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Coplaning of the acromioclavicular joint.

PURPOSE: Coplaning removes inferior spurs or portions of the distal clavicle to decrease injury to the rotator cuff. This study sought to determine if the presence or degree of coplaning influenced the results and if reoperations for distal clavicle symptoms were needed. TYPE OF STUDY: Nonrandomized control study. METHODS: A total of 76 patients undergoing arthroscopic subacromial decompression (ASD) followed for at least 25 months were divided into 3 groups. Group 1 included 28 patients who underwent ASD with removal of only an inferior clavicular osteophyte. Group 2 included 27 patients with resection of any inferior clavicle spur, violation of the inferior joint capsule, and removal of articular cartilage to a level adjacent with the resected acromion. As much as 50% of the articular cartilage was removed in some cases. Group 3 included 21 patients who underwent a complete distal clavicle excision. Radiographs, charts, and arthroscopic videotapes were reviewed and the degree of claviculectomy exactly determined. Follow-up evaluations included the Constant-Murley, American Shoulder and Elbow Surgeons (ASES), SANE, and Rowe shoulder scores, with special attention given to acromioclavicular (AC) joint pain and additional operations. RESULTS: The average age of the patients was 49 years (range, 19 to 81 years) and follow-up averaged 40 months (range, 25 to 68 months). Follow-up Constant, ASES, Rowe, and SANE scores for the various groups were calculated. Group 1 scores were 99.4, 98.7, 98.6, and 98.1, respectively; group 2 scores were 96.8, 98.7, 98.5, and 95.7, respectively; and group 3 scores were 98.4, 99.4, 99.3, and 98.6, respectively. No patient required any additional AC joint surgery. CONCLUSIONS: Violation of the AC joint capsule and partial distal clavicle resection to make it confluent with the resected acromion (coplaning), does not cause increased AC joint symptoms, compromise the results, or lead to additional surgery at an average 40-month follow-up.

Acromioclavicular Joint↗

Sternal closure with resorbable synthetic loop suture material in children.

PURPOSE: The reliability of poly-p-dioxanone (PDS) suture for sternal closure was tested on 264 consecutive sternotomies in the authors' department from April 1987 to May 1998. METHODS: The reason for sternotomy was tetralogy of Fallot in 65 cases (24.6%), ventricular septal defect (VSD) in 38 cases (14.4%), atrial septal defect (ASD) in 77 cases (29.2%), ASD + VSD in 23 (8.7%), mitral valve replacement in 22 cases (8.3%), aortic valve replacement in 10 cases (3.8%), and other cardiac disorders in 29 cases (11.0%). RESULTS: Sternal wound infection, sternal dehiscence, and mediastinitis occurred in 1.5% of patients (4 of 264). The overall hospital mortality rate related to the mediastinitis was 1.1% (3 of 264) in the early postoperative period. CONCLUSION: This absorbable suture and our different technique are a safe alternative to standard sternotomy closure after pediatric open cardiac surgery.

Adolescent↗

Enzyme-based therapy for autism spectrum disorders -- is it worth another look?

Autism is a developmental disease usually manifesting within the first three years of life. To date, no causative agent has been found. Similarly, treatment options have been limited. Of the treatment options available, a number of them have been nutritionally based in an attempt to address one or more of the theories regarding the etiology of the disease. An example would be enzyme therapy for the digestion of purported offending neuroactive peptides collectively known as exorphins. This paper discusses the exorphin theory of autism and subsequent treatment with dietary enzyme therapy. Novel data are presented in support of the theory that enzymes play a critical role in autism. Forty-six patients between the ages of 5 and 31 were selected for inclusion in the study based on a diagnosis placing them in the category of the autism spectrum disorders (ASD). The diets were supplemented with a novel dietary enzyme formulation, ENZYMAID, for a period of 12 weeks. Progress was tracked according to the Symptom Outcome Survey (SOS) (1) form method of symptom charting and presented in a table for further analysis. The novel enzyme formula, ENZYMAID, beneficially and safely affected all 13 of the parameters measured. Improvements ranged from 50-90%, depending on the parameter measured. Enzyme therapy to treat ASD may indeed a viable option in treatment protocols. These results indicate that further controlled studies are warranted.

Adolescent↗

[Acute left heart failure following repair of atrial septal defects. Its treatment by reopening].

Among 716 patients operated on for an ASD, 15 of them, following closure of the defect, developed acute left heart failure requiring partial re-opening. The most prominent anatomical finding in these cases was hypoplasia of the left ventricle. Also remarkable in some patients was severe pulmonary hypertension, which may have led to right ventricular hypertrophy. Therefore, a marked difference of the stroke work of the left and right ventricle, respectively, appeared to be the essential underlying hemodynamic mechanism. The prognosis has been found to depend mainly on prompt surgical intervention; so in all poor-risk cases continued monitoring of left atrial pressure is indicated for early detection of impending left heart failure.--The high incidence of left heart failure as cause of death after repair of an ASD indicates the importance of this complication.

Adolescent↗

Cardiac malformations associated with the Holt-Oram syndrome--report on a family and review of the literature.

The Holt-Oram syndrome (HOS) is characterized by mild-to-severe congenital cardiac defects and skeletal abnormalities of the upper limb. The most common cardiac disorder is an ostium secundum atrial septal defect (ASD), followed by ventricular septal defect (VSD) and ostium primum ASD. Electrocardiographic abnormalities, such as various degrees of atrioventricular block, have also been reported. In addition, hypoplastic peripheral vessels of the upper limbs have been observed. Here, we will report about a family with three sons having HOS, and we will detail the cardiac spectrum of HOS as reported in the literature.

Adult↗

Uniform long-term gene expression using adeno-associated virus (AAV) by ex vivo recirculation in rat-cardiac isografts.

BACKGROUND: Gene therapy in cardiovascular disease promises to be of great impact. The ideal vector for the therapeutic gene transfection remains to be determined. The aim of the present study was to investigate the efficacy of gene transfer using adeno-associated virus vectors carrying the lacZ-reporter gene (AAV-lacZ) in a previously described coronary recirculation model. METHODS: Beating Lewis rat hearts perfused with oxygenated Krebs-Henseleit solution were harvested, after which an atrial septal defect (ASD) was created. All vessels were tied, and AAV-lacZ was injected into the aortic root. The solution was recirculated through the ASD to the left side of the heart and pumped back to the coronary arteries by the left ventricle. Incubation was allowed for 20 min at 15 degrees C, and the hearts were subsequently transplanted heterotopically in syngeneic rats. Three increasing doses (109, 1,010, 1,011 e. u.) of AAV-lacZ virus vectors were used to study the rate of gene transfer. All hearts were harvested after 7-60 days and evaluated histologically for expression of the lacZ-gene. RESULTS: Dose-dependent gene transfer was observed. Even after 60 days, there was no obvious decline in gene expression. CONCLUSION: Adeno-associated virus vectors offer effective and uniform gene transfer in the myocardium after transcoronary injection and recirculation. Due to the lack of immune response previously described, no decrease in gene expression can be observed up to 60 days after injection.

Animals↗

Total anomalous pulmonary venous draining to inferior vena cava.

A 12-year-old girl complained of tachycardia and dyspnea after exertion but without cyanosis. Her echocardiogram showed an ASD and a widened inferior vena cava. A difference in PO2 between inferior and superior vena cava was evident. An anastomosis between left atrium and pulmonary vein, and a long oval pericardium to cover the opening of the descending vein and ASD into left atrium were performed during cardiopulmonary bypass. The patient recovered without complications.

Child↗

Preparing speech-language pathologists as family-centered practitioners in assessment and program planning for children with autism spectrum disorder.

Family-centered care is a practice model that has been described in the literature for the past two decades. The evidence shows that when family-centered care is practiced, outcomes are enhanced for children with autism and other disabilities and their families and teams. This article reviews two innovative programs that practiced family-centered care in assessment and program planning for children with autism spectrum disorders (ASD). The research indicates that the essential training elements required to transform speech-language pathologists (SLPs) from understanding family-centered care to being family-centered practitioners includes a focus on technical and leadership skills as well as a variety of experiences with families who have children with ASD. Preservice programs preparing SLPs and other health professionals must incorporate these essential elements of family-centered care training into their curricula. SLPs who are already practicing can use these key elements as a guide in seeking professional development opportunities.

Autistic Disorder↗

Using social stories and comic strip conversations to promote socially valid outcomes for children with autism.

Very little is documented regarding the efficacy of social stories and comic strip conversations for promoting an understanding of social situations and the appropriate social behaviors of individuals with autism spectrum disorder (ASD). In addition, few studies on the efficacy of social stories have examined whether outcomes are socially valid. The purpose of this article is to respond to some of the gaps in the literature on the efficacy of a frequently used intervention for children with ASD and to describe a family-centered collaborative approach to developing social stories and comic strip conversations. The results of intervention employing an A-B design are reported for two case vignettes. Clinical implications, limitations of the available data, and potential factors contributing to outcome variability are discussed.

Autistic Disorder↗

Early identification of autism: how early can we go?

Identification of young children at risk for Autism Spectrum Disorders (ASD) depends on early behavioral symptomatology and yet conventional criteria provide little guidance for use with infants and toddlers. Recent research, however, has demonstrated that there are patterns of behavior below 2 years of age that distinguish children with autism from those who are developing typically or those with other developmental disabilities. Skill areas with particular promise for early identification include social communication, sensory regulation, and play. This article previews current innovative methodologies, presents a synthesis of recent research findings related to these three key areas, and provides clinicians with practical guidelines for early identification of infants and toddlers at risk for ASD and other disorders.

Age Factors↗

[Transesophageal contrast echocardiography, open foramen ovale and aneurysm of the atrial septum. Experiences with D-galactose suspension].

AIM: The advantages of transesophageal echocardiography using a D-galactose suspension rather than conventional ultrasonic contrast medium for the detection of a cardiac source of peripheral embolism was to be investigated. METHOD: In 100 patients consecutively examined with transesophageal echocardiography applying a conventional ultrasonic contrast medium the examination was repeated adding a Valsalva test and using a solution of D-galactose (Echovist-300, Schering AG) as contrast medium. RESULTS: With conventional contrast media, 5 interatrial shunts only (2 ASD, 3 patent foramina ovalia, PFO) were detected. Completion of echocardiography with a systemic Valsalva test and use of Echovist-300 resulted in the diagnosis of another 40 PFO. In 17 of 26 patients having an atrial septal aneurysm, an interatrial shunt (1 ASD, 16 PFO) was demonstrated. CONCLUSION: The use of a solution of D-galactose as contrast medium appears to increase the sensitivity of echo-cardiography for demonstrating a cardiac source of peripheral embolism.

Adult↗

Effective gene transfer in the rat myocardium via adenovirus vectors using a coronary recirculation model.

BACKGROUND: Gene therapy promises to play an important role in the treatment of heart disease and in transplantation. The limited effectiveness of gene transfer, however, remains an unresolved problem. The aim of the study was to create a model for more effective gene transfer using adenovirus vectors carrying the lacZ-reporter gene (AdV-lacZ). METHODS: Beating Lewis rat hearts perfused with oxygenated Krebs-Henseleit solution were harvested, after which an atrial septal defect (ASD) was created. All vessels were tied and AdV-lacZ was injected into the aortic root. The solution was recirculated through the ASD to the left side of the heart and pumped back to the coronary arteries by the left ventricle. Incubation was allowed for 20 min at 15 degrees C and the hearts were subsequently transplanted heterotopically in syngeneic rats. This method was compared to AdV-lacZ injection into cardioplegic hearts. The hearts were harvested after 2, 7, or 14 days and evaluated histologically for expression of the lacZ gene. RESULTS: Maximal gene expression was achieved after 7 days by the recirculation model. There was less efficient gene expression at day 2 and at day 14. No evidence of ischemic injury of the myocardium was noticed histologically. Almost no successful gene expression was seen in the arrested hearts. CONCLUSION: This novel recirculation method lets the vector be repeatedly exposed to the endothelium, resulting in an effective gene expression after 7 days incubation time rather than after 14, when a decline has set in presumably due to immunologic response.

Adenoviridae↗

[Closure of an atrial septum defect using an umbrella prosthesis].

About three and a half years after direct suture of a secundum atrial septal defect (ASD) the now 72-year-old patient developed heart failure with pulmonary congestion and pleural effusion which responded to medical treatment. The electrocardiogram showed atrial fibrillation with an irregular ventricular rate and right bundle branch block. Right heart catheterization established recurrence of the ASD with a left to right shunt of 60% (Qp/Qs = 2.5) and a pulmonary artery pressure of 45/12 mmHg. Because of the clinical state, the risk of re-operation and the suitable anatomy, non-operative percutaneous transvenous catheter closure of the defect with a double-umbrella device was indicated. Following this procedure the cardiomegaly regressed, while colour-Doppler echocardiography demonstrated a minimal residual left to right shunt. The pulmonary artery pressure had fallen to 32/13 mmHg. Three months later the patient was without symptoms and resumed her usual activity. The ECG now showed sinus rhythm, and there was no evidence of a shunt. Anticoagulation with acetyl salicylic acid (100 mg daily) was continued for 3 months.

Aged↗

[Critical observations on the therapy of hydrocephalus using so-called "arrested" hydrocephalus as an example].

A comparative clinical and radiological follow-up study was conducted on 109 hydrocephalic children to study the criteria of shunt dependency. 50 children had a normal functioning shunt, 34 children had an ASD-shunt combination, 14 children had a non-functioning shunt without pressure symptoms and 11 children had the shunt previously removed. None of the cases showed pressure symptoms during examination. The following conclusions could be drawn: 1. The smaller the ventricular system, the higher the complication rate. 2. The standard of well-being did not correspond with the amount of the redevelopment of the compressed brain tissue. 3. A "well-drained" ventricular system was not necessarily a sign of an adequate hydrocephalic therapy, but rather a sign of a pathologic overdrainage. 4. The broader ventricular aspects of the suction-protected ASD shunt resembled the findings of the "arrested hydrocephalus" and seemed to be closer to the goal of hydrocephalus therapy, namely, shunt independency.

Adolescent↗

Do actions speak louder than words? The case of the disappearance of social communication oddities.

Within the last decade, researchers and clinicians have reported an increase in the incidence and diagnosis of Autism Spectrum Disorder (ASD). Various factors have been proposed for this apparent change including broader diagnostic criteria, greater public awareness, biological and environmental interactions, and earlier detection. However, it has been observed in a certain percentage of children, during the toddler and preschool years and before intervention is introduced, that severe language deficits distort social and self-regulatory behavior to such a degree that they mimic the characteristics of ASD. Professional caution is vital in this regard to describe early functioning and to defer diagnosis until the effects of intervention and treatment can be monitored over time. The case of Nicole, a preschooler with developmental delays and social communication oddities, illustrates what I believe is the most professionally responsible, cautious, family-centered, and data-based diagnostic process that links assessment, intervention, and evaluation for young children with early developmental difficulties.

Autistic Disorder↗

Unique echocardiographic features associated with deployment of the Amplatzer atrial septal defect device.

The purpose of this study was to describe the unique echocardiographic findings associated with deployment of the Amplatzer atrial septal defect (ASD) device. Thirty-five patients (2 to 40 years old; 23 female and 12 male patients) underwent echocardiography during attempted ASD closure with the Amplatzer device. Transesophageal and transthoracic echocardiograms were performed during the placement and follow-up of the device, respectively. In 5 patients, the device was not deployed because of transesophageal echocardiography (TEE) findings (an exceedingly large defect in 3 patients, partial obstruction of the upper right pulmonary vein by the device in 1, and complex atrial septal anatomy in 1). In the remaining 30 patients, after deployment but before release, the device distorted the atrial septum from the normal vertical orientation to an oblique transverse orientation. Excessive septal distortion (i.e., > or =90 degrees in 1 patient) was associated with device embolization upon release. In other patients, TEE also identified mild splaying of the device on the aortic wall, mild abutment of the device upon the mitral valve, and temporary partial obstruction of pulmonary vein flow. Color Doppler revealed residual shunts in 21 of 29 patients immediately after release, but in none of 15 patients at 1-year follow-up. Transesophageal echocardiography is essential to ensure proper Amplatzer device placement. Distortion of the atrial septum and Amplatzer device orientation occur before release but resolve on release from the delivery cable. Small residual shunts are common early, but they resolve in 6 to 12 months.

Adolescent↗

Longitudinal motion of the atrioventricular annuli in children: reference values, growth related changes, and effects of right ventricular volume and pressure overload.

The goals of this study were to characterize normal patterns of longitudinal motion of atrioventricular annuli in the young, establish reference values and assess growth-related changes (n = 40). Sensitivity to changes in right ventricular volume and pressure load was assessed in patients with atrial septal defect (ASD) (n = 25) and with primary pulmonary hypertension (PPH) (n = 20). M-mode of the annuli were obtained from apical views. In healthy subjects, longitudinal %displacement decreased with growth, lateral tricuspid annulus had highest %displacement, velocity of annular descent did not change with growth, tricuspid lateral annulus had the highest velocity of descent, only ascent velocity of the lateral tricuspid annulus decreased with growth, velocities of ascent for the lateral annuli were similar and higher than the septum, and heart rate had no effect on parameters tested. In patients with ASD, the findings were an exaggerated normal pattern. In patients with PPH, %displacement and ascent velocities were blunted. Our results demonstrate the utility of this technique to assess annular dynamics in pediatric patients.

Adolescent↗