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Minimally invasive or interventional repair of atrial septal defects in children: experience in 171 cases and comparison with conventional strategies.

OBJECTIVES: The goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children. BACKGROUND: Concern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization. METHODS: A retrospective analysis was performed on the patients treated from June 1996 to December 1998. RESULTS: One hundred seventy-one children (median age 5.8 years, median weight 22.1 kg) underwent 52 device implants, 72 minimally invasive surgical operations and 50 conventional sternotomy operations. There were no deaths and no residual left to right shunt in any of the groups. The overall complication rate causing delayed discharge was 12.6% for minimally invasive surgery, 12.0% for midline sternotomy and 3.8% for transcatheter device closure (p < 0.01). The mean hospital stay was 2.8 +/- 1.0 days, 6.5 +/- 2.1 days and 2.1 +/- 0.5 days (p < 0.01); the skin-to-skin time was 196 +/- 43 min, 163 +/- 46 min and 118 +/- 58 min, respectively (p < 0.001). Extracorporeal circulation time was 49.9 +/- 10.1 min in the minithoracotomy group versus 37.2 +/- 13.8 min in the sternotomy group (p < 0.01) but without differences in aortic cross-clamping time. Sternotomy was the most expensive procedure (15,000 EUR +/- 1,050 EUR vs. 12,250 EUR +/- 472 EUR for minithoracotomy and 13,000 EUR +/- 300 EUR for percutaneous devices). CONCLUSIONS: While equally effective compared with sternotomy, the cosmetic and financial appeal of the percutaneous and minimally invasive approaches must be weighed against their greater exposure to technical pitfalls. Adequate training is needed if a strategy of surgical or percutaneous minimally invasive closure of ASD in children is planned in place of conventional surgery.

Analysis of Variance↗

Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography.

OBJECTIVE: This study sought to demonstrate that with proper technique, identification of the normal and abnormal pulmonary venous connection can be made with confidence using transesophageal echocardiography (TEE). BACKGROUND: Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital anomaly whose diagnosis has classically been made using angiography. METHODS: We performed a retrospective review of all patients of all ages with PAPVC diagnosed at the Mayo Clinic who had undergone TEE because of either right ventricular volume overload or suspected intracardiac shunting by transthoracic echocardiography or intraoperatively. RESULTS: A total of 66 PAPVCs were detected in 43 patients (1.5/patient); in 2 additional patients, TEE suggested, but did not diagnose, PAPVCs. Shortness of breath was the most common presenting symptom (42.2%), followed by heart murmur and supraventricular tachycardia. Right-sided anomalous veins were identified in 35 patients (81.4%), left-sided in 7 (16.3%) and bilateral in 1 (2.3%). There was a single anomalous connecting vein in 23 patients (53.5%), two in 18 (41.9%), three in 1 (2.3%) and four in 1 (2.3%). The connecting site was the superior vena cava (SVC) in 39 veins (59.1%), right atrial-SVC junction in 6 (9.1%), right atrium in 8 (12.1%), inferior vena cava in 1 (1.5%) and the coronary sinus in 2 (3.0%). Ten anomalous left pulmonary veins were connected by a vertical vein to the innominate vein (15.1%). Sinus venosus atrial septal defect (ASD) was the most common associated anomaly in 22 patients (49%), followed by ostium secundum ASD in 6 and patent foramen ovale in 4. Fifteen patients had an intact atrial septum. Thirty-one patients (68.8%) underwent surgical repair. PAPVC was confirmed in all patients, including the two whose TEE results were suggestive of PAPVC. All 49 PAPVCs detected by TEE preoperatively were confirmed at the time of operation. CONCLUSIONS: TEE is highly diagnostic for PAPVC and can obviate angiography. Accurate anatomic diagnosis may influence the need for medical and surgical management. TEE should be performed in patients with right ventricular volume overload when the precordial examination is inconclusive.

Adult↗

Arthroscopic subacromial decompression: analysis of one- to three-year results.

Arthroscopic subacromial decompression (ASD) is a method of performing anterior acromioplasty utilizing basic arthroscopic techniques. The procedure is indicated in cases of chronic impingement syndrome that have failed to respond to prolonged conservative management. The purpose of this study is to present an analysis of the 1- to 3-year follow-up results of the initial 50 consecutive cases of ASD that I have performed. Forty (80%) of the cases had advanced stage II impingement without rotator cuff tear. Ten (20%) had full-thickness tears of the rotator cuff. Patients were evaluated pre and postoperatively on the UCLA Shoulder Rating Scale, which includes an assessment of pain, function, range of motion (ROM), strength, and patient satisfaction. Eighty-eight percent of the cases were rated "satisfactory" (excellent or good), and 12% were rated "unsatisfactory" (fair or poor). The procedure is technically demanding, and to achieve a satisfactory result the criteria of open anterior acromioplasty must be met. Arthroscopic subacromial decompression is presented as an alternative to open anterior acromioplasty in advanced stage II and selected cases of stage III impingement syndrome.

Acromion↗

The clinical effect of ketoprofen after arthroscopic subacromial decompression: a randomized double-blind prospective study.

The purpose of the study was to evaluate the clinical effect of ketoprofen after arthroscopic subacromial decompression (ASD). The design was randomized, prospective, and double-blind, with a placebo control group. Forty-one consecutive patients with subacromial impingement syndrome, were randomized to treatment with ketoprofen 200 mg once daily or placebo for 6 weeks following ASD. For additional analgesia, patients used paracetamol if necessary. Clinical follow-up was performed at 6 weeks and at 2 years postoperatively. At the 6-week follow-up, the patients treated with ketoprofen had a statistically significant increase in UCLA total score (P<.05), range of movement (P<.05), and satisfaction (P<.05), and they had significantly less pain (P<.05). There was no statistical difference between the ketoprofen and placebo groups regarding strength. Patients receiving ketoprofen had significantly less need for additional analgesia (P<.05). At the 2-year follow-up, there were no differences in the scores between the ketoprofen and placebo group.

Acromion↗

Congenital division of the left atrium: the influence of associated congenital lesions on the timing and mode of presentation.

PURPOSE OF STUDY: Congenital division of an atrium is commonly associated with other cardiac malformations, which may influence presentation. BASIC METHODS: Cases diagnosed at our institution between 1980 and 1997 were reviewed, to determine whether associated lesions influence presentation. PRINCIPLE FINDINGS: 52 patients were diagnosed with atrial division (49 left, 3 right), mean age 4.8 yr. Patients could be divided according to age at diagnosis. Group 1: 32 patients aged < or = 1 yr. 14 patients had associated cardiac malformations other than atrial septal defect (ASD). Presentation was with pulmonary oedema. Group 2: 20 patients > 1 yr. 5 patients had associated major malformations other than ASD. Commonest presentation--isolated shortness of breath. Fifty patients underwent membrane resection and correction of associated anomalies (4% mortality). At follow-up (mean 12 yr) all patients were in NYHA class I or II. CONCLUSIONS: Patients with division of the atrium present early in infancy and with more severe symptoms when associated with other cardiac malformations.

Abnormalities, Multiple↗

Sinus venosus atrial septal defects: surgical follow-up.

OBJECTIVE: To assess early and later results after surgery for sinus venous atrial septal defects (ASDs). METHODS: Forty-four patients of sinus venosus ASDs with anomalous drainage of the right superior pulmonary vein into the superior vena cava, were operated upon between January 1985 and June 1995. Defects were approached by an incision starting from the tip of the right atrial appendage then extending upward along the atrial crest to the medial wall of the superior vena cava. Atrial defects were closed by an autologous pericardial patch. Another pericardial patch was used to enlarge the superior vena cava-right atrial junction to avoid the narrowing of the superior vena cava. RESULTS: There was no operative mortality. The total duration of follow-up was 4 +/- 0.7 years. Follow-up of the echocardiographic study at 6 months and 2 years after surgery revealed a normal study. Two patients developed sinus node dysfunction of short duration in the immediate postoperative period. Follow-up of the electrocardiographic study at 6 months and 2 years revealed sinus node dysfunction in one and two patients, respectively.

Adolescent↗

Cardiac surgery by transxiphoid approach without sternotomy.

OBJECTIVE: In a attempt to avoid the potential drawbacks associated with sternotomy coupled with a desire for a smaller scar led us to investigate the transxiphoid approach without sternotomy. We present our preliminary experience and a comparison between the sternal and thoracic approaches. METHODS: From June 1996, at the Institut Cardiovasculaire Paris Sud, Massy, France (ICPS) and the Heart Institute, Sao Paulo, Brazil (HI) the transxiphoid approach was adopted for the correction of selected congenital cardiac defects. The xiphoid was resected through a 6 cm long vertical skin incision. With a special retractor the sternum was elevated cephalad and anteriorly. Closure of the defect was performed in the conventional manner. Twenty-six patients; 17 boys and 9 girls were entered into the study from representing 19 atrial septal defects (ASDs), 4 ventricular septal defects (VSDs) and 3 partial atrio ventricular septal defect (AVSDs). In addition at ICPS the transxiphoid approach for correction of ASD was compared to the thoracic and sternal approaches performed in the same period. RESULTS: Both the aortic cross clamp time as well as the duration of extracorporeal circulation were increased when compared to either standard sternotomy or thoracotomy approaches. There were no differences within the groups when comparing body surface area, amount of chest drainage or length of either ICU or hospital stay. However the patients in the transxiphoid group showed less pain and respiratory discomfort. CONCLUSION: Our initial experience with the transxiphoid approach without sternotomy confirms that it is a promising technique that can be considered an alternative to conventional sternotomy. The access is adequate for surgical procedures performed through a right atriotomy. The advantages include a better cosmetic scar, less surgical trauma, minimal respiratory discomfort and a potentially lower risk of infection. However cardiopulmonary bypass and cross clamp times are increased. There were no complications, and patient satisfaction was high.

Cardiac Surgical Procedures↗

Autism: a target of pharmacotherapies?

Autism is reaching epidemic proportions. The diagnosis can be made as early as 2 years of age, and autistic patients are expected to have a normal life span. Thus, in terms of the number of 'patient years', autism spectrum disorder (ASD) represents a market that is as large as that of the biggest neurological indication, Alzheimer's disease. However, despite the clear unmet medical need no effective treatment is yet available. This could be because the biology of ASD is not clearly understood and thus proper drug treatment has not been possible. However, significant advances are being made toward understanding the mechanisms of the disease. Here, we review the most recent preclinical advances in the hope that they will lead to a breakthrough in the near future.

Animals↗

[Hemodynamics and delayed growth in children after surgery for atrial septal defect].

BACKGROUND: Ostium secundum-type atrial septal defect (ASD) is usually well tolerated, without severe complications in childhood. However, in many patients its effect on growth is disproproportionate when compared with that of more serious congenital heart disease. OBJECTIVES: To test the hypothesis that hemodynamic disturbances are responsible for failure to thrive in congenital heart disease, we studied growth and the effect of surgical repair in children with ASD. PATIENTS AND METHODS: The study was performed in 72 patients who underwent surgery at a mean age of 8 years and 8 months. The mean follow-up was 17 months. Somatometric and hemodynamic parameters were studied and the correlation between them was verified. The effect of surgery and of age at operation on somatometric changes was evaluated. RESULTS: Fifty-two percent of the patients were malnourished and height was affected in 28 %. The correlation between weight and age at operation was r 5 0.78 (p < 0.05) for the entire group, r 5 0.81 (p < 0.05) for the malnourished subgroup and r 5 0.88 (p < 0.05) for the subgroup with normal nutrition. No relationship was found between hemodynamic and somatometric parameters. Surgery improved growth disturbances but not in all patients (weight: from 52 % to 32 %; height: from 28 % to 16 %). CONCLUSIONS: Hemodynamic factors are not the only cause of growth and nutritional alterations.

Child↗

Autism, primary pragmatic difficulties, and specific language impairment: can we distinguish them using psycholinguistic markers?

Three groups of children with communication disorders were examined using a series of psycholinguistic markers to explore whether the tasks could identify children with impairments other than specific language impairment (SLI), and to examine whether the different groups within this clinical population could be distinguished reliably from one another. The groups comprised children with autistic spectrum disorders (ASD; n = 13, all males; mean age 10 years 10 months, range 10 years 2 months to 12 years 6 months); children with primary pragmatic language impairment (PLI) but who did not have definite ASD diagnoses (n = 25, 22 males, three females; mean age 11 years 3 months, range 10 years 2 months to 12 years 5 months); and children with specific language impairment (SLI) without marked pragmatic language difficulties (n = 29, 25 males, 4 females; mean age 10 years 10 months, range 10 years 2 months to 11 years 9 months). Clinical markers examined were: the Children's Non-Word Repetition (CNRep), the Past Tense Task (PTT), and the Clinical Evaluation of Language Fundamentals, Recalling Sentences. First, it was found that the a priori groupings were not sufficiently defined and that four groups were actually present. The PLI group was in fact two separate samples: those with PLI pure and those with some autistic-like behaviours (referred to here as PLI plus, following Bishop 1998). Second, group comparisons indicated that CNRep was significantly lower for children with SLI than all other groups (although this measure was not such a good discriminator using a specificity analysis). Third, the markers were able to discriminate between all types of communication impairment in normal control participants (n = 100; 51 females, 49 males; mean age 11 years, range 10 years 5 months to 11 years 6 months) with sensitivity levels of at least 75% and specificity of 80%. Recalling Sentences was the most efficient marker for all groups. Finally, analysis showed that children with PLI plus could be accurately distinguished from all others, scoring most favourably overall on communication markers and on performance IQ scores.

Autistic Disorder↗

Sleep patterns and sleep disorders in children with autistic spectrum disorders: insights using parent report and actigraphy.

The present study sought to describe the profile of sleep disturbance reported in children with autistic spectrum disorders (ASDs) and to document any sleep disorders underlying reports of sleeplessness. Sixty-nine children aged 5 to 16 years (mean 9 years 4 months, SD 2 years 7 months; 14 females) with an ASD were assessed by detailed sleep histories taken from parents, the Simonds and Parraga Sleep Questionnaire, a 2-week sleep diary, and actigraphs worn by the child for five nights. Parent-reported sleeplessness featured prominently (64%). Sleep disorders underlying the sleeplessness were most commonly behavioural (i.e. to do with inappropriate sleep-related behaviours), although sleep-wake cycle disorders and anxiety-related problems were also seen. In addition, the sleeplessness patterns of a large minority of children could not be classified by conventional diagnostic criteria. Sleep patterns measured objectively did not differ between those children with or without reported sleeplessness, but the sleep quality of all children seemed to be compromised compared with normal values.

Adolescent↗

Autism interventions: a critical update.

As yet, there is no aetiology-based intervention for autistic spectrum disorders (ASD); despite this, parents and professionals still need to make informed decisions regarding treatment options for children with ASD. This paper seeks to evaluate widely used interventions according to specific research criteria. Interventions presented are grouped into psychoeducational/behavioural approaches, psychopharmacological interventions, and the less traditional or complementary approaches. The conclusions are less than favourable: while some interventions do have empirical support, others have been proven to have no positive effects, and furthermore, there are no robust data favouring one approach over the others. Nevertheless, several criteria for choosing between treatment options are briefly discussed.

Autistic Disorder↗

Characteristics of fetal anticonvulsant syndrome associated autistic disorder.

The aim of this study was to evaluate the clinical features and frequency of autistic disorder or Asperger syndrome (AS; according to Diagnostic and Statistical Manual of Mental Disorders, 4th edition [DSM-IV] criteria) in children exposed to anticonvulsant medication in utero. During a 20-year study period, 626 children were born in Aberdeen to mothers taking antiepileptic drugs (AEDs). The study examined long-term effects of prenatal exposure to AEDs in 260 children (122 males, 138 females). Of these, 26 (16 males) were reported by parents to have social or behavioural difficulties. Eleven children (6 males, 5 females) fulfilled the DSM-IV criteria for autistic disorder and one (female) fulfilled the DSM-IV criteria for AS. These children comprised 4.6% of the exposed children studied, and 1.9% of all exposed children born during the study period. Mean age of these children at diagnosis was 5 years 4 months (SD 2y 11mo) and 9 years 10 months (SD 3y 10mo) at the time of this study. Other children from the group of 26 had difficulties in areas of speech and language development and social communication but did not meet the criteria for an autism spectrum disorder (ASD). Sodium valproate was the drug most commonly associated with autistic disorder, five of 56 (8.9%) of the study children exposed to sodium valproate alone had either autistic disorder or AS. It was concluded that prenatal exposure to anticonvulsant medication is a risk factor for the development of an ASD. Fetal anticonvulsant syndrome associated autistic disorder is characterized by an even sex ratio, absence of regression or skill loss, and language delay in the absence of global delay.

Anticonvulsants↗

Photoaffinity labeling of acyl-CoA oxidase with 12-azidooleoyl-CoA and 12-[(4-azidosalicyl)amino]dodecanoyl-CoA.

Synthesis of 32P-labeled CoA of high specific activity was achieved using partially purified dephospho-CoA kinase (EC 2.7.1.24) from pig liver with [gamma-32P]ATP as donor and dephospho-CoA as acceptor. A photoaffinity dodecanoic acid analog, 12-[(4-azidosalicyl)amino]dodecanoic acid was synthesized, as were its CoA derivative (ASD-CoA) and the CoA derivative of 12-azidooleic acid. The CoA derivatives were synthesized from azido fatty acid analogs by acyl-CoA synthetase. The synthesized photolabile reagents were tested as photoaffinity labels for acyl-CoA oxidase (EC 1.3.99.3) from Arthrobacter species. When a mixture of oxidase and the acyl-CoA analogs were incubated in the absence of ultraviolet light, the analogs were recognized as substrate. Acyl-CoA oxidase was incubated in the presence of acyl-CoA analogs and immediately photolyzed, which resulted in irreversible inhibition. Oleoyl-CoA and dodecanoyl-CoA protect the enzyme from photoactivated inhibition by 12-azidooleoyl-CoA and ASD-CoA, respectively. Analysis of photolyzed enzyme preparations by sodium dodecyl sulfate-polyacrylamide gel electrophoresis and autoradiography revealed that both analogs preferentially labeled a 54,000 molecular weight protein. These results demonstrate that the photoaffinity acyl-CoA analogs have potential application as probes to identify and characterize lipid biosynthetic enzymes and to identify the active site of these proteins.

Acyl Coenzyme A↗

Analysis of the membrane-interacting domain of the sea urchin sperm adhesive protein bindin.

We have investigated the domain of the bindin polypeptide that selectively associates with gel-phase phospholipid vesicles. We found that small trypsin fragments of bindin retain the ability to selectively associate with gel-phase vesicles. The primary amino acid sequence of bindin suggests that these peptides are derived from the central portion of the polypeptide between residues 77 and 126, which is the most hydrophobic region of bindin. We have also employed 3-(trifluoromethyl)-3-(m-[125I]iodophenyl)diazirine (TID) and novel, radioiodinated, photoactivatable derivatives of the polar head group of phosphatidylethanolamine (ASD-PE and ASA-PE) to identify membrane-associated polypeptide segments after the transfer of radiolabel from the probe to the bindin polypeptide. After photolysis, bindin was selectively labeled only from probes incorporated in gel-phase vesicles. The labeling of bindin was much more efficient from the head group probes ASA-PE and ASD-PE (8 and 2% of the total label, respectively) in comparison to the hydrophobic probe TID (less than 0.02% of the total label), suggesting that bindin is localized within the polar part of the bilayer. Protease mapping experiments with V8 protease, trypsin, and endoprotease Lys-C suggest that some of the probe label is distributed along the amino-terminal portion of bindin between residues 1 and 76 and the rest of the label is restricted to the segments between residues 77 and 126 which also selectively bind to gel-phase vesicles. The carboxyl-terminal portion of bindin between residues 127 and 236 is not labeled.

Amino Acid Sequence↗

Petrosamine B, an inhibitor of the Helicobacter pylori enzyme aspartyl semialdehyde dehydrogenase from the Australian sponge Oceanapia sp.

Bioassay-guided fractionation of the MeOH extract of the sponge Oceanapia sp. using the Helicobacter pylori enzyme, aspartyl semialdehyde dehydrogenase, ASD, to detect antibacterial activity, led to the isolation of a new pyridoacridine alkaloid, petrosamine B (1). Petrosamine B is a bright blue compound that is sparingly soluble in many organic solvents. The structure of 1 was determined from detailed NMR studies performed in TFA/D2O. Petrosamine B was found to be a weak inhibitor of ASD with an IC50 of 306 microM.

Acridines↗

Three- and four-dimensional reconstruction of intra-cardiac anatomy from two-dimensional magnetic resonance images.

The present study was designed to evaluate the feasibility and clinical usefulness of three-dimensional (3D) reconstruction of intra-cardiac anatomy from a series of two-dimensional (2D) MR images using commercially available software. Sixteen patients (eight with structurally normal hearts but due to have catheter radio-frequency ablation of atrial tachyarrhythmias and eight with atrial septal defects (ASD) due for trans-catheter closure) and two volunteers were imaged at 1T. For each patient, a series of ECG-triggered images (5 mm thick slices, 2-3 mm apart) were acquired during breath holding. Depending on image quality, T1- or T2-weighted spin-echo images or gradient-echo cine images were used. The 3D reconstruction was performed off-line: the blood pools within cardiac chambers and great vessels were semi-automatically segmented, their outer surface was extracted using a marching cube algorithm and rendered. Intra- and inter-observer variability, effect of breath-hold position and differences between pulse sequences were assessed by imaging a volunteer. The 3D reconstructions were assessed by three cardiologists and compared with the 2D MR images and with 2D and 3D trans-esophagal and intra-cardiac echocardiography obtained during interventions. In every case, an anatomically detailed 3D volume was obtained. In the two patients where a 3 mm interval between slices was used, the resolution was not as good but it was still possible to visualize all the major anatomical structures. Spin-echo images lead to reconstructions more detailed than those obtained from gradient-echo images. However, gradient-echo images are easier to segment due to their greater contrast. Furthermore, because images were acquired at least at ten points in the cardiac cycles for every slice it was possible to reconstruct a cine loop and, for example, to visualize the evolution of the size and margins of the ASD during the cardiac cycle. 3D reconstruction proved to be an effective way to assess the relationship between the different parts of the cardiac anatomy. The technique was useful in planning interventions in these patients.

Arrhythmias, Cardiac↗

Predictors of acute stress following motor vehicle accidents.

The aim of this study was to investigate the predictors of acute stress following motor vehicle accidents (MVA). Sixty-two consecutive adult admissions to a hospital were assessed between two days and four weeks following a MVA. Participants were assessed for acute stress disorder (ASD) with a structured clinical interview and administered the Beck Depression Inventory (BDI), Coping Style Questionnaire, Dissociative Experiences Scale, and the Eysenck Personality Inventory. The prevalences of full and sub-syndromal ASD were 16.1% and 14.5%, respectively. BDI, history of psychiatric treatment, history of posttraumatic stress disorder (PTSD), and history of previous MVA accounted for 61% of the variance of acute stress severity. The findings indicate that predictors of acute stress severity are comparable to the predictors of PTSD and highlight the possibility of identifying those who may benefit from early treatment.

Accidents, Traffic↗