Publications by authors named "Christian Balmer"

59 Publications

Radiofrequency catheter ablation for supraventricular tachycardia in a paediatric population: characteristics of tachycardia mechanisms in a subpopulation with early onset.

Cardiol Young 2020 Oct 25;30(10):1383-1388. Epub 2020 Sep 25.

Paediatric Cardiology, Paediatric Heart Center, Department of Surgery, University Children's Hospital, Steinwiesstr. 75, 8032Zurich, Switzerland.

Background: In children, the first episode of supraventricular tachycardia occurs at various ages. The aim of this study is to describe age-specific tachycardia mechanisms, clinical findings, and outcome in a contemporary cohort of paediatric patients with supraventricular tachycardia.

Methods: Retrospective analysis of 531 consecutive patients with structurally normal hearts under the age of 18 years who underwent invasive electrophysiological study for supraventricular tachycardia. The study population was divided into two groups, early-onset group (n = 57) and late-onset group (n = 474), according to the age of the occurrence of the first tachycardia before or after the age of 12 months.

Results: Accessory pathway-mediated tachycardia was more common (82.5 versus 50.1%, p < 0.001) and the proportion of left-sided accessory pathways was more pronounced (74.5 versus 53.7%, p = 0.01) in the early-onset group than in the late-onset group. The antegrade and retrograde refractory periods of the accessory pathways were similar in both groups, but pre-excitation was more common in the early-onset group (50.9 versus 31.9%, p = 0.007). Typical atrioventricular nodal re-entrant tachycardia was more common (36.7 versus 7.0%, p < 0.001) in the late-onset group. There was no difference among the two groups regarding overall outcome.

Conclusion: Accessory pathway-mediated re-entrant tachycardia is the most common mechanism of recurrent supraventricular tachycardia in infants with structurally normal hearts who are later referred to an electrophysiological study. These pathways often cause pre-excitation and tend to be located on the left side whereas their refractory period is not different from that of patients with late-onset tachycardia.
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http://dx.doi.org/10.1017/S1047951120002954DOI Listing
October 2020

Risk Factors for Postprocedural Arterial Ischemic Stroke in Children With Cardiac Disease.

Stroke 2020 09 19;51(9):e242-e245. Epub 2020 Aug 19.

Department of Neonatology, University of Basel Children's Hospital (UKBB), Switzerland (M.I.H.).

Background And Purpose: Cardiac pathologies are the second most frequent risk factor (RF) in children with arterial ischemic stroke (AIS). This study aimed to analyze RFs for AIS in children with cardiac disease and cardiac intervention.

Methods: Data were drawn from the Swiss Neuropediatric Stroke Registry. Patients with cardiac disease and postprocedural AIS registered from 2000 until 2015 were analyzed for the cause of cardiac disease and for potential RFs.

Results: Forty-seven out of 78 children with cardiac disease had a cardiac intervention. Of these, 36 presented a postprocedural AIS. Median time from cardiac intervention to symptom onset was 4 days (interquartile range, 2-8.5); time to diagnosis of AIS was 2 days (interquartile range, 0-5.8). Main RFs for postprocedural AIS were hypotension, prosthetic cardiac material, right-to-left shunt, arrhythmias, low cardiac output, and infections.

Conclusions: In children with postprocedural AIS, time to diagnosis was delayed. Most patients presented multiple potentially modifiable RFs as hemodynamic alterations and infections.
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http://dx.doi.org/10.1161/STROKEAHA.120.029447DOI Listing
September 2020

Dysphagia and an aberrant subclavian artery: more than just a coincidence.

Interact Cardiovasc Thorac Surg 2020 08;31(2):228-231

Congenital Cardiovascular Surgery, Children's Heart Centre and Children's Research Centre, University Children's Hospital Zurich, Zurich, Switzerland.

Aberrant subclavian artery (arteria lusoria) is the most common congenital malformation of the aortic arch in the general population. As patients are more often asymptomatic, indications for correcting this abnormality, even in those having dysphagia, are very vague. While presenting a case of a 10-year-old girl who suffered from dysphagia lusoria for 2 years before being cured by a simple surgical translocation, we review the literature and debate various aspects of its management from diagnosis to surgery and cosmesis.
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http://dx.doi.org/10.1093/icvts/ivaa091DOI Listing
August 2020

Pacemaker Implantation in Neonates and Infants: Favorable Outcomes with Epicardial Pacing Systems.

Pediatr Cardiol 2020 Jun 27;41(5):910-917. Epub 2020 Feb 27.

Department of Surgery, Pediatric Cardiology, Pediatric Heart Center, University Children's Hospital, Steinweisstrasse 75, 8032, Zurich, Switzerland.

The implantation of pacemakers (PM) in neonates and infants requires particular consideration of small body size, marked body growth potential, and the decades of future pacing therapy to be expected. The aim of this study is to quantify the complications of implantation and outcome occurring at our center and to compare these with other centers. Retrospective analysis of 52 consecutive patients undergoing PM implantation at a single tertiary care center within the first year of life. PMs were implanted at a median age of 3 months (range 0-10 months). Structural heart defects were present in 44 of 52 patients. During a median follow-up time of 40.4 months (range 0.1-114 months), measurements for sensing, pacing thresholds, and lead impedance remained stable. No adverse pacing effect was observed in left ventricular function or dimensions over time. There were 20 reoperations in 13 patients at a median time of 4.7 years (range 0.05-8.2 years) after implantation, for end of battery life (n = 10), lead dysfunction (n = 3), device dislocation (n = 3), infection (n = 3), and diaphragmatic paresis (n = 1). No PM-related mortality occurred. Epicardial pacemaker implantation in neonates and infants is an invasive but safe and effective procedure with a relatively low risk of complications. Our current implantation technique and the use of bipolar steroid-eluting electrodes, which we prefer to implant on the left ventricular apex, lead to favorable long-term results.
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http://dx.doi.org/10.1007/s00246-020-02332-zDOI Listing
June 2020

Reversible cerebral vasoconstriction syndrome during caesarean section.

BMJ Case Rep 2019 Dec 5;12(12). Epub 2019 Dec 5.

Department of Anaesthesiology, Clinical Pharmacology, Intensive Care and Emergency Medicine, Geneva University Hospitals, Geneva, Switzerland

We describe the case of a 25-year-old parturient who presented sudden onset and short-lived severe headache caused by reversible cerebral vasoconstriction syndrome (RCVS) during an emergency caesarean section. The syndrome was triggered by phenylephrine administered intravenously to correct arterial hypotension following spinal anaesthesia. RCVS is a clinical and radiological syndrome attributed to transient disturbance in the control of cerebral arterial tone resulting in vasospasms. The syndrome can be precipitated by several triggers, including vasoactive drugs, often used during spinal anaesthesia, illicit drugs, pregnancy and postpartum state. Diagnosis and management can be challenging during pregnancy, peripartum or post partum, since many medications commonly used during these periods must be avoided to prevent triggering RCVS. The aim of this report is to raise the awareness, particularly for anaesthesiologists and obstetricians, of this rare and potentially serious syndrome. We discuss diagnosis, triggers, pathogenesis, clinical course and complications, as well as coordinated multidisciplinary management plans.
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http://dx.doi.org/10.1136/bcr-2019-230606DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6904171PMC
December 2019

Magnetic resonance imaging of patients with epicardial leads: in vitro evaluation of temperature changes at the lead tip.

J Interv Card Electrophysiol 2019 Dec 12;56(3):321-326. Epub 2019 Nov 12.

Institute for Biomedical Engineering, University and ETH Zurich, Gloriastrasse 35, 8092, Zürich, Switzerland.

Purpose: The aim of this study was to systematically investigate the potential heating effects of magnetic resonance imaging (MRI) in the presence of epicardial leads, which are connected to a device or abandoned, using a series of in vitro measurements.

Methods: The heating effects of MRI in a 1.5-T scanner were measured at the lead tip in a gel-filled tank. First, a transvenous lead (5086-45 cm, Medtronic) was compared with an epicardial lead (4968-35 cm, Medtronic) with and without connection to an MR-conditional pacemaker. Then, experiments were conducted using various lengths of epicardial leads exposed to MRI.

Results: (1) A temperature rise of + 2.5 °C was observed for the transvenous lead attached to an MRI-conditional pacemaker. The epicardial lead attached to the same pacemaker showed four times higher heating. (2) The transvenous lead without pacemaker showed four times higher heating, and the epicardial lead without pacemaker showed 30 times higher heating. (3) The epicardial lead coiled to 20 cm length without pacemaker showed 9 times higher heating. (4) Experiments with various lengths of epicardial leads showed that the shorter the leads were, the smaller was the heating effect.

Conclusion: Standard clinical MRI investigations may result in pronounced heating at the tip of epicardial electrodes. Epicardial leads, which are not connected to a pacemaker and thus mimicking abandoned leads, may even result in a more pronounced rise in temperature at the lead tip. Therefore, current epicardial pacing systems may carry a substantial risk of inducing thermal damage of the neighboring tissue during MRI scanning.
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http://dx.doi.org/10.1007/s10840-019-00627-7DOI Listing
December 2019

Health-related quality of life outcomes in pediatric patients with cardiac rhythm devices: a cross-sectional study with case-control comparison.

Health Qual Life Outcomes 2019 Oct 11;17(1):152. Epub 2019 Oct 11.

Children's Research Center, University Children's Hospital, Steinwiesstrasse 75, CH-8032, Zurich, Switzerland.

Background: Little is known about health-related quality of life (HRQoL) in pediatric patients with cardiac rhythm devices. This study aims to compare self- and proxy-reported HRQoL in patients with pacemaker (PM) and implantable cardioverter-defibrillator (ICD) to that in sex- and age-matched healthy controls and to examine predictors for generic and disease-specific HRQoL.

Methods: The study included 72 PM and ICD patients (39% females) and 72 sex- and age-matched healthy controls from 3 to 18 years of age. HRQoL data was obtained by the PedsQL 4.0 Generic Core Scales and Pediatric Cardiac Quality of Life Inventory. Medical data was collected retrospectively from medical records.

Results: Patients had significantly lower self- and proxy-reported generic overall HRQoL and lower physical health than healthy controls, and ICD patients also had lower psychosocial health. On multivariate analyses, generic overall HRQoL and physical health was significantly predicted by current cardiac medication (β = -.39, p = .02 for overall HRQoL, respectively β = -.44, p = .006 for physical health). Disease-specific overall HRQoL was only marginally predicted by child age, device type, and the presence of a structural congenital heart disease (p < .10).

Conclusions: This study shows that PM and ICD patients have lower HRQoL than healthy controls and that patients who need cardiac medication are seen by their parents at great risk for lower generic overall HRQoL. Our study also indicates a trend towards higher risk for low disease-specific HRQoL in younger patients, ICD patients, and patients with a structural congenital heart disease. Special attention should be given to these patients as they may benefit from a timely clinical evaluation in order to provide supportive interventions.
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http://dx.doi.org/10.1186/s12955-019-1219-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6788066PMC
October 2019

Posttraumatic stress and health-related quality of life in parents of children with cardiac rhythm devices.

Qual Life Res 2019 Sep 16;28(9):2471-2480. Epub 2019 May 16.

Department of Psychosomatics and Psychiatry, University Children's Hospital, Steinwiesstrasse 75, 8032, Zurich, Switzerland.

Purpose: Studies have shown a high prevalence of post-traumatic stress disorders (PTSD) among parents of children with life-threatening diseases. However, it is yet unknown whether parents of children with cardiac rhythm device develop posttraumatic stress symptoms or even PTSD.

Methods: This cross-sectional investigation is part of a comprehensive single-center study of long-term medical and psychosocial outcomes in pediatric patients with pacemaker (PM) and implantable cardioverter defibrillator (ICD). 69 patients (78%) were included in the study, with the participation of 69 mothers and 57 fathers. Parents completed the Posttraumatic Diagnostic Scale and Medical Outcomes Study Short Form-36 item questionnaire. Child's medical data was collected retrospectively from patients' hospital records.

Results: At assessment, the patients (39% females) were on average 11.2 years old. The predominant device type was PM in 56 cases (81%). The mean time since device implantation was 6.3 years (SD = 4.3). Full heart-disease related PTSD was diagnosed in one mother and no father, while partial heart-disease-related PTSD was diagnosed in 3 mothers (4%) and 2 fathers (4%). Parental HRQoL-especially regarding the mental health dimension-was affected in both parents. In both parents, total PTSD symptom severity scores were a significant predictor for mental health summary scores after controlling for child age at implantation, presence of other non-cardiac disease in the child, parental age, and presence of own chronic disease.

Conclusions: Special attention should be given to parental PTSD symptoms in the clinical follow-up of PM and ICD patients as some parents might probably benefit from psychological support.
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http://dx.doi.org/10.1007/s11136-019-02202-zDOI Listing
September 2019

Health-Related Quality of Life and Psychological Adjustment of Children and Adolescents with Pacemakers and Implantable Cardioverter Defibrillators: A Systematic Review.

Pediatr Cardiol 2019 Jan 11;40(1):1-16. Epub 2018 Dec 11.

Department of Cardiology, Pediatric Heart Centre, University Children's Hospital Zurich, Steinwiesstr. 75, 8032, Zurich, Switzerland.

The absence of cardiac symptoms, the improved exercise tolerance and the increased life expectancy are the advantages of a successful cardiac device therapy. Nevertheless, the fact of a lifelong device dependency, the inherent possibility of device malfunction and the progression of the underlying heart disease may impact the health-related quality of life (HRQoL) and the psychological adjustment of these individuals. To date, an overview of findings on these topics is lacking. The objective of this study was to evaluate and summarize the current evidence on generic and disease-specific HRQoL and psychological adjustment in paediatric patients with a pacemaker (PM) or an implantable cardioverter defibrillator (ICD). A systematic literature search was conducted using relevant databases and predefined keywords. Study selection was conducted based on predefined inclusion criteria. Fourteen studies were selected which were of varying methodological quality. Overall, the studies indicated that PM/ICD patients themselves and their parents scored lower than healthy controls on generic HRQoL scales and reported similar disease-related HRQoL as patients with severe forms of congenital heart disease. Regarding psychological adjustment outcome, PM recipients and norms were not significantly different with respect to anxiety and depression symptoms, while ICD patients showed more signs of anxiety than depression. HRQoL and psychological health should be considered when setting therapy goals and evaluating medical treatment success. This approach allows an early intervention in patients at risk of maladaptation, which is especially important in the young patient undergoing crucial developmental stages.
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http://dx.doi.org/10.1007/s00246-018-2038-xDOI Listing
January 2019

Comparison of postoperative chylothorax in infants and children with trisomy 21 and without dysmorphic syndrome: Is there a difference in clinical outcome?

J Pediatr Surg 2019 Jul 6;54(7):1298-1302. Epub 2018 Jul 6.

Department of Neonatology and Pediatric Intensive Care, University Children's Hospital Zurich, Children's Research Center Division, Zurich, Switzerland. Electronic address:

Introduction: Children with trisomy 21 are prone to postoperative chylothorax, caused by malformation of the lymphatic system, after cardiac surgery. The clinical course of patients diagnosed with postoperative chylothorax and trisomy 21 was compared to that of patients without dysmorphic syndromes. Additionally, differences between the groups in composition, amount, and duration of chyle were analyzed to better understand chylothorax in patients with trisomy 21.

Materials And Methods: Retrospective cohort study using inpatient clinical databases during a 10-year period.

Results: A total of 2255 patients underwent cardiac operations during the period, of whom 160 (7.1%) patients were diagnosed with trisomy 21. Chylothorax developed in 122 children; 89 patients were included in our study. Of 160 trisomy 21 patients, 27 (16.9%) developed postoperative chylothorax compared to 62 (3%) of 2095 patients without dysmorphic syndromes (p = <0.001). Time on ventilation, stay in intensive care, hospital stay, mortality, and composition of chylous effusion did not differ between groups. The rate of thrombosis was significantly lower (p = 0.02) in the trisomy 21 group.

Conclusion: Children with trisomy 21 and congenital heart disease are more prone to developing chylothorax after heart surgery than those without dysmorphic syndromes. However if they develop this postoperative complication, mortality, chylous composition, time in ICU, and duration of hospital stay is not different to from that of other infants or children with this complication. This is important information for the medical specialists involved and is helpful in counseling parents of children with trisomy 21 undergoing heart surgery.

Level Of Evidence: This is a treatment study evidence level III.
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http://dx.doi.org/10.1016/j.jpedsurg.2018.06.032DOI Listing
July 2019

Prevention of Low Cardiac Output Syndrome After Pediatric Cardiac Surgery: A Double-Blind Randomized Clinical Pilot Study Comparing Dobutamine and Milrinone.

Pediatr Crit Care Med 2018 07;19(7):619-625

Department of Paediatric Cardiology, University Children's Hospital Zurich, Zurich, Switzerland.

Objectives: Dobutamine and milrinone are commonly used after open-heart surgery to prevent or treat low cardiac output syndrome. We sought to compare efficacy and safety of these drugs in pediatric patients.

Design: Prospective, single-center, double-blinded, randomized clinical pilot study.

Setting: Tertiary-care university children's hospital postoperative pediatric cardiac ICU.

Patients: After written consent, 50 consecutive patients (age, 0.2-14.2 yr; median, 1.2 yr) undergoing open-heart surgery for congenital malformations were included.

Interventions: After cardiopulmonary bypass, a continuous infusion of either dobutamine or milrinone was administered for the first 36 postoperative hours. Maximum dose: dobutamine 6 µg/kg/min, milrinone 0.75 µg/kg/min.

Measurements And Main Results: There were no significant differences in demographic data, complexity of surgery, and intraoperative characteristics between the two study groups (dobutamine vs milrinone). Efficacy was defined as need for additional vasoactive support, which did not differ between groups (dobutamine 61% vs milrinone 67%; p = 0.71). Sodium nitroprusside was used more often in the dobutamine group (42% vs 13%; p = 0.019). Systolic blood pressure showed a trend toward higher values in the dobutamine group, whereas both drugs increased heart rate early postoperatively. Echocardiography demonstrated a consistently good cardiac function in both groups. Central venous oxygen saturation, serum lactate levels, urine output, time to chest tube removal, length of mechanical ventilation, ICU, and hospital stay were similar in both groups. Both drugs were well tolerated, no serious adverse events occurred.

Conclusions: Dobutamine and milrinone are safe, well tolerated, and equally effective in prevention of low cardiac output syndrome after pediatric cardiac surgery. The hemodynamic response of the two drugs is comparable. In uncomplicated cases, a trend toward the more cost-saving dobutamine might be anticipated; however, milrinone demonstrated a trend toward higher efficacy in afterload reduction.
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http://dx.doi.org/10.1097/PCC.0000000000001533DOI Listing
July 2018

Long-term outcome of epicardial implantable cardioverter-defibrillator systems in children: results justify its preference in paediatric patients.

Europace 2018 09;20(9):1484-1490

Departments of Cardiology, Pediatric Heart Centre, University Children's Hospital Zurich, Steinwiesstr. 75, Zurich, Switzerland.

Aims: The question of whether transvenous or epicardial implantable cardioverter defibrillator (ICD) system is more beneficial in children and adolescents is controversially discussed. We sought to analyse the long-term outcome after implantation of ICDs using epicardial pacing/sensing and pleural shock leads.

Methods And Results: Retrospective analysis of 31 consecutive patients undergoing a total of 55 implantations of epicardial/pleural ICD systems below 20 years of age. Median age at implantation was 11.4 years (range 2.2-20) and median follow-up 57 months (range 0.4-127). The ICDs were implanted for primary (n = 17) and for secondary prevention (n = 14). The first defibrillation threshold at implantation was ≤25 J in 94% of the implant procedures. Appropriate shocks occurred in 6 of 31 patients. Inappropriate shocks occurred in 4 of 31 patients triggered by lead fracture, T-wave oversensing, sinus tachycardia, and atrial fibrillation. Freedom from first ICD discharge was 81, 71, and 71% at 3, 6, and 9 years, respectively. Reoperation was indicated in 16 of 31 patients for lead failure (n=11), end of battery life (n=10), generator migration (n=1) and recall (n=1); freedom from reoperation was 74 and 55% at 3 and 6 years.

Conclusion: Paediatric epicardial/pleural ICD therapy is feasible, effective, and safe both in the short-term as well as in the long-term perspective at the price of relatively frequent surgical revisions. They do not generally result in an increased burden of inappropriate shocks. This ICD system meets the needs of the paediatric population and can be recommended as a first choice in this age group.
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http://dx.doi.org/10.1093/europace/eux284DOI Listing
September 2018

Predictors of health-related quality of life in children with chronic heart disease.

Cardiol Young 2017 Oct 18;27(8):1455-1464. Epub 2017 May 18.

1Department of Child and Adolescent Psychiatry/Psychotherapy,University Ulm Medical Centre,Ulm,Germany.

Objective: Chronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease. Patients and methods The Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers.

Results: Greater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart-lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child's health-related quality of life.

Conclusions: Beyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.
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http://dx.doi.org/10.1017/S1047951117000440DOI Listing
October 2017

Digoxin Toxicity in a Neonate Caused by the Interaction with Carvedilol.

Klin Padiatr 2017 03 25;229(2):90-92. Epub 2017 Apr 25.

Intensive Care Unit, Children's Hospital Zurich, Zurich, Switzerland.

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http://dx.doi.org/10.1055/s-0043-100220DOI Listing
March 2017

Transseptal puncture for radiofrequency catheter ablations of left-sided arrhythmias in a paediatric population.

Cardiol Young 2017 Mar 18;27(2):267-272. Epub 2016 Apr 18.

1Department of Paediatric Cardiology,University Children's Hospital Zurich,Zürich,Switzerland.

Puncturing the atrial septum is frequently used in adults. In children, the transseptal puncture is less common, technically more demanding, and the rate of complications is not well described. We studied the feasibility and safety of this procedure in a retrospective analysis of 157 consecutive children undergoing transseptal puncture for radiofrequency catheter ablation of left atrial targets in two tertiary-care centres between 2005 and 2013. The median age of the patients at intervention was 12.5 years (1.1-18 years), with median weight of 42 kg (range 9.0-97.0 kg). Pre-excitation was found in 102 procedures, accessory pathway with exclusively retrograde conduction in 41, focal atrial tachycardia in nine, left-sided permanent junctional/reciprocating tachycardia-like accessory pathways in three, and atypical atrioventricular nodal re-entry tachycardia in two. All the procedures were guided by fluoroscopy. Additional imaging by transoesophageal echocardiography was used in three patients. Successful transseptal puncture was possible in 99.4% of the cases, ablation in 97.4%. The median time, including mapping and radiofrequency ablation, was 120 minutes (range 60-450), the median fluoroscopy time 10.8 minutes (range 1.8-75), and the median radiation dose 3 Gy cm2 (range 0.3-35). In total, five patients (3.2%) had a recurrent arrhythmia during the observation period of a median of 40 months (range 1-103). No complications associated with the transseptal puncture were observed. Transseptal puncture is a feasible and safe procedure in children. This access allows successful and efficient radiofrequency ablation of arrhythmia of the left atrium in the vast majority of the patients and might be considered as the first-line approach in this population.
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http://dx.doi.org/10.1017/S1047951116000457DOI Listing
March 2017

Does superior caval vein pressure impact head growth in Fontan circulation?

Cardiol Young 2016 Oct 15;26(7):1327-32. Epub 2016 Jan 15.

1Division of Pediatric Cardiology,University Children's Hospital Zurich,Switzerland.

Background: Patients with bidirectional cavopulmonary anastomosis have unphysiologically high superior caval vein pressure as it equals pulmonary artery pressure. Elevated superior caval vein pressure may cause communicating hydrocephalus and macrocephaly. This study analysed whether there exists an association between head circumference and superior caval vein pressure in patients with single ventricle physiology.

Methods: We carried out a retrospective analysis of infants undergoing Fontan completion at our institution from 2007 to 2013. Superior caval vein pressures were measured during routine catheterisation before bidirectional cavopulmonary anastomosis and Fontan completion as well as head circumference, adjusted to longitudinal age-dependent percentiles.

Results: We included 74 infants in our study. Median ages at bidirectional cavopulmonary anastomosis and Fontan were 4.8 (1.6-12) and 27.9 (7-40.6) months, respectively. Head circumference showed significant growth from bidirectional cavopulmonary anastomosis until Fontan completion (7th (0-100th) versus 20th (0-100th) percentile). There was no correlation between superior caval vein pressure and head circumference before Fontan (R2=0.001). Children with lower differences in superior caval vein pressures between pre-bidirectional cavopulmonary anastomosis and pre-Fontan catheterisations showed increased growth of head circumference (R2=0.19).

Conclusions: Patients with moderately elevated superior caval vein pressure associated with single ventricle physiology did not have a tendency to develop macrocephaly. There is no correlation between superior caval vein pressure before Fontan and head circumference, but between bidirectional cavopulmonary anastomosis and Fontan head circumference increases significantly. This may be explained by catch-up growth of head circumference in patients with more favourable haemodynamics and concomitant venous pressures in the lower range. Further studies with focus on high superior caval vein pressures are needed to exclude or prove a correlation.
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http://dx.doi.org/10.1017/S1047951115002528DOI Listing
October 2016

Postoperative Hyperglycemia and 4-Year Neurodevelopmental Outcome in Children Operated for Congenital Heart Disease.

J Pediatr 2015 Dec 30;167(6):1253-8.e1. Epub 2015 Jul 30.

Children's Research Center, University Children's Hospital, Zurich, Switzerland; Child Development Center, University Children's Hospital, Zurich, Switzerland.

Objectives: To study the long-term neurodevelopmental effects of hyperglycemia in infant bypass surgery for congenital heart disease (CHD).

Study Design: Prospective cohort study on neurodevelopmental outcome after infant cardiac bypass surgery.

Exclusion Criteria: age older than 1 year at first surgery, genetic comorbidity, and birth weight <2000 g. Of 167 eligible infants, follow-up examination at 4 years was completed in 150 children (90%). Intraoperative and postoperative highest and lowest glucose levels within 24 hours after bypass surgery were prospectively collected. Neurodevelopmental outcome at 4 years of age was assessed using standardized IQ tests and the Movement Assessment Battery for Children-second version for motor outcome assessment.

Results: Mean age at surgery was 2.8 months (0.1-10.7 months), 33% of children had an acyanotic CHD and 67% a cyanotic CHD. Glucose levels were elevated (>8 mmol/L) in 21 (14%) children in the first 24 postoperative hours. Glucose levels normalized in all children within 48 hours, 7 children (4%) received insulin infusions. Mean total IQ was within the normal range, but significantly lower than the normal population (92.5 [SD 15.0], P < .001). Higher postoperative glucose levels were related to better cognitive performance in the univariate analysis (P < .03), but not when other risk factors were taken into account. Independent risk factors for adverse outcome were lower socioeconomic status, higher risk adjustment for congenital heart surgery score, and longer duration of intensive care stay.

Conclusion: Hyperglycemia is common in the early postoperative course after infant bypass surgery for CHD and normalizes within 48 hours. Hyperglycemia has no adverse effect on long-term neurodevelopmental outcome.
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http://dx.doi.org/10.1016/j.jpeds.2015.07.007DOI Listing
December 2015

Pediatric heart transplantation.

J Thorac Dis 2015 Mar;7(3):552-9

1 Department for Congenital Cardiovascular Surgery, 2 Divisions of Pediatric Cardiology and Children's Research Centre, 3 Department for Anesthesiology, 4 Department of Intensive Care and Neonatology, University Children's Hospital, Zurich, Switzerland.

Pediatric heart transplantation (pHTx) represents a small (14%) but very important and particular part in the field of cardiac transplantation. This treatment has lifelong impact on children. To achieve the best short and especially long-term survival with adequate quality of life, which is of crucial importance for this young patient population, one has to realize and understand the differences with adult HTx. Indication for transplantation, waitlist management including ABO incompatible (ABOi) transplantation and immunosuppression differ. Although young transplant recipients are ultimately likely to be considered for re-transplantation. One has to distinguish between myopathy and complex congenital heart disease (CHD). The differences in anatomy and physiology make the surgical procedure much more complex and create unique challenges. These recipients need a well-organized and educated team with pediatric cardiologists and intensivists, including a high skilled surgeon, which is dedicated to pHTx. Therefore, these types of transplants are best concentrated in specialized centers to achieve promising outcome.
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http://dx.doi.org/10.3978/j.issn.2072-1439.2015.01.38DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4387410PMC
March 2015

Can preoperative cranial ultrasound predict early neurodevelopmental outcome in infants with congenital heart disease?

Dev Med Child Neurol 2015 Jul 23;57(7):639-644. Epub 2015 Jan 23.

Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.

Aim: To determine the role of preoperative cranial ultrasound (cUS) in predicting neurodevelopmental outcome in infants undergoing bypass surgery for congenital heart disease (CHD).

Method: Prospective cohort study on 77 infants (44 males, 33 females) operated before 3 months of age (median age at surgery 10d [range 3-88d]) who received at least one preoperative cUS. Outcome at 1 year was assessed with a standardized neurological examination and the Bayley Scales of Infant Development II (mental developmental index [MDI]; psychomotor developmental index [PDI]).

Results: Abnormalities on cUS were detected in 22 (29%) infants and consisted of diffuse brain oedema (n=12, 16%), periventricular white matter injury (n=5, 6%), ventricular dilatation (n=3, 4%), and intraventricular haemorrhage (IVH) (n=2, 3%). Infants undergoing balloon-atrial septostomy (BAS) had a higher rate of subsequent brain oedema than those without BAS (p=0.006). cUS abnormalities were not related to neurodevelopmental outcome.

Interpretation: Preoperative cUS findings in infants undergoing bypass surgery for CHD occur rather frequently, consisting of mild lesions such as brain oedema or white matter changes. These findings, however, do not correlate with early neurodevelopmental outcome.
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http://dx.doi.org/10.1111/dmcn.12701DOI Listing
July 2015

Role of sevoflurane in organ protection during cardiac surgery in children: a randomized controlled trial.

Interact Cardiovasc Thorac Surg 2015 Feb 17;20(2):157-65. Epub 2014 Nov 17.

Department of Anaesthesiology, University Hospital Zurich, Zurich, Switzerland.

Objectives: The protective effects of volatile anaesthetics against ischaemia-reperfusion injury have been shown in vitro, but clinical studies have yielded variable results. We hypothesized that, in children, sevoflurane provides superior cardioprotection after cardiac surgery on cardiopulmonary bypass (CPB) compared with totally intravenous anaesthesia (TIVA).

Methods: In this randomized controlled, single-centre study, 60 children with cyanotic and acyanotic heart defects undergoing elective cardiac surgery under CPB (RACHS-1 1-3) were randomized to sevoflurane or TIVA (midazolam <6 months of age, propofol >6 months of age). The primary end-point was the postoperative peak cardiac troponin I/T (cTnI/T). Perioperative cardiac function (as determined by brain-type natriuretic peptide, echocardiography and postoperative vasopressor/inotrope requirements), short-term clinical outcomes (duration of intubation, intensive care unit and hospital length of stay), postoperative inflammatory profile, and pulmonary, renal and liver function were defined as secondary end-points. Analysis of variance was used for statistical analysis.

Results: There was no statistically significant difference in postoperative peak troponin values or any of the secondary end-points. In the subgroup of acyanotic patients under 6 months, sevoflurane led to significantly lower postoperative troponin levels compared with midazolam [reduction of 54% (95% confidence interval 29-71%, P = 0.002)], without any differences in secondary outcome parameters.

Conclusions: Sevoflurane did not provide superior myocardial protection in our general paediatric cardiac surgical population. In children under 6 months, however, sevoflurane might be beneficial in comparison with midazolam. The conditioning effects of sevoflurane in specific paediatric subgroups need to be further investigated.
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http://dx.doi.org/10.1093/icvts/ivu381DOI Listing
February 2015

Propionic acidemia in a previously healthy adolescent with acute onset of dilated cardiomyopathy.

Eur J Pediatr 2014 Jul 11;173(7):971-4. Epub 2014 Jun 11.

Division of Metabolic Diseases, University Children`s Hospital, Zurich, Switzerland,

Unlabelled: Propionic acidemia (PA) is a rare autosomal recessive organic aciduria resulting from defects in propionyl-CoA-carboxylase (PCC), a key enzyme of intermediate energy metabolism. PA mostly manifests during the neonatal period, when affected newborns develop severe metabolic acidosis and hyperammonemia. We present a previously healthy teenager, who suffered from acute fatigue and breathlessness. The patient was tachycardic, displayed a precordial heave and a systolic murmur. Cardiac investigations revealed severe dilated cardiomyopathy (DCM). Biochemical work up led to the diagnosis of PA. Remarkably, this patient of consanguineous Hispanic origin was in a good general health condition before the acute onset of DCM. Diagnosis of PA was confirmed by enzymatic and molecular genetic analysis, the latter revealing a novel homozygous mutation in the PCCB gene (c.1229G > A; p.R410Q). Residual PCC enzyme activity of approximately 14 % of normal was detected in patient's lymphocytes and fibroblasts, thereby providing a possible explanation for the hitherto asymptomatic phenotype.

Conclusion: Isolated DCM, although rare, can be the leading and/or sole symptom of late-onset PA. Therefore, patients with DCM should receive a comprehensive diagnostic evaluation including selective screening for inborn errors of metabolism.
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http://dx.doi.org/10.1007/s00431-014-2359-6DOI Listing
July 2014

Postoperative arrhythmia in patients with bidirectional cavopulmonary anastomosis.

Eur J Cardiothorac Surg 2014 Apr 19;45(4):620-4. Epub 2013 Aug 19.

Department of Cardiology, University Children's Hospital Zurich, Zürich, Switzerland.

Objectives: Bidirectional cavopulmonary anastomosis (BDCPA) is part of the staged Fontan palliation for congenital heart defects with single-ventricle morphology. The aim of this study was to describe incidence and characteristics of early postoperative arrhythmias in patients undergoing BDCPA.

Methods: Retrospective analysis of 60 patients undergoing BDCPA at the age of <12 months from 2001 to 2008 at a single centre. Arrhythmias were subclassified in sinus bradycardia, premature atrial/ventricular contraction, supraventricular tachycardia and atrioventricular block. The groups were compared according to age at operation and diagnosis. Postoperative follow-up data were included until Fontan completion.

Results: Postoperative arrhythmia was observed in 20 patients: 12 temporary and 8 persisting until hospital discharge. Sinus bradycardia is a common postoperative arrhythmia and occurred in 16 patients (9 transient, 7 persistent until hospital discharge). One patient undergoing BDCPA and a Damus-Kaye-Stansel procedure had a persisting first-degree atrioventricular block. The occurrence of a postoperative arrhythmia was independent of age and diagnostic group (hypoplastic left heart vs non-hypoplasic left heart). After hospital discharge, five of the eight arrhythmia resolved spontaneously resulting in 2 patients with sinus bradycardia and 1 patient with a first-degree AV block immediately before the Fontan completion was undertaken.

Conclusions: Postoperative arrhythmias in patients with BDCPA occur early after surgery and are temporary. Severe and life-threatening arrhythmias are rare although the interventions are complex and the patients very young. The most common arrhythmia is sinus bradycardia.
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http://dx.doi.org/10.1093/ejcts/ezt420DOI Listing
April 2014

Enteroviral myocarditis in neonates.

J Paediatr Child Health 2013 Sep 28;49(9):E451-4. Epub 2013 May 28.

Paediatric Intensive Care Unit, Mater Children's Hospital, Brisbane, Queensland, Australia.

Enteroviruses are a leading cause of viral infections in children. While most enteroviral infections are mild and self-limiting, severe disease such as a viral sepsis syndrome, myocarditis, hepatitis and meningoencephalitis may occur. We present two cases of neonatal enteroviral myocarditis. Cardiorespiratory failure occurred in both cases, and severe shock refractory to conventional treatment required support with extracorporeal membrane oxygenation (ECMO). One child with coxsackievirus B3 myocarditis failed to recover and died after 3 weeks on ECMO, while one child could be decannulated successfully after 9 days of ECMO and recovered completely subsequently. In conclusion, neonatal myocarditis has a very high mortality, and ECMO should be considered early in neonates with rapid clinical and echocardiographic deterioration despite adequate inotropic support.
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http://dx.doi.org/10.1111/jpc.12248DOI Listing
September 2013

Heart transplantation in congenital heart disease: in whom to consider and when?

J Transplant 2013 7;2013:376027. Epub 2013 Feb 7.

Division of Cardiac Surgery, University Hospital Zurich, Zurich, Switzerland.

Due to impressive improvements in surgical repair options, even patients with complex congenital heart disease (CHD) may survive into adulthood and have a high risk of end-stage heart failure. Thus, the number of patients with CHD needing heart transplantation (HTx) has been increasing in the last decades. This paper summarizes the changing etiology of causes of death in heart failure in CHD. The main reasons, contraindications, and risks of heart transplantation in CHD are discussed and underlined with three case vignettes. Compared to HTx in acquired heart disease, HTx in CHD has an increased risk of perioperative death and rejection. However, outcome of HTx for complex CHD has improved over the past 20 years. Additionally, mechanical support options might decrease the waiting list mortality in the future. The number of patients needing heart-lung transplantation (especially for Eisenmenger's syndrome) has decreased in the last years. Lung transplantation with intracardiac repair of a cardiac defect is another possibility especially for patients with interatrial shunts. Overall, HTx will remain an important treatment option for CHD in the near future.
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http://dx.doi.org/10.1155/2013/376027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3614026PMC
April 2013

De novo heterozygous desmoplakin mutations leading to Naxos-Carvajal disease.

Swiss Med Wkly 2012 4;142:w13670. Epub 2012 Sep 4.

Cardiology Department, University Hospital Zurich and University Hospital Balgrist, Zurich, Switzerland.

Study/principles: Arrythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an autosomal-dominantly inherited disease caused by mutations in genes encoding desmosomal proteins and is characterised by fibrofatty replacement occurring predominantly in the right ventricle and can result in sudden cardiac death. Naxos and Carvajal syndrome, autosomal recessive forms of ARVC/D, are characterised by involvement of the right and/or left ventricle in association with palmoplantar keratoderma and woolly hair. The aim of the present study has been to screen for mutations in the desmosomal protein genes of two unrelated patients with Naxos-Carvajal syndrome.

Methods And Results: Desmosomal protein genes were screened for mutations by polymerase chain reaction as well as direct sequencing approach. In each patient we identified a single heterozygous de novo mutation in the desmoplakin gene DSP, p.Leu583Pro and p.Thr564Ile, leading to severe combined cardiac/dermatological and cardiac/dermatological/dental phenotypes. The DSP missense mutations are localised in the N terminal domain of desmoplakin.

Conclusion: The identified variations in DSP involve highly conserved residues. Moreover, the variations are de novo mutations and they are localised in critical protein domains that appear to be mutation hot spots. We assume that these heterozygous variations are causal for the mixed Naxos-Carvajal syndrome phenotype in the screened patients.
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http://dx.doi.org/10.4414/smw.2012.13670DOI Listing
January 2013

Management and outcome of Ebstein's anomaly in children.

Cardiol Young 2013 Feb 15;23(1):27-34. Epub 2012 Mar 15.

Division of Pediatric Cardiology, University Children's Hospital, Zurich, Switzerland.

Objectives: To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly.

Background: Data on long-term outcome of children with Ebstein's anomaly are scarce.

Methods: Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention.

Results: A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%.

Conclusion: In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.
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http://dx.doi.org/10.1017/S1047951112000224DOI Listing
February 2013

Catheter interventional closure of a large right pulmonary artery-to-left atrial communication in a neonate.

Pediatr Cardiol 2012 Dec 9;33(8):1440-2. Epub 2012 Mar 9.

Department of Pediatric Cardiology, University Children's Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland.

Right pulmonary artery-to-left atrial communication is a rare congenital vascular malformation that results in a right-to-left shunting. This report describes the case history of a neonate with a large right pulmonary artery-to-left atrial connection resulting in cyanosis and severe heart failure who underwent successful early catheter interventional therapy. In the neonate, this lesion can be diagnosed accurately using transthoracic echocardiography. Closure of the communication can be achieved even in infants via percutaneous interventional catheterization with a low procedure-related risk and a good midterm follow-up result.
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http://dx.doi.org/10.1007/s00246-012-0262-3DOI Listing
December 2012

Prevalence and predictors of later feeding disorders in children who underwent neonatal cardiac surgery for congenital heart disease.

Cardiol Young 2011 Jun 28;21(3):303-9. Epub 2011 Jan 28.

Division of Speech and Language Pathology, University Children's Hospital, Steinwiesstrasse 75, Zurich, Switzerland.

Aim: We thought of assessing the prevalence and predictors of feeding disorders in patients with congenital heart defects after neonatal cardiac surgery.

Methods: Retrospective study of 82 consecutive neonates (48 males, 34 females) who underwent surgery for congenital heart defects from 1999 to 2002. Information was taken from patient charts and nursing notes. The presence of a feeding disorder was assessed by a questionnaire sent to the paediatricians when the child was 2 years of age. A feeding disorder was defined as a need for tube feeding, inadequate food intake for age, or failure to thrive. Data were analysed with descriptive statistics and logistic regression.

Results: Feeding disorders occurred in 22% of the study population. Reoperation and early feeding disorders were identified as independent risk factors for later feeding disorders (odds ratio 5.8, p 0.01; odds ratio 20.7, p 0.02). There was a trend towards more feeding disorders in patients with neurological abnormalities during the first hospital stay.

Conclusion: Feeding disorder is a frequent, long-term sequela after neonatal cardiac surgery. Patients with congenital heart defects who undergo multiple cardiac surgeries and those with early feeding disorders are at risk of developing later feeding disorders. Patients with these risk factors need to be selected for preventive strategies.
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http://dx.doi.org/10.1017/S1047951110001976DOI Listing
June 2011