ADHD symptomatology of children with congenital heart disease 10 years after cardiac surgery: the role of age at operation.

BACKGROUND: The aim of the present study was to investigate the differences in ADHD symptomatology between healthy controls and children who underwent cardiac surgery at different ages. METHODS: Altogether, 133 children (54 patients with congenital heart disease undergoing first cardiac surgery under 3 years of age, 26 operated at the age of 3 or later, and 53 healthy controls) were examined. Patients completed the Youth Self Report (YSR), while their parents completed the Child Behaviour Checklist (CBCL) and the ADHD Rating Scale-IV. RESULTS: Children receiving surgery for the first time under the age of 3 years were more likely diagnosed with cyanotic type malformation and have undergone to a greater number of operations. However, ADHD symptoms of those treated surgically at or above 3 years of age were more severe than that of the control group or those who were treated surgically at a younger age. The control group and those treated surgically below the age of three did not differ across any of the ADHD symptom severity indicators. CONCLUSIONS: The age at the time of cardiac surgery might be associated with later ADHD symptom severity - with lower age at operation associated with better outcomes. Further, adequately powered studies are needed to confirm these exploratory findings and investigate the moderators of this relationship.

Frequency of syncope as a presenting symptom in channelopathies diagnosed in childhood. Can the multivariable EGSYS score unmask these children?

Pediatric syncope raises cardiac etiology concern as it might be the first sign of life-threatening arrhythmia syndromes. Our aim was to study the incidence of syncope as the presenting symptom in children with arrhythmia syndromes, and if known, warning signs are helpful to reveal the arrhythmic origin. All data on children with channelopathy was followed by a tertiary pediatric cardiac center between 2000 and 2018 and data were reviewed retrospectively. Forty-eight patients were enrolled, representing long QT syndrome (n = 39), catecholaminergic polymorphic ventricular tachycardia (n = 5), and Brugada syndrome (n = 4). Presenting symptoms were syncope in 13 cases [27%] (including 7 initially mislabeled as epilepsy) and sudden cardiac arrest (SCA) in 9 cases [19%]. In the rest of the group, the concern for arrhythmic etiology was raised by either an abnormal ECG during sports medicine screening (n = 13) [27%] or a positive family history of channelopathy (n = 13) [27%]. None of the patients presenting with SCA had a prior syncopal history. Six patients presenting with syncope and afterward treated with ICD had an appropriate shock. Description of witnessed syncope was available in eight out of thirteen children presenting with syncope. Multivariable EGSYS score suggested cardiac origin (≥ 3 points) in 7 out of 8 (88%) patients.Conclusions: Syncope was a relatively uncommon presenting symptom of channelopathies in this sample and did not always precede sudden cardiac arrests. However, we found that multivariable EGSYS score can identify syncope of arrhythmic origin, raising suspicion for pediatric channelopathies even in patients previously misdiagnosed with epilepsy. What is known: • Cardiac syncope is rare in children but can be the first sign of a potentially fatal primary arrhythmia syndrome and is frequently misdiagnosed as atypical/therapy-resistant epilepsy. • Multivariate EGSYS score is effective to diagnose cardiac syncope in adults. What is new: • Cardiac syncope as a presenting symptom is not common in children with cardiac channelopathies and is not often present before sudden cardiac arrest. • Multivariable EGSYS score might identify cardiac syncope in children with a hereditary and secondary channelopathy.

Supraventricular tachycardias in neonates and infants: factors associated with fatal or near-fatal outcome.

Prognosis of supraventricular tachycardias in neonates and infants is thought to be excellent with rare fatal outcomes. Nevertheless, initial management can be challenging. The aim of this study was to perform a retrospective analysis in neonates/infants with non-pos-toperative supraventricular tachycardias regarding risk factors for clinical outcome and type of antiarrhythmic drug therapy. The data of 157 patients aged < 1 year who presented between 2000 and 2015 with symptomatic tachycardias were retrospectively reviewed. Pharmacological therapy was successful in 151 patients (96%); 1 patient (1%) required catheter ablation and 5 patients (3%) died (1 death linked to hemodynamical reasons after effective arrhythmia control). Serious complications following acute medical therapy occurred in 4 patients of survivors. Patients with complications or death had a lower bodyweight, more frequent intrauterine tachycardia, transplacental therapy, urgent caesarian section, higher PRISM II score, longer period to control tachycardia, more frequent proarrhythmia, and major adverse event-defined as life-threatening event without a documented new arrhythmia-compared to the group without complications. There was no significant difference between the groups regarding prematurity, structural heart disease, and type of tachycardia. Proarrhythmia occurred in 6 cases and was related to intravenous drug use with class IC antiarrhythmics in 3/6 cases, digoxin in 2/6 cases, and amiodarone in 1/6 cases. ECG signs of impending proarrhythmia without new-onset arrhythmia requiring cessation of therapy were detected in 6 patients.Conclusion: Although rare, non-post-operative supraventricular tachycardia in neonates and infants might be a serious disease. Acute intravenous pharmacological treatment to control tachycardia might pose a risk for fatal or near-fatal outcome. Detection of proarrhythmia related to class IC antiarrhythmics in neonates might be especially difficult and requires alertness. What is Known • Prognosis of supraventricular tachycardias in children are thought to be excellent with fatal outcomes being rare. • Mortality is increased in the very young and in those with structural heart disease. What is New • Complicated outcome of non-post-operative supraventricular tachycardias in neonates is associated with lower bodyweight, age, prenatal tachycardia, higher PRISM II score, longer period to control tachycardia, and proarrhythmia. • Detection of class IC proarrhythmic effect is especially difficult in neonates because of their narrow QRS and warrants alertness.

Chylothorax after pediatric cardiac surgery complicates short-term but not long-term outcomes-a propensity matched analysis.

BACKGROUND: The occurrence of postoperative chylothorax in children with congenital heart disease is a rare and serious complication in cardiac intensive care units (ICUs). The aim of our study was to identify the perioperative characteristics, treatment options, resource utilization and long term complications of patients having chylothorax after a pediatric cardiac surgery. METHODS: Patients were retrospectively assessed for the presence of chylothorax between January 2002 and December 2012 in a tertiary national cardiac center. Occurrence, treatment options and long term outcomes were analyzed. Chylothorax patients less than 2 years of age were analyzed using propensity-matched statistical analysis in regard to postoperative complications after discharge. RESULTS: During the 10-year period, 48 patients had chylothorax after pediatric cardiac surgery. The highest incidence was observed on the second postoperative day (7 patients, 14.6%). Seven patients (14.6% of the chylothorax population) died. During the follow up period, 5 patients had additional thromboembolic complications (2 had confirmed thrombophilia). Eleven patients had a genetic abnormality (3 had Down's syndrome, 3 had Di-Giorge's syndrome, 1 had an IgA deficiency and 4 had other disorders). During the reoperations (49 cases), no chylothorax occurred. After propensity matching, the occurrence of pulmonary failure (P=0.001) was significantly higher in the chylothorax group, and they required prolonged mechanical ventilation (P=0.002) and longer hospitalization times (P=0.01). After discharge, mortality and neurologic and thromboembolic events did not differ in the matched groups. CONCLUSIONS: Chylothorax is an uncommon complication after pediatric cardiac surgery and is associated with higher resource utilization. Chylothorax did not reoccur during reoperations and was not associated with higher mortality or long-term complications in a propensity matched analysis.