Urs Bauersfeld

Psychological adjustment and quality of life in children and adolescents following open-heart surgery for congenital heart disease: a systematic review

BackgroundChildren with congenital heart defects (CHD) requiring open-heart surgery are a group at high risk for health-related sequelae. Little consensus exists regarding their long-term psychological adjustment (PA) and health-related quality of life (QoL). Thus, we conducted a systematic review to determine the current knowledge on long-term outcome in this population.MethodsWe included randomized controlled trials, case control, or cohort studies published between 1990–2008 evaluating self- and proxy-reported PA and QoL in patients aged between two and 17 years with a follow-up of at least two years after open heart surgery for CHD.ResultsTwenty-three studies assessing psychological parameters and 12 studies assessing QoL were included. Methodological quality of the studies varied greatly with most studies showing a moderate quality. Results were as follows: (a) A considerable proportion of children experienced psychological maladjustment according to their parents; (b) studies on self-reported PA indicate a good outcome; (c) the studies on QoL suggest an impaired QoL for some children in particular for those with more severe cardiac disease; (d) parental reports of psychological maladjustment were related to severity of CHD and developmental delay.ConclusionA significant proportion of survivors of open-heart surgery for CHD are at risk for psychological maladjustment and impaired QoL. Future research needs to focus on self-reports, QoL data and adolescents.

The contribution of pulse oximetry to the early detection of congenital heart disease in newborns

Approximately half of all newborns with congenital heart disease are asymptomatic in the first few days of life. Early detection of ductal-dependant cardiac malformations prior to ductal closure is, however, of significant clinical importance, as the treatment outcome is related to the time of diagnosis. Pulse oximetry has been proposed for early detection of congenital heart disease. The aims of the present study were: 1) to determine the effectiveness of a pulse-oximetric screening performed on the first day of life for the detection of congenital heart disease in otherwise healthy newborns and 2) to determine if a pulse-oximetric screening combined with clinical examination is superior in the diagnosis of congenital heart disease to clinical examination alone. This is a prospective, multi-centre study. Postductal pulse oximetry was performed between six and twelve hours of age in all newborns of greater than 35 weeks gestation. If pulse-oximetry-measured arterial oxygen saturation was less than 95%, echocardiography was performed. Pulse oximetry was performed in 3,262 newborns. Twenty-four infants (0.7%) had repeated saturations of less than 95%. Of these infants, 17 had congenital heart disease and five of the remaining seven had persistent pulmonary hypertension. No infant with a ductal-dependant or cyanotic congenital heart disease exhibited saturation values greater or equal to 95%. Conclusion: postductal pulse-oximetric screening in the first few days of life is an effective means for detecting cyanotic congenital heart disease in otherwise healthy newborns.

Early postoperative arrhythmias after cardiac operation in children

BACKGROUND Arrhythmias are a recognized complication of cardiac operations. However, little is known about the incidence, treatment, and risk factors for early postoperative arrhythmias in children after cardiac operations. METHODS Diagnosis and treatment of early postoperative arrhythmias were prospectively analyzed in an intensive care unit in 100 consecutive children with a median age of 17 months (range, 1 day to 191 months) who had undergone cardiac operation. Patients were grouped in three different categories of surgical complexity. RESULTS During a median postoperative time of 1 day (range, 0 to 15 days), 64 critical arrhythmias occurred in 48 patients. Arrhythmias consisted of sinus bradycardia in 30, atrioventricular block II to III in 7, supraventricular tachyarrhythmias in 14, and premature complexes in 13 instances. Treatment of 52 arrhythmias was successful and included pacing in 41, intravenous amiodarone in 8, body cooling in 5, overdrive pacing in 3, and electrolyte correction in 2 cases, with more than one treatment modality in 8 cases. Risk factors for arrhythmias were lower body weight (p < 0.05), longer cardiopulmonary bypass duration (p < 0.05), and a category of higher surgical complexity (p < 0.001). CONCLUSIONS Early postoperative arrhythmias occur frequently after cardiac operations in children. Sinus bradycardia, atrioventricular block II to III, and supraventricular tachyarrhythmias are the most frequent arrhythmias, which, however, can be treated effectively by means of temporary pacing, cooling, and antiarrhythmic drug therapy. Lower body weight, longer cardiopulmonary bypass duration, and a higher surgical complexity are risk factors for early postoperative arrhythmias.

A 12-year experience of bipolar steroid-eluting epicardial pacing leads in children.

BACKGROUND Cardiovascular abnormalities and small vascular size may preclude transvenous pacing and necessitate epicardial lead implantation. This study evaluates the performance of steroid-eluting, bipolar epicardial pacing leads. METHODS We prospectively enrolled 114 children with 239 atrial and ventricular bipolar epicardial leads (Medtronic CapSure 10366 or 4968, Minneapolis, MN), followed up to 12.2 years (median, 3.2). Lead data were obtained at implant and at semi-annual visits. Analysis was done for left or right atrial and ventricular leads. RESULTS Median atrial and ventricular pacing thresholds remained below 1.2 V at 0.5 ms. Thresholds did not differ between pacing sites: left atrial, 0.82 V at 0.5 ms; right atrial, 0.74 V at 0.5 ms (p = 0.85); and left ventricular, 0.96 V at 0.5 ms; right ventricular, 0.94 V at 0.5 ms (p = 0.65). Sensing demonstrated no difference for atrial leads, at left atrial, 3.4 mV; and right atrial, 2.9 mV (p = 0.12), but there was superiority of left over right ventricular leads (11.2 vs 7.7 mV, p = 0.002). During follow-up, the 239 atrial and ventricular leads experienced 19 (8%) lead failures. Lead survival at 2 and 5 years was 99% and 94% for atrial leads and 96% and 85% for ventricular leads, respectively. CONCLUSIONS Bipolar steroid-eluting epicardial leads demonstrate excellent sensing characteristics and persistent low median pacing thresholds below 1.2 V at 0.5 ms in children during up to 12 years follow-up. Considering growing and active patients with most having congenital heart disease, the lead survival of 85% to 94% at 5 years is favorable. Subanalysis shows superior sensing for left ventricular leads. Bipolar steroid-eluting leads provide an alternative approach for permanent pacing and may also be considered for left atrial and ventricular pacing, resynchronization, or defibrillator therapy.

Adverse effects of Wolff-Parkinson-White syndrome with right septal or posteroseptal accessory pathways on cardiac function

AIMS Wolff-Parkinson-White syndrome with right septal or posteroseptal accessory pathways causes eccentric septal mechanical activation and may provoke left ventricular (LV) dyssynchrony and dysfunction. The aim of the study was to evaluate the effect of radiofrequency catheter ablation (RFA) of the accessory pathways on LV function. METHODS AND RESULTS Retrospectively, transthoracic echocardiography and electrocardiogram recordings were analysed in 34 patients (age: 14.2 +/- 2.5 years) with right septal or posteroseptal accessory pathways prior and after (median: 1 day) successful RFA. Results prior to RFA, LV ejection fraction was decreased (<55%) in 19/34 patients (56%). After RFA, QRS duration was normalized (129 +/- 23 vs. 90 +/- 11, P < 0.0001), LV function improved (ejection fraction: 50 +/- 10 vs. 56 +/- 4%, P = 0.0005) and septal-to-posterior wall motion delay as a global measure for LV dyssynchrony decreased (110 +/- 94 vs. 66 +/- 53, P = 0.012). Longitudinal two-dimensional strain evaluated in five patients demonstrated a decrease of left intraventricular mechanical delay from 292 +/- 125 to 118 +/- 37 ms after RFA. CONCLUSION Wolff-Parkinson-White syndrome with right septal or posteroseptal accessory pathways may cause LV dyssynchrony and jeopardize global LV function. Radiofrequency catheter ablation resulted in normalized QRS duration, mechanical resynchronization, and improved LV function. Even in the absence of arrhythmias, RFA of right septal or posteroseptal pathways may be considered in patients with significantly decreased LV function.

Cardiac output measurement in children: comparison of Aesculon® cardiac output monitor and thermodilution

BACKGROUND We compared cardiac output (CO) measurements by the non-invasive electrical velocimetry (Aesculon) monitor with the pulmonary artery catheter (PAC) thermodilution method in children. METHODS CO values using the Aesculon monitor and PAC thermodilution were simultaneously recorded during cardiac catheterization in children. Measurements were performed under general anaesthesia. To compare, three consecutive measurements for each patient within 3 min were obtained. The means of the three values were compared using simple regression and Bland-Altman analysis. Data were presented as mean (sd). A mean percentage of <30% was defined to indicate clinical useful reliability of the Aesculon monitor. RESULTS A total of 50 patients with a median (range) age of 7.5 (0.5-16.5) yr were enrolled in the study. Mean CO values were 3.7 (1.5) litre min(-1) (PAC thermodilution) and 3.1 (1.7) litre min(-1) (Aesculon) monitor). Analysis for CO measurement showed a good correlation between the two methods (r=0.894; P<0.0001). The bias between the two methods was 0.66 litre min(-1) with a precision of 1.49 litre min(-1). The mean percentage error for CO measurements was 48.9% for the Aesculon monitor when compared with PAC thermodilution. CONCLUSIONS Electrical velocimetry using the Aesculon monitor did not provide reliable CO values when compared with PAC thermodilution. Whether the Aesculon monitor can be used as a CO trend monitor has to be assessed by further investigations in patients with changing haemodynamics.

Liver tissue oxygenation as measured by near-infrared spectroscopy in the critically ill child in correlation with central venous oxygen saturation

Objective: To evaluate the clinical usefulness of near-infrared spatially resolved spectroscopic quantitative assessment of liver tissue oxygenation for simple, non-invasive estimation of global tissue oxygenation in critically ill neonates and children. Design: Prospective observational clinical study. Setting: A tertiary multidisciplinary neonatal and paediatric intensive care unit (23 beds). Patients: One hundred neonates and children consecutively admitted to the paediatric intensive care unit. Measurements and results: Near-infrared spectroscopic single-point assessment of liver tissue oxygenation index (TOILiver) was compared with global tissue oxygenation as measured by central venous oxygen saturation (SvO2) and derived haemodynamic parameters. Data were compared using linear and multiple regression analysis. Overall correlation between TOILiver and SvO2 was good (r=0.72, p<0.0001). Multivariable regression revealed that SvO2 alone explained 51% of the observed variance of TOILiver. However, our data demonstrated large inter-individual differences between SvO2 and TOILiver values. Conclusion: Near-infrared spatially resolved spectroscopic quantitative measurement of liver tissue oxygenation correlates well with SvO2 in critically ill neonates and children. Large inter-individual SvO2 to TOILiver differences may prevent its use for non-invasive single-point estimation of global tissue oxygenation. Further clinical studies are required to validate the method with other regional and global haemodynamic parameters and to evaluate its clinical use for continuous non-invasive haemodynamic monitoring.

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.

Chylous Bronchial Casts After Fontan Operation

A 4-year old boy with D-transposition of the great arteries, pulmonary atresia with intact ventricular septum, and a hypoplastic left ventricle with mitral atresia underwent a Fontan operation. He had previously been treated with neonatal balloon atrial septostomy followed by a left modified Blalock-Taussig shunt and, at the age of 2 years, a Hemi-Fontan operation was performed. The Fontan operation was completed with an extracardiac conduit (Goretex) from the inferior vena cava to the superior vena cava. High central venous pressures and bilateral chylothoraces complicated the postoperative course. Despite 6 weeks of conservative treatment with fat-free …

Familial X-linked cardiomyopathy (Danon disease): diagnostic confirmation by mutation analysis of the LAMP2gene.

A boy presented at age 2.5 years with mild left ventricular hypertrophy and mild myopathy. Hypertrophic cardiomyopathy progressed relentlessly, leading to death at age 16 years shortly before planned heart transplantation. During the course of the disease, his mother developed severe dilated cardiomyopathy and died of its complications at 46 years of age. The combination of myopathy and cardiomyopathy, the biochemical and electron microscopy findings in a muscle biopsy, and the pedigree suggested Danon disease (MIM 300257), an X-linked lysosomal storage disorder caused by deficiency of lysosome-associated membrane protein-2 (LAMP2). The diagnosis was confirmed by the identification of a novel mutation, G138A, in the LAMP2gene, leading to the premature stop codon W46X. Conclusion:Early diagnosis of Danon disease is important for genetic counselling and timely cardiac transplantation, the only effective therapeutic option.