19 research outputs found
¿Conocemos los lÃmites del corazón?
Heart diseasesMalalties del corEnfermedades del corazó
Case report: Removal of a subcutaneous implantable cardiac defibrillator in a pediatric patient with hypertrophic cardiomyopathy after a septal myectomy. Insights on current indications of type of ICD in children with hypertrophic cardiomyopathy and left ventricular tract obstruction
Hypertrophic cardiomyopathy; Myectomy; Sudden cardiac deathMiocardiopatia hipertròfica; Miectomia; Mort cardÃaca sobtadaMiocardiopatÃa hipertrófica; MiectomÃa; Muerte cardÃaca súbitaHypertrophic cardiomyopathy is a heart muscle disease with an annual incidence between 0.24 and 0.47/100000 in childhood. Sudden cardiac death is the most common cause of death in this population. Although some medical treatment can decrease the risk of sudden cardiac death, implantable cardioverter defibrillator continues to be the most reliable treatment. Different types of devices and programming strategies can be used in patients with hypertrophic cardiomyopathy depending on each center and specific patient condition. We report a pediatric patient affected with hypertrophic cardiomyopathy who had and ICD implantation in primary prevention. Four years later he developed symptomatic left ventricular outflow tract obstruction and a surgical septal myectomy was performed. After the myectomy the patient developed complete left bundle branch block on his 12 lead ECG, and unfortunately none of the S-ICD vectors were suitable after the myectomy and it had to be explanted and replaced for a new transvenous ICD
Development and Content Validity of the Physical Activity Questionnaire-Young Children (PAQ-YC) to Assess Physical Activity in Children between 5 and 7 Years
Pediatria; Activitat fÃsica; QüestionariPediatrÃa; Actividad fÃsica; CuestionarioPaediatrics; Physical activity; QuestionnaireChildhood is a critical period in the development and consolidation of healthy habits, such as the practice of physical activity (PA). It is essential to have valid instruments to measure PA from an early age. The aim of this study was to design and evaluate the content validity of the Physical Activity Questionnaire-Young Children (PAQ-YC) to measure the PA level in children aged 5–7 years. The first version of the questionnaire was tested by a 2-round Delphi study. It was established as a consensus criterion that the relative interquartile range (RIR) and/or the coefficient of variation (CV) were ≤20%. The most significant discrepancies in the Delphi survey (n = 11–13) were observed for items about hours of Physical Education or similar activities at school (item 7: RIR = 20, CV = 38.73) and for items about participation in Physical Education (item 8: RIR = 25, CV = 15.45). The cognitive interviews (n = 5) confirmed the version agreed by the experts. The results show that the PAQ-YC presents adequate content validity in terms of relevance, comprehensiveness and comprehensibility.This research received no external funding
Different profiles of lipoprotein particles associate various degrees of cardiac involvement in adolescents with morbid obesity
Dyslipidemia secondary to obesity is a risk factor related to cardiovascular disease events, however a pathological conventional lipid profile (CLP) is infrequently found in obese children. The objective is to evaluate the advanced lipoprotein testing (ALT) and its relationship with cardiac changes, metabolic syndrome (MS) and inflammatory markers in a population of morbidly obese adolescents with normal CLP and without type 2 diabetes mellitus, the most common scenario in obese adolescents. Prospective case-control research of 42 morbidly obese adolescents and 25 normal-weight adolescents, whose left ventricle (LV) morphology and function had been assessed. The ALT was obtained by proton nuclear magnetic resonance spectroscopy, and the results were compared according to the degree of cardiac involvement - normal heart, mild LV changes, and severe LV changes (specifically LV remodeling and systolic dysfunction) - and related to inflammation markers [highly-sensitive C-reactive protein and glycoprotein A (GlycA)] and insulin-resistance [homeostatic model assessment for insulin-resistance (HOMA-IR)]. A second analysis was performed to compare our results with the predominant ALT when only body mass index and metabolic syndrome criteria were considered. The three cardiac involvement groups showed significant increases in HOMA-IR, inflammatory markers and ALT ratio LDL-P/HDL-P (40.0 vs. 43.9 vs. 47.1, p 0.012). When only cardiac change groups were considered, differences in small LDL-P (565.0 vs. 625.1 nmol/L, p 0.070), VLDL size and GlycA demonstrated better utility than just traditional risk factors to predict which subjects could present severe LV changes [AUC: 0.79 (95% CI: 0.54-1)]. In the second analysis, an atherosclerotic ALT was detected in morbidly obese subjects, characterized by a significant increase in large VLDL-P, small LDL-P, ratio LDL-P/HDL-P and ratio HDL-TG/HDL-C. Subjects with criteria for MS presented overall worse ALT (specially in triglyceride-enriched particles) and remnant cholesterol values. ALT parameters and GlycA appear to be more reliable indicators of cardiac change severity than traditional CV risk factors. Particularly, the overage of LDL-P compared to HDL-P and the increase in small LDL-P with cholesterol-depleted LDL particles appear to be the key ALT's parameters involved in LV changes. Morbidly obese adolescents show an atherosclerotic ALT and those with MS present worse ALT values
Flecainide Is Associated With a Lower Incidence of Arrhythmic Events in a Large Cohort of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia
BACKGROUND: In severely affected patients with catecholaminergic polymorphic ventricular tachycardia, beta-blockers are often insufficiently protective. The purpose of this study was to evaluate whether flecainide is associated with a lower incidence of arrhythmic events (AEs) when added to beta-blockers in a large cohort of patients with catecholaminergic polymorphic ventricular tachycardia. METHODS: From 2 international registries, this multicenter case cross-over study included patients with a clinical or genetic diagnosis of catecholaminergic polymorphic ventricular tachycardia in whom flecainide was added to beta-blocker therapy. The study period was defined as the period in which background therapy (ie, beta-blocker type [beta1-selective or nonselective]), left cardiac sympathetic denervation, and implantable cardioverter defibrillator treatment status, remained unchanged within individual patients and was divided into pre-flecainide and on-flecainide periods. The primary end point was AEs, defined as sudden cardiac death, sudden cardiac arrest, appropriate implantable cardioverter defibrillator shock, and arrhythmic syncope. The association of flecainide with AE rates was assessed using a generalized linear mixed model assuming negative binomial distribution and random effects for patients. RESULTS: A total of 247 patients (123 [50%] females; median age at start of flecainide, 18 years [interquartile range, 14-29]; median flecainide dose, 2.2 mg/kg per day [interquartile range, 1.7-3.1]) were included. At baseline, all patients used a beta-blocker, 70 (28%) had an implantable cardioverter defibrillator, and 21 (9%) had a left cardiac sympathetic denervation. During a median pre-flecainide follow-up of 2.1 years (interquartile range, 0.4-7.2), 41 patients (17%) experienced 58 AEs (annual event rate, 5.6%). During a median on-flecainide follow-up of 2.9 years (interquartile range, 1.0-6.0), 23 patients (9%) experienced 38 AEs (annual event rate, 4.0%). There were significantly fewer AEs after initiation of flecainide (incidence rate ratio, 0.55 [95% CI, 0.38-0.83]; P=0.007). Among patients who were symptomatic before diagnosis or during the pre-flecainide period (n=167), flecainide was associated with significantly fewer AEs (incidence rate ratio, 0.49 [95% CI, 0.31-0.77]; P=0.002). Among patients with ≥1 AE on beta-blocker therapy (n=41), adding flecainide was also associated with significantly fewer AEs (incidence rate ratio, 0.25 [95% CI, 0.14-0.45]; P<0.001). CONCLUSIONS: For patients with catecholaminergic polymorphic ventricular tachycardia, adding flecainide to beta-blocker therapy was associated with a lower incidence of AEs in the overall cohort, in symptomatic patients, and particularly in patients with breakthrough AEs while on beta-blocker therapy.</p
Flecainide Is Associated With a Lower Incidence of Arrhythmic Events in a Large Cohort of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia
BACKGROUND: In severely affected patients with catecholaminergic polymorphic ventricular tachycardia, beta-blockers are often insufficiently protective. The purpose of this study was to evaluate whether flecainide is associated with a lower incidence of arrhythmic events (AEs) when added to beta-blockers in a large cohort of patients with catecholaminergic polymorphic ventricular tachycardia. METHODS: From 2 international registries, this multicenter case cross-over study included patients with a clinical or genetic diagnosis of catecholaminergic polymorphic ventricular tachycardia in whom flecainide was added to beta-blocker therapy. The study period was defined as the period in which background therapy (ie, beta-blocker type [beta1-selective or nonselective]), left cardiac sympathetic denervation, and implantable cardioverter defibrillator treatment status, remained unchanged within individual patients and was divided into pre-flecainide and on-flecainide periods. The primary end point was AEs, defined as sudden cardiac death, sudden cardiac arrest, appropriate implantable cardioverter defibrillator shock, and arrhythmic syncope. The association of flecainide with AE rates was assessed using a generalized linear mixed model assuming negative binomial distribution and random effects for patients. RESULTS: A total of 247 patients (123 [50%] females; median age at start of flecainide, 18 years [interquartile range, 14-29]; median flecainide dose, 2.2 mg/kg per day [interquartile range, 1.7-3.1]) were included. At baseline, all patients used a beta-blocker, 70 (28%) had an implantable cardioverter defibrillator, and 21 (9%) had a left cardiac sympathetic denervation. During a median pre-flecainide follow-up of 2.1 years (interquartile range, 0.4-7.2), 41 patients (17%) experienced 58 AEs (annual event rate, 5.6%). During a median on-flecainide follow-up of 2.9 years (interquartile range, 1.0-6.0), 23 patients (9%) experienced 38 AEs (annual event rate, 4.0%). There were significantly fewer AEs after initiation of flecainide (incidence rate ratio, 0.55 [95% CI, 0.38-0.83]; P=0.007). Among patients who were symptomatic before diagnosis or during the pre-flecainide period (n=167), flecainide was associated with significantly fewer AEs (incidence rate ratio, 0.49 [95% CI, 0.31-0.77]; P=0.002). Among patients with ≥1 AE on beta-blocker therapy (n=41), adding flecainide was also associated with significantly fewer AEs (incidence rate ratio, 0.25 [95% CI, 0.14-0.45]; P<0.001). CONCLUSIONS: For patients with catecholaminergic polymorphic ventricular tachycardia, adding flecainide to beta-blocker therapy was associated with a lower incidence of AEs in the overall cohort, in symptomatic patients, and particularly in patients with breakthrough AEs while on beta-blocker therapy.</p
Design and evaluation of a new paediatric pretransport risk score
Introducció: els avanços en la tecnologia mèdica s'han associat amb una millora en els resultats clÃnics dels pacients pedià trics ingressats en unitats de cures intensives (UCI). La complexitat de la UCI ha conduït a la reorganització de l'atenció en centres de gran volum i complexitat. Per oferir la mateixa atenció a tota la població independentment d'on viuen, el paper del sistema de transport Inter hospitalari s'ha convertit en essencial per garantir els mateixos resultats clÃnics. Actualment no existeix cap escala de risc estandarditzat que pugui predir la mortalitat, la necessitat d’ingrés a la UCI o la necessitat de realitzar intervencions mèdiques durant el transport des del centres perifèrics a centres de referència en la edat pedià trica. Amb aquest estudi es pretén dissenyar i validar una nova escala de risc pedià trica pretransport (PPTRS).
Pacients i mètodes: Es va dissenyar un estudi observacional prospectiu de pacients pedià trics transportats per l'equip de transport pedià tric de l'Hospital Universitari de la Vall d'Hebron (SEM-P VH) des de l'1 d'octubre de 2010 fins al 30 de juliol de 2017. La població de l'estudi incloïa tots els pacients, des de 0 dies de vida fins a 16 anys d'edat. Es va dissenyar una PPTRS que incloïa 10 variables clÃniques (respiració, oximetria, pressió inspiratòria mà xima, pressió arterial sistòlica, consciència, pupil·les, diüresis, excés de base està ndard, glucosa i temperatura) obtinguts durant la primera trucada de contacte amb el centre de coordinació.
Resultats: D'un total de 4292 pacients inicialment obtinguts de la nostra base de dades, es van excloure 854 (19,8%) casos, que van deixar un total de 3439 pacients per a l'anà lisi final. La distribució d'edat va demostrar que 1597 (46,4%) dels nostres pacients eren nounats, amb 503 (14,6%) dels quals eren nounats prematurs, i 1842 (53,6%) eren nens majors d’un més de vida. El grup patològic principal de la nostra cohort va ser respiratori amb 1402 pacients (40,8%), seguits de causes neurològiques amb 716 pacients (20,8%) i cardÃacs, amb 378 pacients (11,0%). La majoria dels pacients, 2344 (68,2%), van ser ingressats en unitats de cures intensives al arribar als hospitals receptors. Només 165 pacients (4,8%) necessitaven cirurgia urgent. El PPTRS mitjà va ser de 4.55 ± 2.77, amb una mitjana de 4.0 (0-17). La mortalitat global al final del transport va ser del 0,5%. amb 16 pacients que van morir abans d’arribar al hospital receptor La mortalitat a les 48 h després del trasllat va ser del 1,9% (65/3439). Els pacients que morts presentaven un PPTRS significativament més alt en comparació amb els vius (10.95 ± 3.59 vs 4.42 ± 2.57 p <0.001) i l'anà lisi multivariant va mostrar que la PPTRS era l'únic factor independent de la mortalitat (OR: 1.744 (95 IC: 1,599-1,902; p <0,001). Els pacients ingressats en cures intensives presentaven també un PPTRS significativament més elevat en comparació amb aquells pacients que no requereixen ingrés a UCI (3,32 ± 1,77 vs. 5,07 ± 2,88; p <0,001). A més, els PPTRS més alts es van associar amb un major risc de totes les intervencions mèdiques, a excepció de la canalització d’una via perifèrica. Els fà rmacs antiepilèptics i l'ús del sistemes d’immobilització cervical no van demostrar cap relació amb la PPTRS.
Conclusions: La PPTRS és un sistema de puntuació simple i útil per predir la mortalitat 48 h després del transport, la probabilitat d’ingrés a la UCI i la necessitat d'intervencions mèdiques durant el transport.Introduction: Advances in medical technology have been associated with an improvement in clinical outcomes for paediatric patients admitted in intensive care units. The complexity of the ICU has led to centralization of care with high volume and complexity centers. In order to offer the same care to all the population independently of where they live, the role of the interhospital transport system has become essential in order to guarantee the same clinical outcomes.. As far as we know there is no standardised score system specifically designed to use pre transport clinical data to predict mortality and tertiary centre disposition. With this study we seek to design and validate a novel Paediatric PreTransport Risk Score (PPTRS).
Patients and Methods: We designed a prospective observational study of paediatric patients transported by the paediatric transport retrieval team of Vall d’Hebron Hospital (SEM-P VH)) since to 1st of October 2010 until 30th July 2017. The study population included all paediatric patients, from 0 days of life to 16 y old that were transported by our SEM-P VH. A PPTRS was designed including 10 clinical variables (respiration, pulse oximetry, peak inspiratory pressure, systolic blood pressure, consciousness, pupils, diuresis, standard base excess, glucose and temperature) obtained during the first contact call with the coordination center.
Results: Out of a total of 4292 patients initially entered into our database, 854 (19.8%) cases were excluded leaving a total of 3439 patients for the final analysis. The age distribution demonstrated that 1597 (46.4%) of our patients were neonates, with 503 (14.6%) of them that were preterm neonates and 1842 (53.6%) were children. The main pathological group of our cohort was respiratory with 1402 patients (40.8%), followed by neurological causes with 716 patients (20.8%) and cardiac, with 378 patients (11.0%). The majority of patients, 2344 (68.2%), were admitted into intensive care units at their receiving hospitals. Only 165 (4.8%) patients were felt to need urgent surgery. The overall mortality at the end of transport was 0.5% with 16 patients that died at the time of arrival at the destination hospital. Mean PPTRS was 4.55 ± 2.77 with a median of 4.0 (0-17). A total of 65 (1.9%) of the patients died after 48 hours of the transport. Patients who died had a significantly higher PPTRS compared with those patients that were alive 48 h after transport (10.95 ± 3.59 vs 4.42 ± 2.57 p <0.001) and multivariate analysis showed that PPTRS was the only independent factor for mortality ( OR: 1.744 (95% CI: 1.599-1.902; p < 0.001). Patients admitted in intensive care had a significantly higher PPTRS compared with those patients who did not require admission in intensive care (3.32 ± 1.77 vs 5.07 ± 2.88; p <0.001). Furthermore, higher PPTRS were associated with higher risk of all medical interventions but insertion of a peripheral vain access. Antiepileptic drugs and the use of cervical collar did not have any relation with the PPTRS.
Conclusions:
The PPTRS is a simple and useful scoring system to predict the mortality 48 h after transport, need for ICU and need for medical interventions
MiocardiopatÃa restrictiva: la importancia de su diagnóstico precoz
Cardiomyopathy; Early diagnosisMiocardiopatÃa; Diagnóstico precozMiocardiopatia; Diagnòstic precoçComo conclusiones, este artÃculo expone la serie espanola de pacientes pediátricos con MCR más extensa publicada, una entidad infrecuente en pediatrÃa con una evolución clÃnica muy desfavorable y supervivencia libre de muerte o trasplante del 20% a los 5 años