Changes in Central Aortic Pressure Levels, Wave Components and Determinants Associated with High Peripheral Blood Pressure States in Childhood: Analysis of Hypertensive Phenotype.

The aims were to determine whether children's high peripheral blood pressure states (HBP) are associated with increased central aortic blood pressure (BP) and to characterize hemodynamic and vascular changes associated with HBP in terms of changes in cardiac output (stroke volume, SV), arterial stiffness (aortic pulse wave velocity, PWV), peripheral vascular resistances (PVR) and net and relative contributions of reflected waves to the aortic pulse amplitude. We included 154 subjects (mean age 11; range 4-16 years) assigned to one of two groups: normal peripheral BP (NBP, n = 101), defined as systolic and diastolic BP < 90th percentile, or high BP (HBP, n = 53), defined as average systolic and/or diastolic BP levels ≥90th percentile (curves for sex, age and body height). The HBP group included children with hypertensive and pre-hypertensive BP levels. After a first analysis, groups were compared excluding obese and dyslipidemic children. Peripheral and central aortic BP, PWV and pulse wave-derived parameters (augmentation index, forward and backward wave components' amplitude) were measured using gold-standard techniques, applanation tonometry (SphygmoCor) and oscillometry (Mobil-O-Graph). Independent of the presence of dyslipidemia and/or obesity, aortic systolic and pulse BP were higher in HBP than in NBP children. The increase in central BP could not be explained by an increase in the relative contribution of reflections to the aortic pressure wave, higher PVR or by an augmented peripheral reflection coefficient. Instead, the rise in central BP would be explained by an increase in the amplitude of both incident and reflected wave components.

2024 SIAC guidelines on cardiorespiratory rehabilitation in pediatric patients with congenital heart disease.

The 2024 Interamerican Society of Cardiology (SIAC) guidelines on cardiorespiratory rehabilitation (CRR) in pediatric patients with congenital heart disease aim to gather and evaluate all relevant evidence available on the topic to unify criteria and promote the implementation of CRR programs in this population in Latin America and other parts of the world. Currently, there is no unified CRR model for the pediatric population. Consequently, our goal was to create these CRR guidelines adapted to the characteristics of congenital heart disease and the physiology of this population, as well as to the realities of Latin America. These guidelines are designed to serve as a support for health care workers involved in the care of this patient group who wish to implement a CRR program in their workplace. The guidelines include an easily reproducible program model that can be implemented in any center. The members of this Task Force were selected by the SIAC on behalf of health care workers dedicated to the care of pediatric patients with congenital heart disease. To draft the document, the selected experts performed a thorough review of the published evidence.

[Use of ivabradine for the treatment of congenital junctional ectopic tachycardia]. / Uso de la ivrabadina para el tratamiento de la taquicardia ectópica de la unión congénita.

The congenial form of junctional ectopic tachycardia is a rare variant of pediatric tachyarrhythmia that due to its incessant nature and its refractoriness to the traditionally used antiarrhythmic agents has a high morbimortality The clinical cases of two patients with a diagnosis of congenital junctional ectopic tachycardia with inadequate response to the regular pharmacological options, who developed dilated cardiomyopathy and ventricular dysfunction secondary to sustained tachycardia, are presented. In both ivrabadine, a new innovative option was used with excellent clinical response.

La taquicardia ectópica de la unión en su variante congénita es una taquiarritmia pediátrica poco frecuente, que por su naturaleza incesante y su refractariedad a los agentes farmacológicos tradicionales lleva asociada una alta morbimortalidad. Se presentan los casos clínicos de dos pacientes pediátricos con diagnóstico de taquicardia ectópica de la unión congénita, que mostraron respuesta inadecuada a las alternativas de tratamiento habituales y que, en consecuencia, desarrollaron miocardiopatía dilatada y disfunción ventricular secundaria a la taquicardia sostenida. En ambos se utilizó ivabradina como alternativa farmacológica innovadora pare el control de ésta con excelente respuesta clínica.

Changes in Body Size during Early Growth Are Independently Associated with Arterial Properties in Early Childhood.

Nutritional status in early life stages has been associated with arterial parameters in childhood. However, it is still controversial whether changes in standardized body weight (z-BW), height (z-BH), BW for height (z-BWH) and/or body mass index (z-BMI) in the first three years of life are independently associated with variations in arterial structure, stiffness and hemodynamics in early childhood. In addition, it is unknown if the strength of the associations vary depending on the growth period, nutritional characteristics and/or arterial parameters analyzed. AIMS: First, to compare the strength of association between body size changes (Δz-BW, Δz-BH, Δz-BWH, Δz-BMI) in different time intervals (growth periods: 0-6, 0-12, 0-24, 0-36, 12-24, 12-36, 24-36 months (m)) and variations in arterial structure, stiffness and hemodynamics at age 6 years. Second, to determine whether the associations depend on exposure to cardiovascular risk factors, body size at birth and/or on body size at the time of the evaluation (cofactors). Anthropometric (at birth, 6, 12, 24, 36 m and at age 6 years), hemodynamic (peripheral and central (aortic)) and arterial (elastic (carotid) and muscular (femoral) arteries; both hemi-bodies) parameters were assessed in a child cohort (6 years; n =632). The association between arterial parameters and body size changes (Δz-BW, Δz-BH, Δz-BWH, Δz-BMI) in the different growth periods was compared, before and after adjustment by cofactors. RESULTS: Δz-BW 0-24 m and Δz-BWH 0-24 m allowed us to explain inter-individual variations in structural arterial properties at age 6 years, with independence of cofactors. When the third year of life was included in the analysis (0-36, 12-36, 24-36 m), Δz-BW explained hemodynamic (peripheral and central) variations at age 6 years. Δz-BH and Δz-BMI showed limited associations with arterial properties. CONCLUSION: Δz-BW and Δz-BWH are the anthropometric variables with the greatest association with arterial structure and hemodynamics in early childhood, with independence of cofactors.

Stroke volume and cardiac output non-invasive monitoring based on brachial oscillometry-derived pulse contour analysis: Explanatory variables and reference intervals throughout life (3-88 years).

BACKGROUND: Non-invasive assessment of stroke volume (SV), cardiac output (CO) and cardiac index (CI) has shown to be useful for the evaluation, diagnosis and/or management of different clinical conditions. Through pulse contour analysis (PCA) cuff­based oscillometric devices would enable obtaining ambulatory operator-independent non-invasive hemodynamic monitoring. There are no reference intervals (RIs), when considered as a continuum in childhood, adolescence and adult life, for PCA-derived SV [SV(PCA)], CO [CO(PCA)] and CI [CI(PCA)]. The aim of the study were to analyze the associations of SV(PCA), CO(PCA) and CI(PCA) with demographic, anthropometric, cardiovascular risk factors (CVRFs) and hemodynamic parameters, and to define RIs and percentile curves for SV(PCA), CO(PCA) and CI(PCA), considering the variables that should be considered when expressing them. METHODS: In 1449 healthy subjects (3-88 years) SV(PCA), CO(PCA) and CI(PCA) were non-invasively obtained (Mobil-O-Graph; Germany). ANALYSIS: associations between subject characteristics and SV(PCA), CO(PCA) and CI(PCA) levels (correlations; regression models); RIs and percentiles for SV(PCA), CO(PCA) and CI(PCA) (parametric methods; fractional polynomials). RESULTS: Sex, age, and heart rate would be explanatory variables for SV, CO, and CI levels. SV levels were also examined by body height, while body surface area (BSA) contributing to evaluation of CO and CI. CVRFs exposure did not contribute to independently explain the values of the dependent variables. SV, CO and CI levels were partially explained by the oscillometric-derived signal quality. RIs and percentiles were defined. CONCLUSIONS: Reference intervals and percentile for SV(PCA), CO(PCA) and CI(PCA), were defined for subjects from 3-88 years of age, results are expressed according to sex, age, heart rate, body height and/or BSA.

Arterial Structural and Functional Characteristics at End of Early Childhood and Beginning of Adulthood: Impact of Body Size Gain during Early, Intermediate, Late and Global Growth.

An association between nutritional characteristics in theearlylife stages and the state of the cardiovascular (CV) system in early childhood itself and/or at the beginning of adulthood has been postulated. It is still controversial whether changes in weight, height and/or body mass index (BMI) during childhood or adolescence are independently associated with hemodynamics and/or arterial properties in early childhood and adulthood. AIMS: First, to evaluate and compare the strength of association between CVproperties (at 6 and 18 years (y)) and (a) anthropometric data at specific growth stages (e.g., birth, 6 y, 18 y) and (b) anthropometric changes during early (0-2 y), intermediate (0-6 y), late (6-18 y) and global (0-18 y) growth. Second, to determine whether the associations between CVproperties and growth-related body changes depend on size at birth and/or at the time of CVstudy. Third, to analyze the capacity of growth-related body size changes to explain hemodynamic and arterial properties in early childhood and adulthood before and after adjusting for exposure to CV risk factors. Anthropometric, hemodynamic (central, peripheral) and arterial parameters (structural, functional; elastic, transitional and muscular arteries) were assessed in two cohorts (children, n = 682; adolescents, n = 340). Data wereobtained and analyzed following identical protocols. RESULTS: Body-size changes in infancy (0-2 y) and childhood (0-6 y) showed similar strength of association with CV properties at 6 y. Conversely, 0-6, 6-18 or 0-18 ychanges were not associated with CV parameters at 18 y. The association between CV properties at 6 yand body-size changes during growth showed: equal or greater strength than the observed for body-size at birth, and lower strength compared to that obtained for current z-BMI. Conversely, only z-BMI at 18 y showed associations with CV z-scores at 18 y. Body size at birth showed almost no association with CVproperties at 6 or 18 y. CONCLUSION: current z-BMI showed the greatest capacity to explain variations in CV properties at 6 and 18 y. Variations in some CV parameters were mainly explained by growth-related anthropometric changes and/or by their interaction with current z-BMI. Body size at birth showed almost no association with arterial properties at 6 or 18 y.

Aortic pressure and forward and backward wave components in children, adolescents and young-adults: Agreement between brachial oscillometry, radial and carotid tonometry data and analysis of factors associated with their differences.

Non-invasive devices used to estimate central (aortic) systolic pressure (cSBP), pulse pressure (cPP) and forward (Pf) and backward (Pb) wave components from blood pressure (BP) or surrogate signals differ in arteries studied, techniques, data-analysis algorithms and/or calibration schemes (e.g. calibrating to calculated [MBPc] or measured [MBPosc] mean pressure). The aims were to analyze, in children, adolescents and young-adults (1) the agreement between cSBP, cPP, Pf and Pb obtained using carotid (CT) and radial tonometry (RT) and brachial-oscillometry (BOSC); and (2) explanatory factors for the differences between approaches-data and between MBPosc and MBPc.1685 subjects (mean/range age: 14/3-35 y.o.) assigned to three age-related groups (3-12; 12-18; 18-35 y.o.) were included. cSBP, cPP, Pf and Pb were assessed with BOSC (Mobil-O-Graph), CT and RT (SphygmoCor) records. Two calibration schemes were considered: MBPc and MBPosc for calibrations to similar BP levels. Correlation, Bland-Altman tests and multiple regression models were applied. Systematic and proportional errors were observed; errors´ statistical significance and values varied depending on the parameter analyzed, methods compared and group considered. The explanatory factors for the differences between data obtained from the different approaches varied depending on the methods compared. The highest cSBP and cPP were obtained from CT; the lowest from RT. Independently of the technique, parameter or age-group, higher values were obtained calibrating to MBPosc. Age, sex, heart rate, diastolic BP, body weight or height were explanatory factors for the differences in cSBP, cPP, Pf or Pb. Brachial BP levels were explanatory factors for the differences between MBPosc and MBPc.

Increases in Peripheral Systolic Pressure Levels and Z-score Associate Gradual Aortic Pressure Increase and Functional Arterial Impairment in Children and Adolescents.

BACKGROUND: Arterial changes associated with children and adolescents high blood pressure (HBP) states would vary depending on the arterial type, arterial indexes considered and/or on blood pressure (BP) levels. AIMS: To determine in children and adolescents: 1) if there is gradual structural-functional arterial impairment associated with gradual peripheral (brachial) systolic BP (pSBP) level or z-score increases, and 2) whether subjects with HBP levels and those with normal BP differ in the profiles of arterial changes associated with pSBP deviations. METHODS: 1005 asymptomatic children and adolescents were included. Clinical, anthropometric and arterial non-invasive evaluations were performed. Heart rate, brachial BP, aortic BP and wavederived parameters (i.e. augmentation index), carotid and femoral diameters, blood velocities and elastic modulus, carotid intima-media thickness and aortic pulse wave velocity, were obtained. Two groups were assembled: Reference (without cardiovascular risk factors (CVRFs); n=379) and HBP (n=175). Additionally, subjects were ascribed to groups according to their pSBP z-scores (z-score ≤ 0, 0< z-score < 1 or z-score ≥ 1). Age and sex-related mean and standard deviation equations were obtained for each variable (Reference group). Using those equations, data (entire population) were converted into z-scores. Groups were compared (absolute and z-scored variables) before and after adjusting for cofactors (ANOVA/ANCOVA). Linear regression analyses were done considering: pSBP and z-pSBP (independent) and absolute levels and z-scores for hemodynamic and arterial indexes (dependent variables). Differences in hemodynamic and arterial levels and z-scores variations (dependent) associated with variations in pSBP and z-pSBP (independent variable) were assessed. The slopes of the models for Reference and HBP groups were compared. CONCLUSION: HBP states associate hemodynamic and arterial changes not explained by exposure to other CVRFs, anthropometric or demographic factors. The higher the pSBP deviations from ageand sex-expected mean value in the Reference group, the higher the hemodynamic and arterial indexes deviation. The pSBP-related variations in hemodynamic and arterial indexes would not differ depending on whether HBP states are present or not.

Peripheral and Central Aortic Pressure, Wave-Derived Reflection Parameters, Local and Regional Arterial Stiffness and Structural Parameters in Children and Adolescents: Impact of Body Mass Index Variations.

AIM: The aim was to analyze and compare the associations between body mass index (BMI) and structural and functional cardiovascular variables measured in children and adolescents. METHODS: 609 healthy subjects (mean age/range 12/4-18 years, 45% females) were studied. Subjects' BMI and the corresponding z-scores (z-BMI) were determined. Cardiovascular measurements: peripheral and aortic blood pressure (BP), aortic wave-derived parameters, common carotid, femoral and brachial artery diameters and stiffness, carotid intima-media thickness, carotid-radial and carotid-femoral pulse wave velocity (crPWV, cfPWV) and cfPWV/crPWV ratio. Cardiovascular data were standardized (z-scores) using equations (fractional polynomials) obtained from a sub-group (reference population, n = 241) non-exposed to cardiovascular risk factors (CVRFs). Simple and multiple regression models were obtained for the associations between cardiovascular z-scores and z-BMI and/or z-BMI, age, sex and CVRFs. RESULTS: z-BMI was associated with standardized cardiovascular variables, regardless of age, sex and CVRFs. BP (peripheral rather than aortic) was the variable with the greatest variations associated with z-BMI. Systolic (SBP) and pulse pressure (PP; in that order) were the variables with the highest variations associated with z-BMI. Carotid, but not femoral or brachial stiffness showed BP-dependent variations associated with z-BMI. Arterial diameters were associated with z-BMI, without differences among arteries. CONCLUSION: In children and adolescents, z-BMI was gradually and positively associated with haemodynamic (peripheral and central BP) and vascular parameters (structural and functional) with independence of age, sex and other CVRFs (Dyslipidemia, Hypertension, Smoke, Diabetes). There were differences in the associations depending on the arteries studied and on whether central or peripheral haemodynamic parameters were analyzed.

Forward and Backward Aortic Components and Reflection Indexes in Children and Adolescents: Determinants and Role in High Pressure States.

BACKGROUND: High blood pressure states (HBP) would differ in wave components and reflections indexes, which could associate clinical and prognostic implications. The study aims: 1) to characterize the association of aortic wave components and reflection parameters (backward [Pb], forward [Pf], Pb/Pf ratio and augmentation index [AIx]) with demographic, anthropometric, hemodynamic and arterial parameters in healthy children and adolescents; 2) to generate multivariate prediction models for the associations, to contribute to understand the main determinants of Pf, Pb, Pb/Pf and AIx; 3) to identify if differences in wave reflection indexes observed in HBP could be explained by differences in the analyzed parameters. METHODS: Healthy children and adolescents (n=816, females: 386; Age: 3-20 years) were studied. EVALUATIONS: central aortic pressure and wave components (Pb, Pf, Pb/Pf and AIx determination with SphygmoCor [SCOR] and Mobil-o-Graph [MOG]); anthropometric assessment; regional arterial stiffness (carotid-femoral, carotid-radial pulse wave velocity [PWV] and PWV ratio); carotid intima-media thickness; carotid and femoral distensbility; cardiac output; systemic vascular resistances (SVR). Simple and multiple regression models were constructed to determine aortic wave parameters; the main explanatory variables. Normotensive and HBP groups were compared. Differences in wave reflection indexes were analyzed before and after controlling for explanatory variables. Equivalences between SphygmoCor and Mobil-O-Graph data were assessed (correlation and Bland-Altman analyses). RESULTS AND CONCLUSION: There were systematic and proportional differences between the data obtained with SphygmoCor and Mobil-O-Graph devices. Heart rate (HR), peripheral pulse pressure, height and weight were the variables that isolated (simple associations) or combined (multiple associations), showed the major capability to explain interindividual differences in Pf, Pb, Pb/Pf and AIx. Arterial stiffness also showed explanatory capacity, being the carotid the artery with the major contribution. HBP associated higher Pf, Pb, AIx and lower Pb/Pf ratio. Those findings were observed together with higher weight, arterial stiffness and HR. After adjusting for anthropometric characteristics, HR, cardiac output and SVR, the HBP group showed greater Pf and Pb. Then, Pf and Pb characteristics associated with HBP would not be explained by anthropometric or hemodynamic factors. Evaluating wave components and reflection parameters could contribute to improve the comprehension and management of HBP states.

Complete Resection of a Right-Sided Interventricular Septal Fibroma.

A 15-month-old infant with a systolic cardiac murmur developed an episode of nonsustained ventricular tachycardia during transthoracic echocardiography. A large intracardiac mass, which widened the interventricular septum causing right ventricular outflow tract obstruction, was seen and confirmed by magnetic resonance imaging. A cardiac fibroma was suspected, and because of the risk of life-threatening arrhythmias, surgery was rapidly considered. Despite the challenging location, the tumor was completely and successfully resected. Microscopic examination confirms the diagnosis of ventricular fibroma.

Children and Adolescents Macrovascular Reactivity Level and Dynamics, But Not the Microvascular Response, is Associated with Body Mass Index and Arterial Stiffness Levels.

INTRODUCTION: The existing evidence indicates that primary prevention of atherosclerotic disease should begin in childhood. Detection of children and adolescents at risk for atherosclerosis may allow early intervention to decrease the atherosclerotic process, thereby preventing or delaying cardiovascular disease. Vascular reactivity (VR) is altered even by early atherosclerosis. Obesity is a main cardiovascular risk factor (CVRF) observed in childhood. If childhood obesity associates impaired macro and/or micro VR is controversial. AIMS: To characterize macro and micro VR analyzing the stimulus and vascular response temporal profiles in children and adolescents considering their body mass index (BMI); and to assess potential associations between subjects' characteristics and the hyperemic stimulus and/or VR. METHODS: Healthy subjects (n = 99, age 5-17 years, female 46%) were included. Considering the BMI, normal, overweight and obese groups were defined. CVRF exposure was assessed. Brachial flow-mediated dilation and reactive hyperemia, associated with transient ischemia (forearm cuff-inflation) were evaluated. Diameter, flow velocities, resistive indexes and shear-stress were determined before, during and after cuff-release. Complimentary VR indexes were computed. Aortic stiffness and aortic and brachial blood pressure were determined. RESULTS: Obese showed the lowest and slowest macrovascular response (p < 0.05). Micro VR was not associated with obesity. Higher aortic stiffness levels were associated with slower macrovascular responses (p < 0.05). CONCLUSION: Childhood obesity associates not only reduced, but also slowed macrovascular reactivity. Microvascular response to transient ischemia is preserved in obese children. Macro and microvascular responses would be non-associated phenomena in childhood. During childhood, VR dynamics would depend on the arterial stiffness.

Comparative Analysis of Arterial Parameters Variations Associated with Inter-Individual Variations in Peripheral and Aortic Blood Pressure: Cross-Sectional Study in Healthy Subjects Aged 2-84 years.

INTRODUCTION: The association between arterial parameters and blood pressure (BP) interindividual variations could depend on the arterial segment, BP component (systolic, SBP; diastolic, DBP; pulse pressure, PP) and/or on whether central (cBP) or peripheral (pBP) BP variations are considered. AIM: To assess and compare arterial parameters variations associated with interindividual variations in cBP and pBP. METHODS: Healthy subjects (n = 923; 488 males, 2-84 years) were included. pBP and cBP waves were obtained (Mobil-O-Graph; SphygmoCor). Arterial diameter, intima-media thickness, local elastic modulus (carotid, CEM; brachial, BEM; femoral, FEM) and regional (carotid-radial and carotid-femoral pulse wave velocity; crPWV and cfPWV) arterial stiffness were determined. Associations between BP and arterial parameters interindividual variations were analyzed and compared (correlations; linear regressions; slopes comparisons) considering data transformed into z-scores. RESULTS: Given a variation in z-cSBP or z-pSBP, z-CEM, z-FEM and z-cfPWV (stiffness indexes), were among the parameters with major BP-associated variations. z-crPWV and z-cfPWV, rather than local stiffness indexes were the parameters with major variations associated with z-DBP variations. z-cPP or z-pPP were associated with z-CEM and z-FEM variations, but not with brachial or regional stiffness variations. Most of the arterial parameters-BP slopes did not show significant differences when considering a variation in z-cSBP and z-pSBP. z-CEM and z-FEM were mainly associated with z-cPP and z-pPP variations, respectively. CONCLUSION: Disregard of age and sex, the variations in arterial parameters associated with BP interindividual variations showed differences depending on whether variations were central or peripheral; in SBP, DBP or PP and depending on the arterial segment considered.

High Central Aortic Rather than Brachial Blood Pressure is Associated with Carotid Wall Remodeling and Increased Arterial Stiffness in Childhood.

INTRODUCTION: In adults, central blood pressure (cBP) is reported to associate target organ damages (TODs) rather than peripheral blood pressure (pBP). However, data regarding the association of pre-clinical TODs with cBP and pBP in pediatric populations are scarce. AIM: To evaluate in children and adolescents the importance of cBP and pBP levels, in terms of their association with hemodynamic and vascular changes. METHODS: 315 subjects [age (mean/range) 12/8-18 years] were included. MEASUREMENTS: pBP (oscillometry, Omron-HEM433INT and Mobil-O-Graph), cBP levels and waveforms (oscillometry, Mobil-O-Graph; applanation tonometry, SphygmoCor), aortic wave reflection-related parameters, carotid intima-media thickness (CIMT) and carotid (elastic modulus, stiffness-index) and aortic stiffness (carotid-femoral pulse wave velocity, PWV). Four groups were defined considering pBP and cBP percentiles (th): cBP ≥90th, cBP <90th, pBP ≥90th, pBP <90th. In each group, haemodynamic and vascular parameters were compared for subgroups defined considering the level of the remaining blood pressure (cBP or pBP). Subgroups were matched for anthropometric and cardiovascular risk factors (propensity matching-score). RESULTS: Subjects with high cBP showed a worse cardiovascular risk profile in addition to worse peripheral hemodynamic conditions. The CIMT, carotid and aortic stiffness levels were also higher in those subjects. CIMT and carotid stiffness remained statistically higher when subjects were matched for pBP and other cardiovascular risk factors. There were no differences in arterial properties when subjects were analyzed (compared) considering similar pBP levels, during normal and high cBP conditions. CONCLUSION: Compared with pBP, the cBP levels show a greater association with vascular alterations (high CIMT and arterial stiffness), in children and adolescents.

Uso de la ivrabadina para el tratamiento de la taquicardia ectópica de la unión congénita / Use of ivabradine for the treatment of congenital junctional ectopic tachycardia

Resumen La taquicardia ectópica de la unión en su variante congénita es una taquiarritmia pediátrica poco frecuente, que por su naturaleza incesante y su refractariedad a los agentes farmacológicos tradicio nales lleva asociada una alta morbimortalidad. Se presentan los casos clínicos de dos pacientes pediátricos con diagnóstico de taquicardia ectópica de la unión congénita, que mostraron respuesta inadecuada a las alternativas de tratamiento habituales y que, en consecuencia, desarrollaron miocardiopatía dilatada y disfunción ventricular secundaria a la taquicardia sostenida. En ambos se utilizó ivabradina como alternativa farmacológica innovadora pare el control de ésta con excelente respuesta clínica.

Abstract The congenial form of junctional ectopic tachycardia is a rare variant of pediatric tachyarrhythmia that due to its incessant nature and its refractoriness to the traditionally used antiarrhythmic agents has a high morbimortality The clinical cases of two patients with a diagnosis of congenital junctional ectopic tachycardia with inadequate response to the regular pharmacological options, who developed dilated cardiomyopathy and ventricular dysfunc tion secondary to sustained tachycardia, are presented. In both ivrabadine, a new innovative option was used with excellent clinical response.

Childhood Obesity Associates Haemodynamic and Vascular Changes That Result in Increased Central Aortic Pressure with Augmented Incident and Reflected Wave Components, without Changes in Peripheral Amplification.

The aims were to determine if childhood obesity is associated with increased central aortic blood pressure (BP) and to characterize haemodynamic and vascular changes associated with BP changes in obese children and adolescents by means of analyzing changes in cardiac output (stroke volume, SV), arterial stiffness (aortic pulse wave velocity, PWV), peripheral vascular resistances (PVR), and net and relative contributions of reflected waves to the aortic pulse wave amplitude. We included 117 subjects (mean/range age: 10 (5-15) years, 49 females), who were obese (OB) or had normal weight (NW). Peripheral and central aortic BP, PWV, and pulse wave-derived parameters (augmentation index, amplitude of forward and backward components) were measured with tonometry (SphygmoCor) and oscillometry (Mobil-O-Graph). With independence of the presence of dyslipidemia, hypertension, or sedentarism, the aortic systolic and pulse BP were higher in OB than in NW subjects. The increase in central BP could not be explained by the elevation in the relative contribution of reflections to the aortic pressure wave and higher PVR or by an augmented peripheral reflection coefficient. Instead, the rise in central BP could be explained by an increase in the amplitude of both incident and reflect wave components associated to augmented SV and/or PWV.

Children and Adolescent Obesity Associates with Pressure-Dependent and Age-Related Increase in Carotid and Femoral Arteries' Stiffness and Not in Brachial Artery, Indicative of Nonintrinsic Arterial Wall Alteration.

Aim. To analyze if childhood obesity associates with changes in elastic, transitional, and/or muscular arteries' stiffness. Methods. 221 subjects (4-15 years, 92 females) were assigned to normal weight (NW, n = 137) or obesity (OB, n = 84) groups, considering their body mass index z-score. Age groups were defined: 4-8; 8-12; 12-15 years old. Carotid, femoral, and brachial artery local stiffness was determined through systodiastolic pressure-diameter and stress-strain relationships. To this end, arterial diameter and peripheral and aortic blood pressure (BP) levels and waveforms were recorded. Carotid-femoral, femoropedal, and carotid-radial pulse wave velocities were determined to evaluate aortic, lower-limb, and upper-limb regional arterial stiffness, respectively. Correlation analysis between stiffness parameters and BP was done. Results. Compared to NW, OB subjects showed higher peripheral and central BP and carotid and femoral stiffness, reaching statistical significance in subjects aged 12 and older. Arterial stiffness differences disappeared when levels were normalized for BP. There were no differences in intrinsic arterial wall stiffness (elastic modulus), BP stiffness relationships, and regional stiffness parameters. Conclusion. OB associates with BP-dependent and age-related increase in carotid and femoral (but not brachial) stiffness. Stiffness changes would not be explained by intrinsic arterial wall alterations but could be associated with the higher BP levels observed in obese children.

Optimización de la protección en radiología y cardiología intervencionista pediatrica en América Latina y el Caribe (OPRIPALC) / Optimization of protection in pediatric interventional cardiology and radiology in Latin America and the Caribbean (OPRIPALC)

El objetivo del presente artículo ha sido describir el programa "Optimización de la Protección en Radiología Intervencionista Pediátrica en América Latina y el Caribe" (OPRIPALC) que nace el año 2018 como respuesta conjunta de la Organización Panamericana de la Salud y la Organización Mundial de la Salud, en cooperación con el Organismo Internacional de Energía Atómica, para colaborar con sus Estados miembros en asegurar que las exposiciones a la radiación de los pacientes pediátricos sean las mínimas necesarias durante los procedimientos intervencionistas. Actualmente, hay 18 centros de los siguientes 10 países que participan: Argentina, Brasil, Chile, Colombia, Costa Rica, Cuba, Ecuador, México, Perú y Uruguay. Para el desarrollo del programa se plantean una serie de objetivos, productos, actividades y resultados esperados. La puesta en marcha de la WEB de OPRIPALC ha significado un instrumento muy válido para seguir la información actualizada del programa. Un programa actualizado de formación en radioprotección para los profesionales implicados en el programa, se está realizando por medio de "webinars". Se deberá seguir actuando en la aplicación del programa de control de calidad básico para los equipos de rayos X participantes y validar los valores de los Niveles de Referencia para Diagnóstico (NRDs). Se propone formar un equipo de trabajo entre los Físicos Médicos y Tecnólogos Médicos participantes de OPRIPALC para implicarse en las pruebas de control básicas que todos los centros debieran realizar. Se han presentado algunos resultados iniciales de OPRIPALC en eventos científicos internacionales. Se está avanzando en proponer unos primeros valores sobre NRDs en procedimientos de intervencionismo cardiológico pediátrico por bandas de edad y peso. OPRIPALC es una de las pocas iniciativas de carácter regional para obtener valores de NRDs en procedimientos intervencionistas pediátricos. Se espera que tanto los valores de referencia como la metodología empleada en OPRIPALC, puedan ser utilizados en otras regiones del mundo.

The objective of this article has been to describe the program "Optimization of Protection in Pediatric Interventional Radiology in Latin America and the Caribbean" (OPRIPALC) that was born in 2018 as a joint response of the Pan American Health Organization and the World Organization of the Health, in cooperation with the International Atomic Energy Agency, to collaborate with its member states in ensuring that radiation exposures of pediatric patients are the minimum necessary during interventional procedures. Currently, there are 18 centers from the following 10 countries participating: Argentina, Brazil, Chile, Colombia, Costa Rica, Cuba, Ecuador, Mexico, Peru and Uruguay. For the development of the program, a series of objectives, products, activities and expected results are proposed. The launch of the OPRIPALC WEBSITE has been a very valid instrument for following up-to-date information on the program. An updated training program in radiation protection for the professionals involved in the program is being carried out through webinars. It should continue acting in the application of the basic quality control program for the participating X-ray equipment and validate the values of the Diagnostic Reference Levels (DRLs). It is proposed to form a work team among the OPRIPALC participating medical physicists to get involved in the basic control tests that all centers should carry out. Some initial results of OPRIPALC have been presented at international scientific events. Progress is being made in proposing first values on DRLs in pediatric cardiac intervention procedures by age and weight bands. OPRIPALC is one of the few regional initiatives to obtain DRLs values in pediatric interventional procedures. It is expected that both the reference values and the methodology used in OPRIPALC can be used in other regions of the world.

Captura y extracción percutánea de cuerpos extraños intravasculares / Percutaneous retrieval of intravascular foreign bodies / Remoção percutânea de corpos estranos intravenosos

Resumen: En el cuidado del paciente pediátrico en el ámbito del centro de tratamiento intensivo y en sala de hemodinamia se utilizan materiales (guías, catéteres, dispositivos de cierre) que en ocasiones pueden quedar libres en el árbol vascular y embolizar, sea a nivel venoso como arterial. Esto implica la necesidad de contar obligatoriamente, en esos lugares, con material adecuado para su extracción percutánea de forma de evitar las complicaciones potenciales por la permanencia de esos cuerpos a nivel intravascular. Se presenta en este trabajo nuestra experiencia de 20 años en la captura y extracción por vía percutánea de 40 cuerpos intravasculares, a saber: 25 restos de catéteres venosos centrales, 7 restos de porth a cath, 1 guía metálica y 7 coils embolizados. En 35 pacientes la vía utilizada para el rescate fue la vía venosa femoral (en un caso se asoció el acceso por vía venosa yugular) y en los cinco restantes fue la vía arterial femoral. El material utilizado fue en cinco casos un catéter pigtail (en casos de restos de catéteres) para la movilización y localización en una posición más accesible. Luego, en ocho casos, se utilizó un catéter cestilla, en 29 casos un catéter lazo (Goose Neck Snare) y en tres casos un biótomo, para la captura y extracción de los materiales embolizados. Treinta y nueve cuerpos embolizados fueron rescatados sin complicaciones, uno no pudo ser extraído por estar firmemente adherido al ápex ventricular derecho.

Summary: Handling pediatric patients at Intensive Care Centers and in catheterization rooms often leads to using materials (guidewires, catheters, closure devices) that may sometimes remain in the vascular tree and cause embolism, either at the venous or arterial level. This means that ICUs should have material to perform percutaneous retrieval, in order to avoid potential complications due to the intravascular presence of these bodies. In 20 years, we have retrieved 40 intravascular bodies percutaneously: 25 remains of central venous catheters, 7 remains of Porth to Cath, 1 metallic guide and 7 embolized coils. We used the femora vein in 35 patients (in one case, we also used jugular venous access) and in the remaining three patients we used the femoral arterial route. In 5 cases, we used a Pigtail catheter (in case of catheter remains) in order to move and place patients in a more accessible position. In 8 cases we used a catheter, in 27 cases a loop catheter (Goose Neck Snare) and in 3 cases we used a bioptome in order to capture and retrieve the embolized materials. We managed to extract 37 embolized objects without complications, one could not be extracted because it was firmly attached to the right ventricular apex.

Resumo: A manipulação de pacientes pediátricos nas Unidades de Terapia Intensiva e nas salas de cateterismo geralmente leva ao uso de materiais (fios-guia, cateteres, dispositivos de fechamento) que às vezes podem permanecer na árvore vascular e causar embolia, no nível venoso ou arterial. Isso significa que as UTIs devem ter material para realizar a remoção percutânea, a fim de evitar possíveis complicações devido à presença intravascular desses corpos. Em 20 anos, recuperamos 40 corpos intravasculares por via percutânea: 25 restos de cateteres venosos centrais, 7 restos de Porth to Cath, 1 guia metálico e 7 bobinas embolizadas. Utilizamos a veia femoral em 35 pacientes (em um caso, também utilizamos acesso venoso jugular) e nos três pacientes restantes utilizamos a via arterial femoral. Em 5 casos, utilizamos um cateter Pigtail (no caso de restos de cateter) para mobilizar e colocar os pacientes em uma posição mais acessível. Em 8 casos utilizamos um cateter, em 27 casos um cateter de laço (Goose Neck Snare) e em 3 casos utilizamos um biótomo para capturar e recuperar os materiais embolizados. Conseguimos extrair 37 objetos embolizados sem complicações, um deles não pôde ser retirado porque estava firmemente preso ao ápice do ventrículo direito.

Muerte súbita de causa cardíaca en pediatría. Análisis de 95 casos en los últimos 20 años / Sudden death of cardiac etiology in pediatrics. Analysis of 95 cases in the last 20 years / Morte súbita de etiologia cardíaca em pediatria. Análises de 95 casos nos últimos 20 anos

Resumen: Introducción: acerca del tema muerte súbita de origen cardíaco en niños y adolescentes es mucho lo que se describe en la literatura mundial, pero son pocos los datos publicados en nuestro país. Más de la mitad de los casos de muerte súbita en este grupo etario responden a una etiología cardíaca. Objetivo: analizar los eventos de muerte súbita en edad pediátrica a partir de casos de muerte súbita (incluidas las frustras o abortadas) referidos por los colegas intervinientes en su evaluación de manera correlativa, según fueron sucediendo en el tiempo, tratando de investigar en ellos y en sus familiares cercanos la existencia de patologías cardíacas responsables de dicho evento. Evaluar mecanismos de prevención. Resultados: se estudiaron 95 casos, el 68% de sexo masculino, con edades entre 6 meses y 18 años. En el 65% de los casos se encontró una etiología cardíaca responsable. De ellos, en dos tercios se trató de una patología estructural cardíaca y en el tercio restante de una canalopatía o arritmia. Hubo antecedentes familiares de muerte súbita en el 50% de los casos. En el 35% de éstos se encontró una etiología potencialmente causante de muerte súbita. Conclusiones: la muerte súbita en edad pediátrica es un hecho real. Desconocemos su incidencia en nuestro país. La etiología cardíaca tuvo un papel preponderante en los casos estudiados. Por lo tanto, debemos poder detectar las situaciones de riesgo de un evento de muerte súbita previo a su aparición, y, si este ocurriera, tener previstos los mecanismos necesarios para revertirla.

Summary: Introduction: much has been described in the global literature about Sudden Cardiac Death, but few reliable data is available in Uruguay. More than half of the cases of sudden death in this age group arise from a cardiac etiology. Objective: to analyze sudden death in pediatric populations by studying sudden death cases (including frustrated or aborted deaths) referred to by colleagues involved in their assessment, as they happened over time. We studied the existence of cardiac pathologies in patients and their close relatives and evaluated prevention mechanisms. Results: 95 cases were studied, 68% males, aged between 6 months and 18 years. In 65% of the cases, we were able to find a cardiac etiology responsible for the event, in two thirds of the cases it was a structural cardiac pathology, and in the remaining third it was a canalopathy or arrhythmia. There was a family history of sudden death in 50% of cases. Of these cases, 35% showed a potential etiology that caused the sudden death. Conclusions: sudden death in children is a real fact. We do not know its incidence in Uruguay. Cardiac etiology played a significant role in the cases studied. Therefore, we should be able to detect situations of risk of a sudden death event prior to its appearance and if it occurred, we should be able to prevent the necessary mechanisms to reverse it.

Resumo: Introdução: muito tem sido descrito na literatura internacional sobre morte súbita cardíaca, mas poucos dados confiáveis estão disponíveis no Uruguai. Mais da metade dos casos de morte súbita nessa faixa etária vem duma etiologia cardíaca. Objetivo: analisar eventos de morte súbita durante a idade pediátrica, estudando casos de morte súbita (incluindo mortes frustradas ou abortadas) referidos pelos colegas envolvidos em sua avaliação, assim tal como ocorreram ao longo do tempo. Estudamos a existência de patologias cardíacas em pacientes e familiares próximos e avaliamos os mecanismos de prevenção. Resultados: foram estudados 95 casos, 68% do sexo masculino, com idade entre 6 meses e 18 anos. Em 65% dos casos, conseguimos encontrar uma etiologia cardíaca responsável pelo evento, e em dois terços dos casos era uma patologia cardíaca estrutural e no terço restante era uma canalopatia ou arritmia. Em 50% dos casos existia história familiar de morte súbita. Desses casos, 35% mostraram uma etiologia potencial que causou a morte súbita. Conclusões: a morte súbita em crianças é um fato real. Não sabemos sua incidência em Uruguai. A etiologia cardíaca teve um papel importante nos casos estudados. Portanto, devemos ser capazes de detectar situações de risco de morte súbita antes de que ocorra e, se ocorrer, devemos prever os mecanismos necessários para revertê-lo.