic interatrial baffle may close spontaneously over a period of weeks to months. The mechanism and timing of spontaneous closure, as well as the effect of antiplatelet therapy on this process, are poorly understood. To better define this process, the interatrial septum of 15 mongrel dogs was excised and replaced with a fenestrated Gore-Tex (W.L. Gore, Flagstaff, AZ) patch. Postoperative echocardiography confirmed the patency of the ASD and left-to-right shunting. Animals were sacrificed 4 to 6 weeks postoperatively, or sooner if infection or other postoperative complications developed. Eight animals underwent no antiplatelet or anticoagulation therapy postoperatively, and 7 received antiplatelet therapy with aspirin. Patches were removed at the end of the study period and analyzed. By 6 weeks, all 2.7-mm and 4-mm holes had closed spontaneously in all animals that had not received antiplatelet therapy. The earliest closure occurred at 1 week. With antiplatelet therapy, hole closure was found to be delayed but not prevented, and was complete by 6 weeks in all but 1 animal. Histologic examination of the explanted patches revealed that closure was accomplished primarily through the ingrowth of fibrous tissue, accompanied by an inflammatory cell infiltrate.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Induction of high SCEs in normal cells by 11-12 kDa plasma protein overexpressed in some sleep deprived volunteers.

The present study confirmed our earlier preliminary observation of induction of high SCEs in 1 day sleep-deprived healthy volunteers and showed that the property of induction of high SCE by after-sleep-deprived (ASD) plasma was retained both in 50% ammonium sulphate precipitates and in the dialysed plasma using a membrane of 12 kDa porocity. Further, nitrocellulose bound 11-12 kDa electrophoretically isolated plasma protein from ASD individuals was found responsible for the induction of high SCE in normal whole blood cultures. A short exposure of 3 h to 5% ASD plasma was enough to induce significantly high SCEs in normal lymphocytes which had undergone 2 replication cycles after the exposure to this factor.

Adult↗

Residuae, sequelae, and complications of surgery for congenital heart disease.

Definitive, if not curative surgery is available for the eight most common congenital cardiac defects-ductus arteriosus, ASD, coarctation, pulmonary valve stenosis, aortic valve stenosis, tetralogy of Fallot, and transposition. The results of surgery for uncomplicated cases of DA, ASD, VSD, and coarctation usually can be determined by clinical means (including chest radiogram and ECG). Postoperative heart catheterization is recommended for evaluation of the patient who has had surgery for pulmonary valve stenosis or artic stenosis and is necessary after tetralogy of Falot or transposition of the great arteries repair to identify the important postoperrative residua and sequelae. The term "curative" surgery probably shoud be reserved for operation for divion of ductus arteriosus unassociated with pulmonary hypertension and performed in childhood. After closure of ASD, patients should continue to be observed for late development of arrhythmias and persistent cardiac enlargement, although the incidence of these problems is low. After VSD closure the patient is still followed at intervals for possible ill effects of the ventriculotomy scar, manifest as arrhythmias, ventricular aneurysm or myocardial insufficiency. The patient with coarctation repair must be observed for a possible late complication from one of the several clinically silent cardiovascular or cerebrovascular anomalies as well as for the change of restenosis or unrelieved hypertension...

Aortic Coarctation↗

Isolated atrial amyloidosis: a clinicopathologic study indicating increased prevalence in chronic heart disease.

Congo red screening of 211 consecutive cardiac biopsy specimens obtained during cardiac surgery from 167 patients revealed 26 (16%) instances of isolated atrial amyloidosis (IAA). The ages of IAA-positive patients ranged from 25 to 52 years (mean age, 39 years). Twenty-three (88%) IAA-positive biopsy specimens were from patients with chronic rheumatic heart disease (CRHD) while three (12%) were from patients with an atrial septal defect (ASD). The prevalence of IAA in the CRHD patients was 23%, appreciably higher than that in the ASD patients (15%) and in other patients with atrial biopsies. The prevalence of IAA in both CRHD and ASD patients was significantly higher (P < .001) than in controls. Controls consisted of 247 healthy adults who were autopsied after traumatic deaths, with an age range of 18 to 89 years (mean age, 38 years). Only seven (3%) control subjects were IAA positive; all were over 40 years of age. Isolated atrial amyloidosis deposits were permanganate resistant and immunohistochemically positive for human amyloid P (AP) protein and negative for human amyloid-associated (AA) protein and immunoglobulin light chains. They were observed as fine congophilic and birefringent deposits in intramyocardial vessel walls, along the myocardial sarcolemma, and in the subendocardium. There was associated myocyte hypertrophy but no atrophy. Electron microscopy demonstrated typical nonbranching amyloid fibrils. It is postulated that stretching of the atria in chronic heart disease results in a raised prevalence of IAA. Recent reports that IAA contains atrial natriuretic peptide, a polypeptide hormone product of atrial myocytes, supports this view.

Adolescent↗

Lipopolysaccharide (LPS) binding to 73-kDa and 38-kDa surface proteins on lymphoreticular cells: preferential inhibition of LPS binding to the former by Rhodopseudomonas sphaeroides lipid A.

Using a photoactivable, radioiodinated lipopolysaccharide probe, [125I]ASD-LPS (derivatized from purified E. coli 0111:B4 S-LPS), we earlier reported the presence of a 73-kDa (p73) predominant LPS-binding protein on mouse lymphocytes and macrophages with specificity for the lipid A region of LPS. Both Re-LPS from Salmonella minnesota and purified lipid A will inhibit the binding of LPS to the p73 LPS receptor. In the studies reported here, we have found that non-toxic diphosphoryl lipid A purified from Rhodo-pseudomonas sphaeroides has the capability to inhibit the binding of [125I]ASD-LPS to the p73 protein. However, using the same LPS probe and photoaffinity cross-linking techniques, our data suggest that a less dominant 38-kDa (p38) LPS-specific binding protein identified on mouse splenocytes, J774.1 macrophage-like cell line, and 70Z/3 pre B-cell line by SDS-PAGE is not inhibited by purified lipid A, even at a concentration in 50-fold excess of that of [125]ASD-LPS. The binding of the LPS probe to the 38 protein could be inhibited in a dose-dependent manner by underivatized native S. minnesota Re-LPS (composed only of Kdo and lipid A). We speculate that this p38 LPS-binding protein may manifest a specificity for inner core oligosaccharide determinants on LPS.

Acute-Phase Proteins↗

Oral immunization with recombinant Salmonella typhimurium expressing surface protein antigen A (SpaA) of Streptococcus sobrinus: effects of the Salmonella virulence plasmid on the induction of protective and sustained humoral responses in rats.

Recombinant strains of Salmonella typhimurium have been studied as antigen delivery systems to determine their effectiveness as multivalent vaccines. Here we compare the efficacy of two strains of S. typhimurium. chi 4072 (pYA2905) and chi 3987 (pYA2905), expressing SpaA of Streptococcus sobrinus 6715 as oral vaccines for dental caries. Both strains are attenuated delta cya delta crp delta asd mutants with their Asd phenotypes complemented by the Asd+ plasmid pYA2905, which also encodes a peptide fragment of SpaA. S. typhimurium chi 3987 (pYA2905), unlike S. typhimurium chi 4072 (pYA2905), contains the 100 kb S. typhimurium virulence plasmid. Fischer rats were orally immunized with approximately 10(9) S. typhimurium chi 3987 (pYA2905) or chi 4072 (pYA2905) and then challenged with cariogenic S. sobrinus 6715. Rats orally immunized with either strain of recombinant Salmonella developed salivary IgA anti-SpaA responses and had lower levels of S. sobrinus-induced dental caries than nonimmunized, infected animals. In a second series of experiments, the kinetics and isotype of the serum and salivary antibody responses were determined in rats orally immunized with S. typhimurium chi 3987 (pYA2905) or chi 4072 (pYA2905) on weeks 0 and 8. IgG and IgM serum antibody responses to SpaA and S. typhimurium were detected after the primary and secondary immunizations, and the secondary immunization boosted serum IgG anti-Salmonella activity. In general, animals immunized with chi 3987 (pYA2905) had higher serum anti-SpaA, as well as serum and salivary anti-Salmonella, responses than animals immunized with chi 4072 (pYA2905). This study demonstrates the effective use of two recombinant S. typhimurium strains as oral vaccines for inducing protective and sustained immune responses against a mucosal pathogen and suggests that the recombinant Salmonella vaccine strain carrying the virulence plasmid induced similar or higher protective immune responses than the strain lacking the virulence plasmid.

Administration, Oral↗

Arthroscopic subacromial decompression with and without the Holmium:YAG-laser. A prospective comparative study.

The purpose of this study was to critically evaluate the results of laser-assisted arthroscopic subacromial decompression (ASD) and compare them with the results of a conventional arthroscopic procedure. In a prospective study, 52 patients with stage II and III (partial tear) impingement syndrome undergoing ASD were divided into two consecutive groups. The first 18 patients underwent conventional arthroscopic surgery using shaver and electrocautery (group S). The following 34 patients underwent ASD with the Holmium:YAG-laser (22 watt) and without electrocautery (group L). Subjective, objective, and functional results were assessed using the constant score preoperatively and postoperatively at specific intervals up to 1 year. The greatest improvement in the laser group was seen in the areas of pain with activity, pain at night, activity and movement at 1 week and at 6 weeks. The patients in group L also showed significantly better values for abduction power (P < .05). There were no complications in either group. Radiographic evaluation with the outlet view, preoperatively and postoperatively, showed an adequate bone resection in all cases. The postoperative Constant score for group L was significantly better. The average score increased from 54.7 to 79.8 in group L and from 50.3 to 68.7 in group S. Because of the low level of postoperative pain, the absence of adhesions and the almost complete lack of swelling, the patients in group L were able to regain full range of shoulder motion sooner than those in group S.

Adult↗