Lower age, lower body weight, higher Aristotle Basic Score, longer cardiopulmonary bypass time, aortic crossclamp time, and use of deep hypothermia and circulatory arrest are the risk factors for postoperative arrhythmias. Junctional ectopic tachycardia and supraventricular tachycardia were the most common postoperative arrhythmias.
Background
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an uncommon, potentially lethal, ion channelopathy. Standard therapies have high failure rates and little is known about treatment in children. Newer options such as flecainide and left cardiac sympathetic denervation (LCSD) are not well validated. We sought to define treatment outcomes in children with CPVT.
Methods and Results
This is a Pediatric and Congenital Electrophysiology Society (PACES) multicenter, retrospective cohort study of CPVT patients diagnosed before 19 years of age. The cohort included 226 patients, including 170 probands and 56 relatives. Symptomatic presentation was reported in 176 (78%). Symptom onset occurred at 10.8 (IQR 6.8–13.2) years with a delay to diagnosis of 0.5 (0–2.6) years. Syncope (p<0.001), cardiac arrest (p<0.001) and treatment failure (p=0.008) occurred more often in probands. Beta-blockers were prescribed in 205 of 211 patients (97%) on medication, and 25% experienced at least one treatment failure event. Implantable cardioverter defibrillators (ICDs) were placed in 121 (54%) and was associated with electrical storm in 22 (18%). Flecainide was used in 24% and LCSD in 8%. Six deaths (3%) occurred during a cumulative follow-up of 788 patient-years.
Conclusions
This study demonstrates a malignant phenotype and lengthy delay to diagnosis in CPVT. Probands were typically severely affected. Beta-blockers were almost universally initiated; however, treatment failure, non-compliance and sub-therapeutic dosing were often reported. ICDs were common despite numerous device-related complications. Treatment failure was rare in the quarter of patients on flecainide. LCSD was not uncommon although the indication was variable.
This large, multicentre study identifies contemporary challenges related to the diagnosis and prognostication of CPVT patients. Structural modelling of RyR2 can improve our understanding severe CPVT phenotypes. Wakeful rest, rather than exertion, often precipitated life-threatening cardiac events.
The current study sought to assess cognitive and emotional functions among children and adolescents with atrioventricular reentry tachycardia (AVRT) and atrioventricular nodal reentry tachycardia (AVNRT). 113 patients (62 girls and 51 boys ages, 9-18 years) scheduled for radiofrequency ablation due to AVRT or AVNRT underwent neuropsychologic examination. The study excluded patients who had experienced cardiac arrest, congenital heart defects, neurologic disorders, or other diseases affecting cognitive or emotional development. Standardized tests for examining verbal and visual memory as well as visual-spatial functioning were performed. For patients exhibiting deficits in two or more tests, a diagnosis of "cognitive deficits" was determined. Levels of anxiety were tested using the State-Trait Anxiety Inventory. Cognitive deficits were found in 47.8 % of the patients. The age at first arrhythmia attack was related to memory dysfunction. The mean age at which the first symptoms occurred was significantly lower for patients with deficits (8.3 years) than for patients who had no deficit (10.2 years) (t = 2.15; p = 0.03). Boys exhibited a significantly higher level of trait anxiety than girls (t = 3.42; p = 0.0009). A significant negative correlation was found between anxiety and the age at appearance of the first symptoms (r = -0.26; p = 0.005). These findings led us to conclude that cognitive and emotional developments can be negatively affected by AVNRT and AVRT, particularly if tachycardia appears early in life.
Children require often replacement of leads even several times. Repeated extraction in this group nay be challenging. We report the case of a 22-year-old man with the first pacemaker implanted in the first year of life, after Transvenous Lead Extraction procedure (TLE) and implantation of a dual-chamber pacemaker in the age of 12 years, who was admitted to the reference center for repeated TLE and to replace the entire pacing system. The presence of complete venous occlusion, lead strain and strong lead-to-lead adherence with calcified connecting tissue scar effected TLE complicity and needed utility of numerous tools and atypical technique and tricks among of them simultaneous extraction of strongly connected each one leads together via one tool showed to be crucial.
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