Pavel Vít

Normal limits for heart rate as established using 24-hour ambulatory electrocardiography in children and adolescents.

BACKGROUND To the best of our knowledge, normal limits of heart rate with respect to gender, and as established using 24-hour ambulatory Holter electrocardiography, have yet to be published for the entire age range of children and adolescents. OBJECTIVES To establish the normal limits for heart rate in newborns, infants, children, and adolescents of both genders. PATIENTS AND METHODS We obtained 24-hour Holter recordings from 616 healthy subjects aged from birth to 20 years with structurally normal hearts. The subjects were not receiving medication, and had not been submitted to prior cardiac intervention. Off-line analysis was performed with Mars 8000 scanners, analysing 5 consecutive RR intervals by the software available for automatic calculation of heart rate. All subjects were in sinus rhythm. Best-fit non-linear regressions were applied to correlate age and gender with minimum and mean heart rate, as well as with maximal RR-interval, and to calculate the 5th, 25th, 75th and 95th percentiles. RESULTS We observed significant gender-dependent differences in heart rate for persons aged 10 years and older, with the males exhibiting lower minimal and mean heart rates, and higher RR-intervals, than the females. Correlation of heart rate with age and gender could be established with sufficient accuracy using non-linear regression (p less than 0.0001): Minimum heart rate (male: R(2)=0.778, female: R(2) = 0.664) and mean heart rate (male: R(2) = 0.820, female: R(2) = 0.736) decreased with age, while the maximal RR-interval prolonged (male: R(2) = 0.562, female: R(2) = 0.486). Age and gender-related graphs of centiles were constructed. CONCLUSIONS Heart rate, as documented using Holter recodings, can be correlated with age and gender, permitting establishments of normal gender-specific limits for children and adolescents.

Long-term results of paediatric radiofrequency catheter ablation: a population-based study

AIMS We aimed to evaluate long-term utilization and results of paediatric radiofrequency catheter ablation (RFCA) in a population-based study. METHODS AND RESULTS Data from all three centres performing paediatric RFCA for the whole population of the Czech Republic between 1993 and 2010 were retrospectively reviewed. A total of 708 ablation procedures in 633 consecutive patients <18 years for 716 different substrates were tracked, with accessory pathways = 439 (61.3%) and atrioventricular nodal reentry tachycardia (AVNRT) = 205 (28.6%) being most frequent. Incidence of RFCA reached 0.049 per 1000 children <18 years of age in the recent era (2006-10). Indications included patient preference (68.0%), drug refractoriness (15.5%), asymptomatic Wolff-Parkinson-White pre-excitation (8.4%), and malignant arrhythmia (6.1%). Median follow-up was 13.7 (interquartile range 5.7-21.5) months. Overall acute/long-term success of the primary procedure was 89.1/77.2% (accessory pathways 87.2/77.7%, AVNRT 98.5/84.4%). Re-ablation was performed in 73 of 163 substrates after a primary unsuccessful ablation resulting in a long-term cumulative efficacy of 96.3%. Between 1993-2005 and 2006-10, procedure/fluoroscopy time decreased from median 154/24 to 105/14 min. (P < 0.001 for both). Serious complications occurred in nine patients (1.4%). CONCLUSION This population-based study could replicate data from previous single- or multi-centre reports confirming RFCA as a safe method of arrhythmia treatment in children with long-term cumulative efficacy exceeding 90% and significant decrease in the procedure and fluoroscopy time during the study period. The need for RFCA can be estimated at ∼0.05/1000 children <18 years using current indication criteria.

Electrophysiologic Profile and Results of Invasive Risk Stratification in Asymptomatic Children and Adolescents With the Wolff–Parkinson–White Electrocardiographic Pattern

Background—Data on the results and clinical effect of an invasive risk stratification strategy in asymptomatic young patients with the Wolff–Parkinson–White electrocardiographic pattern are scarce. Methods and Results—Eighty-five consecutive patients aged <18 years with a Wolff–Parkinson–White pattern and persistent preexcitation at maximum exercise undergoing invasive risk stratification were retrospectively studied. Adverse accessory pathway (AP) properties were defined according to currently consented criteria as any of the following: shortest preexcited RR interval during atrial fibrillation/rapid atrial pacing ⩽250 ms (or antegrade effective refractory period ⩽250 ms if shortest preexcited RR interval was not available) or inducible atrioventricular re-entrant tachycardia. Age at evaluation was median 14.9 years. Eighty-two patients had a structurally normal heart and 3 had hypertrophic cardiomyopathy. A single manifest AP was present in 80, 1 manifest and 1 concealed AP in 4, and 2 manifest APs in 1 patient. Adverse AP properties were present in 32 of 85 patients (37.6%) at baseline and in additional 16 of 44 (36.4%) after isoproterenol. Ablation was performed in 41 of these 48 patients. Ablation was deferred in the remaining 7 for pathway proximity to the atrioventricular node. In addition, 18 of the low-risk patients were ablated based on patient/parental decision. Conclusions—Adverse AP properties at baseline were exhibited by 37.6% of the evaluated patients with an asymptomatic Wolff–Parkinson–White preexcitation persisting at peak exercise. Isoproterenol challenge yielded additional 36.4% of those tested at higher risk. Ablation was performed in a total of 69.4% of patients subjected to invasive risk stratification.

Clinical characteristics of 30 Czech families with long QT syndrome and KCNQ1 and KCNH2 gene mutations: importance of exercise testing

BACKGROUND Classic symptoms of long QT syndrome (LQTS) include prolongation of QT interval on electrocardiograph, syncope, and cardiac arrest due to a distinctive form of polymorphic ventricular tachycardia, known as Torsade de Pointes. We assessed occurrence of LQTS signs in individuals from 30 Czech families with mutations in KCNQ1 and KCNH2 genes. METHODS AND RESULTS One hundred five individuals from 30 Czech families with LQTS were genotyped for KCNQ1 and KCNH2. The occurrence of typical LQTS signs (pathologic prolongation of QT interval; syncope; cardiac arrest; Torsade de Pointes) was clinically assessed by exercise test with QT interval analysis. Family history of sudden cardiac death was taken. Statistical analysis was performed to determine correlation of clinical results and mutation status. KCNQ1 gene mutations were found in 23 families, and KCNH2 gene mutations in eight families. Only 46 (70%) of the 66 mutation carriers had at least two of the typical LQTS signs. The others were minimally or asymptomatic. From 39 noncarrier individuals, only 1 fulfilled the clinical criteria of LQTS diagnosis, another 4 had an intermediate probability of diagnosis. The exercise test had 92% sensitivity and 93% specificity for LQTS diagnosis. CONCLUSIONS Incidence of classical signs of LQTS was not high in Czech carriers of KCNQ1 and KCNH2 mutations. Therefore, proper diagnosis relies on detection of symptoms at presentation. The exercise test may be beneficial owing to its high sensitivity and specificity for LQTS diagnosis.

Clinical Characteristics and Mutational Analysis of the RyR2 Gene in Seven Czech Families with Catecholaminergic Polymorphic Ventricular Tachycardia

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare hereditary arrhythmia. The onset of clinical symptoms usually occurs during childhood, and is typically related to exercise. The aim of our study was to describe the clinical characteristics of seven Czech families with CPVT and the results of mutational analysis of the RyR2 gene in these families.