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PubMed2003

Platypnea-orthodeoxia in a patient with ostium primum atrial septal defect with normal right heart pressures.

We describe an adult patient with an ostium primum atrial septal defect (ASD) and a patent foramen ovale (PFO) with normal right heart pressures who presented with platypnea and orthodeoxia. A dilated aortic root encroaching into the region of the ASD and PFO, along with a tricuspid regurgitant jet moving into the left atrium through the ASD found on the transesophageal echocardiogram may have been responsible for orthodeoxia. Surgical closure of these defects resulted in the disappearance of both platypnea and orthodeoxia.

Echocardiography, Transesophageal↗
PubMed2002

Intraoperative measurements of the subacromial distance.

PURPOSE: The purpose of this study was 2-fold: to document the accuracy of a new measuring device and to intraoperatively compare the subacromial distance between controls and patients with impingement syndrome before and after arthroscopic subacromial decompression (ASD). TYPE OF STUDY: Clinical study. METHODS: When performing an ASD, it is important that bone resection is adequate. Today the correct subacromial distance after bone resection is only assessed by eye, directly or indirectly. The subacromial distance was measured between the anterolateral corner of the acromion and the supraspinatus tendon in the lateral decubitus position. The device was inserted 2 to 3 cm below the anterolateral acromion. There was no subacromial pathology among the controls (n = 15, mean age, 28 years). In 30 patients with impingement syndrome (average age, 53 years) an ASD was performed. The subacromial distance was measured after bursectomy and then after bone resection. Intraindividual and interindividual assessment was performed. RESULTS: The mean value of the subacromial distance in controls was 16 mm, the 95% confidence limits between 14 and 18 mm. The mean value in the group of patients with impingement syndrome was 8 mm before and 16 mm after the decompression. Due to the pressure within the subacromial space, the subacromial distance increased 1 mm. Intraindividual measurements never varied more than 1 mm (n = 5). The correlation coefficient between the measurements by both authors was 0.99. CONCLUSIONS: In this study, we assessed and described the use of a measuring device that enables the surgeon to quantify the subacromial distance before and after bone resection. After bone resection, the mean value of the subacromial distance was well within the control values. The amount of bone resected varied from 5 to 13 mm. This new device enables documentation in clinical work as well as in research.

Acromioclavicular Joint↗
PubMedDate not supplied

Red blood cell fatty acid compositions in a patient with autistic spectrum disorder: a characteristic abnormality in neurodevelopmental disorders?

The fatty acid compositions of red blood cell (RBC) phospholipids from a patient with autistic spectrum disorder (ASD) had reduced percentages of highly unsaturated fatty acids (HUFA) compared to control samples. The percentage of HUFA in the RBC from the autistic patient was dramatically reduced (up to 70%) when the sample was stored for 6 weeks at -20 degrees C. However, only minor HUFA reductions were recorded in control samples stored similarly, or when the autistic sample was stored at -80 degrees C. A similar instability in RBC HUFA compositions upon storage at -20 degrees C has been recorded in schizophrenic patients. In a number of other neurodevelopmental conditions, including attention deficit hyperactivity disorder (ADHD) and dyslexia, reduced concentrations of RBC HUFA have been recorded. The extent and nature of these aberrations require further assessment to determine a possible common biochemical origin of neurodevelopmental disorders in general. To facilitate this, a large scale assessment of RBC fatty acid compositions in patients with ASD, and related disorders, should be performed as a matter of urgency. Supplementing cells in culture with the tryptophan metabolite indole acrylic acid (IAA) affected the levels of cellular HUFA and prostaglandin production. Indole acroyl glycine (IAG), a metabolite of IAA excreted in urine, is found in high concentrations in patients with neurodevelopmental disorders including ASD, ADHD, dyslexia, Asperger's syndrome and obsessive compulsive disorder.

Asperger Syndrome↗
PubMed2002

[A hemodynamically active type II atrial septal defect in a 78-year-old patient. Indications for interventional catheter occlusion?].

HISTORY AND ADMISSION FINDINGS: A 78-year-old woman presented with a first episode of syncope. She reported increasing fatigue and dyspnoea upon exertion over a period of 20 years and chest pain 2 months prior to admission. Auscultation revealed fixed doubling of the second heart sound. INVESTIGATIONS: Laboratory tests showed increased troponin I. Transaminases were moderately elevated. Chest X-ray showed an enlarged right heart and a dilated pulmonary artery (2 cm). Echocardiography discovered a large secundum atrial septal defect with a diameter of 3 cm but no right to left shunt (no Eisenmenger reaction). Cardiac catheterization revealed a stenosis of the right coronary artery and severe systolic pulmonary hypertension of 80 mmHg. DIAGNOSIS, TREATMENT AND COURSE: A significant stenosis of the right coronary artery was successfully dilated. The ASD was closed by interventional implantation of a commercial closure device (Amplatzer). One month later, echocardiography indicated in an estimated systolic pulmonary pressure of 30 mmHg. The patient's condition improved considerably. CONCLUSION: This case is remarkable in that a very large ASD was asymptomatic up into old age and without the development of an Eisenmenger reaction. Also, large ASD can be by catheterization with the appropriate closure device. Fixed pulmonary hypertension is not obligatory. Non-invasive closure is a good alternative of surgery in elderly patients with risk factors.

Aged↗
PubMed2002

Minimally invasive surgery for congenital heart defects in paediatric patients.

BACKGROUND: In selected cases, minimally-invasive approaches are favoured for the correction of congenital heart defects with regard to better cosmetic results. METHODS: Between July 1999 and April 2001, 25 children (9 male; mean age 5.8 +/- 4.1 years; mean weight 19.6 +/- 12.6 kg) were operated on using minimally invasive approaches. Diagnoses were: ASD (n = 19), VSD (n = 2), ostium primum defect (n = 3) and Tetralogy of Fallot (n = 1). Female patients with ASD underwent a limited right anterolateral thoracotomy. A ministernotomy was chosen in male patients, in patients under 6 months of age, and in patients with malformations other than ASD. Cannulation was always performed via the chest incision. RESULTS: There were no perioperative complications. Mean operation time was 3.23 +/- 0.89 h. Twelve patients were extubated immediately after surgery, mean ventilation time in the others was 12.1 +/- 11.7 h. Mean ICU stay and hospital stay were 1.5 +/- 0.75 days and 8.3 +/- 2.2 days, respectively. Follow-up (mean 4.8 +/- 4.6 months) was uneventful. CONCLUSIONS: Intracardiac repair of some congenital heart defects can be performed safely and effectively via minimally-invasive approaches. Indications are expanding towards more complex defects. Exposure for precise repair is good, additional incisions can be avoided, and cosmetic results have been excellent.

Adolescent↗
PubMed2002

[The role of MRI for the evaluation of atrial septal defects before and after percutaneous occlusion with the amplatzer septal occluder(R)].

PURPOSE: Evaluation of morphologic and functional MRI of atrial septal defects (ASD) before and after percutaneous occlusion with the Amplatzer Septal Occluder (AOC). Comparison of MRI with transesophageal echocardiography (TEE), balloon measurement (IVBM) and cardiac catheterization with shunt quantification (CCSQ). MATERIALS AND METHODS: Twenty patients with ASD were examined before and three months after AOC implantation. ECG-triggered, breath-hold T1-weighted Turbo Spin Echo Segmented FLASH 2D and dynamic turbo-FLASH-GRE sequences after application of 0.2 mmol gadolinium DTPA per kg body weight were obtained in a 1.5 T MRI system. Defect size, and distance to coronal sinus (CS) and right upper pulmonary vein (RUPV) were determined for pre-interventional planning, and the AOC size was measured quantitatively for post-interventional follow-up. The shunts were evaluated qualitatively (occurrence of jets), semiquantitatively (jet length, turbulence square product) and quantitatively (flow measurement in the thoracic aorta and in the left and right pulmonary arteries). RESULTS: The average size of the ASD measured by MRI was 17.6 mm (11 - 24.8 mm) in the axial view, 15.9 mm (10.8 - 28.9 mm) in the sagittal view and 16.4 mm (12.1 - 24.8 mm) in the short axis view. In comparison, the average defect size was 15 mm (8 - 24 mm) by TEE and 20 mm (13 - 27 mm) by IVBM. The average distance to the RUPV was 17 mm (9.6 - 21.9 mm) and to the CS 11.2 mm (5 - 17 mm). The AOC was visualized with only minimal artifacts. Qualitative analysis of the MRI findings revealed an occurrence of jets in 17/20 patients. Semiquantitative analysis documented a high correlation for jet length and square product of the turbulence to defect size r = 0.81 resp. r = 0.82. Mean QP/QS-ratio measured by MR-volumetry was 1.6 +/- 0.29 and by MR-flow 1.6 +/- 0.26. The corresponding measurements were 1.7 +/- 0.3 for TEE and 1.5 +/- 0.5 for CCSQ. In comparison to TEE, the correlation coefficient was r = 0.96 for MR-volumetry and r = 0.85 for MR-flow measurement. CONCLUSION: MRI is found to be a valuable diagnostic method for pre-interventional planning and post-interventional follow-up of atrial septal defects occluded by the Amplatzer septal occluder.

Adult↗
PubMed2006

Risk factors for atrial fibrillation in adult patients in long-term observation following surgical closure of atrial septal defect type II.

BACKGROUND: The aim of the study was to find the factors predictive for paroxysmal atrial fibrillation (AF) following surgical correction of atrial septal defect type II (ASD t.II). METHODS: 93 patients, who underwent isolated surgical closure of ASD t.II between 1990 and 2001 were included. Follow-up studies were performed 2 - 11 years after surgery. Patients were divided into two groups according to the presence of AF before and after surgery. Group AF (+) consisted of 29 and group AF (-) of 64 patients. All patients underwent echocardiography, electrocardiogram (ECG) at rest, and signal-averaged P-wave duration (PWD) in signal-averaged ECG. The following parameters were assessed in echocardiography: pulmonary artery systolic pressure, left and right atrial dimensions, right ventricular dimension, tricuspid and mitral regurgitation. RESULTS: Paroxysmal AF was observed in 27 patients before surgery and in 29 after surgery. Analyzing all potential risk factors we proved that PWD may independently predict occurrence of postoperative AF. CONCLUSION: PWD may independently predict postoperative AF in long-term follow-up after surgical correction of ASD t.II.

Adult↗
PubMed2006

[MRI safety aspects and artifacts of atrial septal defect and patent foramen ovale occluders at 1.5 tesla: a phantom study].

PURPOSE: To investigate the magnetic resonance safety and magnetic resonance artifact intensities of patent foramen ovale and atrial septal occluders in a phantom study. MATERIALS AND METHODS: Seven different commercial occluder models (Amplatzer ASD, Angel Wing ASD, Helex Septal Occluder, Cardia PFO-Star Generation I & III, Sideris Buttoned Device, Starflex ASD) were tested in a 1.5 Tesla whole-body MR scanner. Following a deflection test, the non-magnetic devices were imaged using different MRI pulse sequences including conventional spin echo, spoiled gradient echo as well as fast refocused steady-state pulse sequences (trueFISP) commonly used for cardiac MRI. Additionally, device temperatures were measured in the MR scanner during two imaging protocols (trueFISP, HASTE) with an MR-compatible fiber-optical thermometer. RESULTS: One ferromagnetic device (Sideris Buttoned Device) did not pass the deflection test and was excluded from further experiments. For typical cardiac imaging protocols, the remaining non-ferromagnetic devices showed only minor image artifacts with artifact sizes not exceeding 2 mm next to a strut. Heating was not observed in the devices. CONCLUSION: Non-ferromagnetic occluder models used in this study can safely be imaged with typical cardiac MRI protocols at 1.5 Tesla. The device artifact sizes are small enough to allow anatomical and functional MRI even in the immediate vicinity of the occluders.

Artifacts↗
PubMed1999

[Diagnosis and surgical treatment of atrial septal defects in adults].

BACKGROUND AND OBJECTIVE: The value of surgical closure of an atrial septal defect (ASD) in adults is currently under discussion, because the operative risk is thought to be high and there are no reliable data about postoperative change in quality of life. PATIENTS AND METHODS: The case notes of 205 patients (149 women, 56 men, mean age 41.4 years) in whom an ASD had been surgically closed were analysed retrospectively. Preoperative symptoms, complications, arrhythmias, pulmonary artery pressure and the influence of the operative closure on quality of life (criteria of the New York Heart Association [NYHA]) were noted. RESULTS: At the time of diagnosis 138 (66%) of patients had complained of dyspnoea or palpitations, four had sustained a stroke and two had had endocarditis. Atrial fibrillation or flutter was present in 55 (27%). 47 (23%) were in class III or IV (NYHA). Mean age at operation was 42.2 (18-74.9) years. One patient, a woman with pulmonary hypertension, died and one patient suffered a stroke. The number of patients with atrial fibrillation or flutter fell to 31 (15%) postoperatively and 176 patients (86%) moved to a better NYHA class, especially those who had been in class IV. CONCLUSIONS: These data show that surgical closure of ASD in adults is reasonable, because the operative mortality is low, the quality of life is improved postoperatively and there is a reduced incidence of arrhythmias.

Adolescent↗
PubMedDate not supplied

[Arthroscopic subacromial decompression. 1-3 year results].

In 148 patients with impingement lesion type I or type II, we performed an arthroscopic subacromial decompression (ASD). 122 patients ran a follow up one to three years post-operatively. All patients were pre- and postoperatively documented by a 100 point shoulder score. The mean score was 57.9 (+/- 11.5) preoperatively. Postoperatively there was a significant increase to 80.7 (+/- 17.9) (p < 0.05). 15% of the patients with a postoperative score less than 70 points were determined as failures. Patients with a preoperative pain history of more than one year had a significantly worse result (79.1 +/- 8.4) compared to those patients with a preoperative course less than one year (88.8 +/- 11.6) (p < 0.05). Other significant factors were the patient's age, and calcific tendinitis, whereas sex, preoperative range of motion, muscle atrophy, and degeneration of the acromioclavicular joint did not significantly influence the result. Our results after ASD in patients with subacromial pathology without a rupture of the rotator cuff are encouraging. Therefore, ASD seems to be a reasonable alternative to open acromioplasty.

Acromion↗
PubMed2001

Emergency department evaluation of acute stress disorder symptoms in violently injured youths.

STUDY OBJECTIVE: We assess the feasibility of evaluating acute stress disorder (ASD) symptoms in the emergency department and provide an initial estimate of the prevalence, severity, and variability of these symptoms in violently injured urban children and young adults. METHODS: The Immediate Stress Response Checklist (ISRC) was administered to violently injured patients between the ages of 8 and 24 years who presented to 2 urban, academic medical center EDs during a 10-week period. The ISRC is designed to assess ASD symptoms in children and youths immediately after a traumatic event. Responses are described using summed symptom severity scores and counts of symptoms. RESULTS: Out of 109 identified youths, 81 participated. Fear, helplessness, or horror during the injury event was reported by 59 (73%) of study participants. Each of the following categories of ASD symptoms was reported by a significant number of youths: peritrauma dissociation, 63 (78%); posttrauma dissociation, 33 (41%); re-experiencing intrusive thoughts or images, 66 (82%); avoidance, 53 (65%); and hyperarousal, 32 (39%). The ISRC demonstrated high internal consistency (Cronbach alpha=.88) in this cohort. CONCLUSION: The ISRC can be used to assess acute stress reactions in violently injured youths in the immediate period after injury. Although these reactions appear to be common, there is substantial variability among distinct symptom categories. These results support the need for further investigation of the relationship between immediate stress responses and longer-term emotional impact of violent injury. Clinicians should consider assessing the emotional responses of children and adolescents who are injured by interpersonal violence, even when these injuries seem minor.

Academic Medical Centers↗
PubMed2006

Soluble aldose sugar dehydrogenase from Escherichia coli: a highly exposed active site conferring broad substrate specificity.

A water-soluble aldose sugar dehydrogenase (Asd) has been purified for the first time from Escherichia coli. The enzyme is able to act upon a broad range of aldose sugars, encompassing hexoses, pentoses, disaccharides, and trisaccharides, and is able to oxidize glucose to gluconolactone with subsequent hydrolysis to gluconic acid. The enzyme shows the ability to bind pyrroloquinoline quinone (PQQ) in the presence of Ca2+ in a manner that is proportional to its catalytic activity. The x-ray structure has been determined in the apo-form and as the PQQ-bound active holoenzyme. The beta-propeller fold of this protein is conserved between E. coli Asd and Acinetobacter calcoaceticus soluble glucose dehydrogenase (sGdh), with major structural differences lying in loop and surface-exposed regions. Many of the residues involved in binding the cofactor are conserved between the two enzymes, but significant differences exist in residues likely to contact substrates. PQQ is bound in a large cleft in the protein surface and is uniquely solvent-accessible compared with other PQQ enzymes. The exposed and charged nature of the active site and the activity profile of this enzyme indicate possible factors that underlie a low affinity for glucose but generic broad substrate specificity for aldose sugars. These structural and catalytic properties of the enzymes have led us to propose that E. coli Asd provides a prototype structure for a new subgroup of PQQ-dependent soluble dehydrogenases that is distinct from the A. calcoaceticus sGdh subgroup.

Acinetobacter calcoaceticus↗
PubMed2002

Profiles of everyday executive function in acquired and developmental disorders.

Executive function profiles were examined within and between several clinical disorders via the multi-domain Behavior Rating Inventory of Executive Function (BRIEF; Gioia, Isquith, Guy, & Kenworthy, 2000). Parent ratings of children with Inattentive and Combined types of Attention-Deficit/Hyperactivity Disorder (ADHD-I, ADHD-C), Autistic Spectrum Disorders (ASD), moderate and severe Traumatic Brain Injury (TBI), and Reading Disabilities (RD) were compared with controls. Profile analysis revealed significant (p<.01) and substantial (eta2>.10) differences in global elevations and in profile of scale elevations between diagnostic groups. ASD, ADHD-I and ADHD-C groups exhibited greater elevations across the BRIEF scales than did RD and Severe TBI groups, who were in turn more elevated than Moderate TBI and Control groups. The ADHD-C group was unique in its frequency and severity of inhibitory deficits, while the ASD group was distinguishable by its deficits in flexibility. Within diagnostic groups, relative risk for executive dysfunction was calculated with variability present in the frequency of clinically elevated scales. While the BRIEF captures executive profiles characteristic of specific disorders in the clinical setting, it is not diagnostic in its own right and is best used within the context of a broad based evaluation.

Attention Deficit Disorder with Hyperactivity↗
PubMed2002

Repair of atrial septal defects via limited right anterolateral thoracotomy.

INTRODUCTION: Right thoracotomy is a well known alternative for median sternotomy to gain access to the right atrium. The Port-Access technique is a surgical option to reduce the skin incision to 5 cm and have a considerable gain in cosmesis and post-operative rehabilitation. PATIENTS AND METHODS: From February 1997 to May 2000, 50 patients (19M/31F) had Port-Access atrial septal defect repair (ASD) with the Heartport Endo-CPB and Endo-aortic clamp system. Mean age was 46 years (range 10.5-74 years). Forty-nine patients had a type II ASD. Most of the patients were asymptomatic (48 were NYHA class I-II). Five patients underwent combined procedures: two mitral valve repairs, one mitral valve replacement, one plasty of the superior vena cava and one mini Cox-Maze. Direct closure was obtained in 34 patients (68%). Mean aortic cross clamp time was 56 minutes (range 24-134 min) and mean perfusion time was 102 minutes (range 32-196 min). RESULTS: There were no conversions to sternotomy. The procedure was complicated in six patients: revision for bleeding (n = 1), stenting of the iliac vein (n = 1), enlargement plasty of the femoral artery (n = 1), transient renal failure (n = 1), sick sinus syndrome requiring pacemaker implantation (n = 1) and one lymphocoele. No thromboembolic or peripheral ischaemic complications were noted. Per- and postoperative echocardiography showed no leakage in any patients. There was no hospital mortality. Mean ICU and hospital stay were 1.14 days (range 1-3 days) and 6.41 days (range 4-10 days) respectively. CONCLUSION: The Port-Access approach of ASD closure constitutes a valid alternative to sternotomy with the same standards of results and quality.

Adolescent↗
PubMed2001

Atrial septal defect presenting with Brucella endocarditis.

Brucella endocarditis, although a rare complication of brucellosis, is the main case of death attributable to this disease. An atrial septal defect (ASD) presenting with endocarditis in an adult is also extremely rare. We report the case of a male patient who presented with an infective endocarditis and was found to have an ASD. The diagnosis was proven by means of positive serology and isolation of Brucella melitensis from blood cultures and excised vegetation arising from the border of the ASD. The patient was successfully treated by means of excision of the vegetation and specific antibiotic therapy for 3 months.

Brucella melitensis↗
PubMedDate not supplied

Thrombotic microangiopathy in adult Still's disease.

Adult Still's disease (ASD) is a rare systemic disorder characterized by fever, arthralgia, cutaneous rash, and lymphadenopathy, with high polymorphonuclear leucocytosis and low glycosylated ferritinaemia. Kidney involvement has been reported rarely. We present a patient with ASD who developed haemolytic uraemic syndrome (HUS). The 42-year-old patient was admitted for unexplained fever related to ASD according to Yamaguchi's classification criteria. As Still's disease was resistant to prednisone, high-dose intravenous immunoglobulins (IV Ig) were administered. During the follow-up the patient developed acute renal failure and non-immune haemolytic anaemia with high levels of antiphospholipid antibodies (IgG anticardiolipin antibodies and anti-beta2 glycoprotein 1 antibodies). Renal biopsy disclosed thrombotic microangiopathy (TMA) with arteriolar and glomerular involvement. Treatment with steroids and intravenous IV Ig was reinitiated but renal function worsened towards end-stage renal failure. In this case, we suggest that antiphospholipid antibodies could have promoted arteriolar and glomerular TMA. HUS may be the cause of acute renal failure in Still's disease.

Acute Kidney Injury↗
PubMed1991

Complications of third molar surgery among university students.

The authors analyzed complications during and after 145 consecutive operative removals of mandibular third molars performed on 109 healthy university students in Finland. The operations were all performed by the same oral surgeon under similar conditions and with similar postoperative procedures. The rate of intraoperative complications was 9%. All complications could be taken care of during the operations, and all the postoperative complications were reversible. Swelling was the most common postoperative complication and was related to the length and difficulty of the operation. Trismus at the control visit (6th postoperative day) was also related to the length of the operation. Mandibular lateral movement was more restricted to the nonoperated than to the operated side. Alveolitis sicca dolorosa (ASD) was diagnosed as true ASD in 5% of the cases and as nonspecific ASD in 15% of the cases.

Adult↗
PubMed2002

Cardiopulmonary exercise parameters in children with atrial septal defect and increased pulmonary blood flow: short-term effects of defect closure.

UNLABELLED: Markedly increased pulmonary blood flow because of a relevant atrial septal defect (ASD) leads to impaired cardiopulmonary function during maximum exercise in adults. No comparative preoperative and postoperative data are available on the short-term effects of shunt closure on cardiorespiratory function at peak exercise in children. Pulmonary function testing at rest and cardiopulmonary exercise testing together with haemodynamic assessment was done prospectively in children with an ASD preoperatively and again after full recovery at 3-4 mo postoperatively and compared with a matched normal population. Sixteen children, aged 6.8-16.1 y, with a defect of 8-23 mm (median 15 mm) and a pulmonary/systemic flow ratio of 1.5-3.5 (median 2.2) were tested and compared with 15 healthy children. Preoperatively, baseline pulmonary function parameters and exercise capacity were no different from normals. At peak exercise, patients with a shunt had increased pulmonary resistance, especially of the distal airways (p = 0.04), with a significantly larger proportion of children having a paradoxical increase in total airway resistance during exercise (p < 0.05). Maximum serum lactate at peak exercise was elevated (p < 0.05) in patients. In patients, maximum oxygen uptake was impaired (p = 0.03) and remained so at repeat evaluation postoperatively. The same observation was made for chronotropic response to exercise. CONCLUSION: Cardiopulmonary exercise parameters in patients with ASD differed only slightly from those in normal children. The most important deviations were a lower maximum oxygen uptake and an increase in airway resistance at maximum exercise.

Adolescent↗