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Biomedical subjects

U Bauersfeld

Publications and source records attributed to U Bauersfeld.

32 records · Page 2Linked to original sources

Serum and CSF levels of neuron-specific enolase (NSE) in cardiac surgery with cardiopulmonary bypass: a marker of brain injury?

We investigated whether neuron-specific enolase (NSE) in serum or cerebrospinal fluid (CSF) reflects subtle or manifest brain injury in children undergoing cardiac surgery using cardiopulmonary bypass (CPB). NSE was measured in serum (s-NSE) before, and up to, 102 h after surgery in 27 children undergoing cardiac surgery with CPB. In 11 children, CSF-NSE was also measured 48 or 66 h post-surgery. As erythrocytes contain NSE, hemoglobin concentration in the samples was determined spectrophotometrically at 550 nm (cut-off limit: absorbance 0.4 = 560 mg/l) in 14 children and in a further 13 children by spectroscopic multicomponent analysis (cut-off limit 5 micromol/l = 80 mg/l). One hundred and one of 214 post-operative serum samples (47%) had to be discarded because of hemolysis (18% spectrophotometrically at 550 nm and 88% with spectroscopic multicomponent analysis). On the first and second post-operative day, the median s-NSE values were significantly higher when compared with samples taken after 54 h or longer (P = 0.008 and P = 0.002). All CSF-NSE levels were within the normal range and below the s-NSE measured in the same patient. Although in our study elevated s-NSE seems to indicate brain injury in CPB-surgery, the low concentration of NSE in the post-operative CSF of 11 children puts the neuronal origin of s-NSE in question. NSE from other non-neuronal tissues probably contributes to the elevated s-NSE. Additionally, normal post-operative CSF-NSE values in two children with post-operative neurological sequelae might question the predictive value of CSF-NSE with regard to brain injury.

Biomarkers↗

22q11.2 deletions in a series of patients with non-selective congenital heart defects: incidence, type of defects and parental origin.

Previous studies have indicated a wide spectrum of incidences of 22q11.2 deletions in isolated and syndromic (sporadic or familial) cases of conotruncal heart defects, whereby the detection rate of the deletion varied from 65% in one study to 0 in another. We analysed 110 patients with non-selective syndromic or isolated non-familial congenital heart malformations by fluorescence in situ hybridization (FISH) using the D22S75 DiGeorge chromosome (DGS) region probe. A 22q11.2 microdeletion has been detected in 9/51 (17.6%) syndromic patients. Five were of maternal origin and four of paternal origin. None of the 59 patients with isolated congenital cardiac defect had a 22q11.2 deletion. We compared the cardiac anomalies of our patients with a 22q11.2 deletion with those of previously published series and we describe types of congenital heart defects which appear to be often associated with a 22q11.2 deletion. The ability to detect such types of heart defects and to provide an early diagnosis of 22q11.2 deletion is particularly relevant in very young infants, who often show only very mild expression of the otherwise well-characterized phenotypes of the DiGeorge/velo-cardio-facial syndrome (DG/VCFS).

Adolescent↗

Safety issues in the treatment of paediatric supraventricular tachycardias.

Paroxysmal supraventricular tachycardia caused by atrioventricular re-entry is the most frequent arrhythmia in children of all age groups. It represents the most frequent clinical situation where antiarrhythmic drug therapy has to be considered in a child. Acute termination of an episode of tachycardia in all paediatric age groups is nowadays best achieved with an intravenous bolus injection of adenosine. Since the introduction of adenosine into clinical practice, the need to proceed to electrocardioversion has been limited to the infant (or in rare cases an older child) with severe cardiovascular collapse. In the haemodynamically stable infant or child, several other antiarrhythmic agents such as flecainide or propafenone can be used with relative safety and with a high probability of immediate success. The same is true for verapamil, although intravenous administration should be avoided in the first year of life. In newborns and in infants with first presentation of an episode of tachycardia, drug prophylaxis of recurrences is usually recommended for the whole of the first year of life. Prophylactic treatment may consist of oral digoxin as first choice, with a beta-blocker as an alternative. In an infant with Wolff-Parkinson-White syndrome it may be wise to avoid digoxin and to start treatment with a beta-blocker. Antiarrhythmic class Ic drugs such as propafenone or flecainide, and the class III agent sotalol, are widely used as the next steps of therapy when digoxin and beta-blockers fail to prevent recurrences. These agents are about equivalent with regard to their efficacy and risk profile. Amiodarone is considered to be an agent that should be reserved for use in situations when the tachycardia is refractory to the previously named agents. Older children may commence treatment with a beta-blocker and the subsequent steps of treatment are the same as those for infants. Curative catheter ablation of accessory pathways has been shown to be as efficient and well tolerated in the paediatric age group as it is in adults. This treatment option is nowadays quite often offered to older children. However, in infants and smaller children, ablation is used as a last resort. Rare forms of paediatric supraventricular tachycardia (other than atrioventricular re-entry through the atrioventricular node or accessory pathways) are occasionally difficult to treat and present special problems. For each of these arrhythmias, a specially tailored individual therapeutic approach is needed.

Anti-Arrhythmia Agents↗

Congenital aortopulumonary window: diagnosis, surgical technique and long-term results.

OBJECTIVE: Congenital aortopulmonary window is rare, often associated with other cardiac anomalies. Surgical repair as the only treatment should be performed before pulmonary vascular changes have developed. This study presents the long-term outcome after surgical correction for this condition. MATERIALS AND METHODS: Between 1971 and 1993, 13 patients with congenital aortopulmonary window were found. 10 had type I, 2 type II and 1 had type III. Concomitant cardiac anomalies were present in 10/13. Eleven patients were operated on at a mean age of 31.2 +/- 48.3 months (range 6 days-10 years). Thoracotomy was used in 3 and sternotomy in 9 patients. In 4/11, the aorto-pulmonary window was simply ligated, 4 had a transpulmonary approach and 1 combined with a transaortic approach. The aortopulmonary window was closed directly in 2 and with a Dacron patch in 1. Cardiopulmonary bypass was used in 6 patients. Associated anomalies in 10/11 patients. RESULTS: There was one operative death (9%): a six-day old boy with interrupted aortic arch died 6 h postoperatively due to low-cardiac output. The mean follow-up period is 8.1 +/- 7.3 years (range 2-24 years). Clinical examination, transaortic echocardiography and/or cardiac catheterization were obtained in the follow-up. There was no late death. All are in New York Heart Association (NYHA) class I. One had to be reoperated on for a recurrent shunt 29 months after ligation and one had angioplasty after 23 months for residual stenosis of the reimplanted right pulmonary artery. The actuarial survival rate is 90% after 1, 5 and 10 years. CONCLUSIONS: The surgical treatment of aortopulmonary window has a low risk, even if associated with major cardiac anomalies. Prompt operative treatment achieves excellent long-term results.

Actuarial Analysis↗

The effect of the N-methyl-D-aspartate receptor antagonist dextromethorphan on perioperative brain injury in children undergoing cardiac surgery with cardiopulmonary bypass: results of a pilot study.

Experimental evidence indicates a role of the N-methyl-D-aspartate receptor in the pathogenesis of brain injury occurring during cardiac surgery with cardiopulmonary bypass (CPB). Dextromethorphan is a noncompetitive antagonist of this receptor with a favorable safety profile. Thirteen children age 3-36 months undergoing cardiac surgery with expected CPB of 60 minutes or more were randomly assigned to treatment with dextromethorphan (36-38 mg/kg/day) or placebo administered by naso-gastric tube. Dextromethorphan was absorbed well and reached putative therapeutic levels in blood and cerebrospinal fluid. Adverse effects were not observed. Mild hemiparesis developed after operation in one child of each group, and severe encephalopathy in one of the placebo group. Sharp waves were recorded in postoperative continuous electroencephalography in all placebo (n = 7) but only in 2/6 dextromethorphan treated children (p = 0.02). Pre- and postoperative cranial magnetic resonance imaging (MRI) revealed less pronounced ventricular enlargement in the dextromethorphan group (not significant). An increase of periventricular white matter lesions was visible in two placebo-treated children only. No elevations of cerebrospinal fluid enzymes were observed in either group. Although children with dextromethorphan showed less abnormalities in electroencephalography and MRI, dissimilarities of the treatment groups by chance diminished conclusions to possible protective effects of dextromethorphan at this time.

Brain Chemistry↗

Ivemark syndrome. A case with successful surgical intervention.

A 19-day-old boy with Ivemark syndrome (splenic agenesis associated with complex cardiac malformations and visceral abnormality) underwent palliative surgery including Glenn and hemi-Fontan procedures. Five months later the child is alive and well. We believe that early palliative surgery is worthwhile in Ivemark syndrome with a single ventricle.

Abnormalities, Multiple↗

[Sudden loss of consciousness in childhood].

Sudden loss of consciousness in childhood presents itself usually as a syncope and occurs in approximately 15% of all children. Although syncope in the pediatric age group appears to be an isolated phenomenon with good prognosis, it could also be a manifestation of a life-threatening disease. Thus, even the first syncope should be evaluated in children. A thorough history and a careful physical examination with special attention to cardiovascular and neurologic abnormalities usually point to the etiology of the syncope and must result in the appropriate laboratory tests. About 50% of pediatric syncopes are non-cardiovascular, 20 to 30% are cardiovascular, and 20 to 30% are of unknown etiology. The most frequent syncope in childhood is neurocardiogenic. It is important to distinguish neurocardiogenic syncope from cardiac syncope and epileptic seizures. Neurocardiogenic and cardiac syncopes can be discriminated from epileptic seizures by their usually shorter duration of unconsciousness and rare postsyncopal disorientation. Cardiac syncope is rare in children with a structurally normal heart and may be associated with Wolff-Parkinson-White syndrome, long QT syndrome or congenital AV block. Children with congenital heart defects and cardiomyopathies who present with syncope must raise a high degree of suspicion for a cardiac syncope. Cardiac syncopes often yield a poor prognosis with substantial percentages of sudden death; therefore, a vigorous attempt has to be made to diagnose and adequately treat cardiac syncope in children.

Child↗

[Current pacemaker treatment and technology].

The increasing use of microprocessor technology in pacemaker devices results, by multiple programmability, in a more reliable follow-up of pacemaker systems and accounts for individual patient program settings. Rate-adaptive and AV-sequential pacing allows physiologic pacing. The implantation of dual-chamber pacemakers is no longer precluded in patients with atrial tachyarrhythmias with the availability of pacemakers with automatic mode switching (DDD[R] to DDI[R]). The actual vast variability of pacemaker treatment modalities requires a careful evaluation of the indication for pacemaker implantation, with international guidelines being helpful in decision-making. Besides frequent pacemaker indications, the occasional patient with hypersensitive carotid sinus syndrome, neurocardiogenic syncope and hypertrophic cardiomyopathy may benefit from pacemaker treatment. Temporary pacemaker functions with programmable stimulation extend the diagnostic and therapeutic horizon of various arrhythmia treatments.

Adult↗

Kartagener's syndrome: clinical presentation and cardiosurgical aspects.

BACKGROUND: Long-term observations in patients with Kartagener's syndrome (situs inversus, bronchiectasis, and sinusitis) are rare. The role of additional cardiac malformations and their surgical repair is not well known. METHODS: Nine patients (5 female and 4 male) with Kartagener's syndrome were identified and followed. Four patients had associated cardiac anomalies; 4 underwent total surgical repair at the ages of 4 (2 patients), 7, and 34 years. RESULTS: The postoperative period was uneventful, and these 4 patients are doing well 7 months and 2, 9, and 19 years after repair. The other patients are being treated with conservative therapy and are in relatively good condition. CONCLUSIONS: This disease can be temporarily benign when treated with antibiotics and physiotherapy. Associated cardiac anomalies seem to be quite common, and such patients need careful cardiologic follow-up. Surgical intervention can be safely performed in patients suffering from Kartagener's syndrome associated with a congenital cardiac malformation and produces good long-term results. Bilateral lung transplantation seems to be the therapy of choice in patients with respiratory insufficiency but without concomitant cardiac anomalies.

Aftercare↗

Treatment of atrial ectopic tachycardia in infants < 6 months old.

Nineteen infants < 6 months old who had atrial ectopic tachycardia (AET) were treated with antiarrhythmic drugs. AET was controlled with digoxin in 1 patient, propafenone in 2, digoxin with propafenone in 9, digoxin with amiodarone in 4, and digoxin with propafenone and amiodarone in 2; radiofrequency ablation was performed in 1 drug-resistant case. AET resolved in 14 of 15 infants within 1 year. Drug side effects occurred in 5 patients. We conclude that AET in infants < 6 months old can be successfully treated with antiarrhythmic drugs, and we recommend a three-step approach with digoxin, a class 1C antiarrhythmic drug, and a class 3 drug. Resolution of AET in infants is frequent.

Amiodarone↗

[Anti-arrhythmia agents in the therapy of ventricular arrhythmias in the post-CAST era].

This review provides a 20-year perspective on the most significant reports on antiarrhythmic drug therapy of ventricular arrhythmias, with particular reference to the CAST trials. The following conclusions are drawn: 1. Based on the CAST trials and other studies investigating the effectiveness of antiarrhythmic agents during the last 20 years, prophylactic treatment with antiarrhythmic class I, III and IV drugs after myocardial infarctions is not indicated. 2. Betablockers without intrinsic sympathomimetic activity are effective in the prevention of reinfarction and sudden cardiac death. 3. The effectiveness of amiodarone as secondary prophylaxis is currently under investigation in several multicenter trials. 4. Antiarrhythmic intervention is indicated in patients with hemodynamically compromising ventricular tachycardia or ventricular fibrillation, irrespective of spontaneous ventricular arrhythmias. Effective treatment with arrhythmic agents can be achieved in approximately half of the patients. Arrhythmia surgery, implantable cardioverters/defibrillators, or heart transplantation are possible alternative treatment modalities.

Adrenergic beta-Antagonists↗

[Pericardectomy and acute infectious pericarditis].

Between 1980 and 1990 12 patients (5 male, 7 female) were operated on for acute infectious pericarditis at a mean age of 42 years. The infections were 6 bacterial (purulent 4, abscess 2), 4 tuberculous, 1 viral and 1 Candida. Pericarditis resulted from contiguous spread of infection from bilateral pneumonia in 3 patients, from subphrenic abscess in 2 and followed bacteremia in 1. Clinical signs were: tamponade/shock in 9, elevated jugular venous pressure in 11, edema in 6, hepatomegaly in 6, ascites in 1, and pericardial friction rub in 3. A preoperative pericardiocentesis in 9 patients allowed only 4 positive microbiological diagnoses and was an insufficient drainage in all cases. The preoperative mean NYHA class was 3.3. The pericardectomy was total in 9 patients and partial in 3. Total mortality was 1/12 patients (8%) with one late death due to recurrent tuberculous pericarditis. No patient with purulent pericarditis died. Another recurrence occurred 6 months after acute viral pericarditis. Atrial fibrillation in one patient was the only postoperative complication. After a mean follow-up period of 48.5 months no cardiac constriction had occurred in 11 surviving patients Actuarial survival after pericardectomy is 100% after 1 month and remains 91% after 5 years. The mean NYHA class has significantly improved to 1.2 (p less than 0.05) at the end of the follow-up. We conclude that pericardectomy combined with a specific antimicrobial therapy is a safe treatment for acute infectious and especially purulent pericarditis with low mortality and excellent longterm results. Early pericardectomy allows rapid decompression of the heart, removal of intrapericardial adhesions and infected tissue and prevents late constriction.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗

Cardiac surgery for Kartagener syndrome.

Two patients (one girl, one boy) with Kartagener syndrome (situs inversus, bronchiectasis, sinusitis), despite pulmonary problems and associated congenital cardiac anomalies, were operated on at the ages of 4 years and 7 years, respectively. They had had previous palliative treatment at the age of 3 months and 1.3 years, respectively. Both postoperative periods after total correction were without significant complications. Long-term follow-up was available for 9 and 19 years, respectively, with no manifestations of heart insufficiency. Both patients are physically active, and neither requires cardiac medication. Patients with Kartagener syndrome and associated congenital cardiac anomalies can successfully undergo multiple cardiac operations with good long-term outcome.

Cardiac Surgical Procedures↗