Three-Dimensional Echocardiography and Global Longitudinal Strain in Follow-Up After Multisystem Inflammatory Syndrome in Children: Six-Month, Single-Center, Prospective Study.

OBJECTIVE: To assess the potential long-term cardiac effects after multisystem inflammatory syndrome in children (MIS-C) with cardiovascular involvement in the acute phase. STUDY DESIGN: Our prospective study involved children consecutively diagnosed with MIS-C between October 2020 and February 2022 and followed 6 weeks and 6 months after the disease. In patients with severe cardiac involvement during the acute phase, an additional check-up after 3 months was scheduled. In all patients at all check-ups, 3-dimensional echocardiography and global longitudinal strain (GLS) were used to assess ventricular function. RESULTS: The study enrolled 172 children aged 1-17 years (median, 8 years). The means of ejection fraction (EF) and GLS for both ventricles were within normal limits after 6 weeks with no relationship with initial severity: left ventricular EF (LVEF) 60% (59%-63%), LV GLS -21.08% (-18.63% to -23.2%), right ventricular (RV) EF 64% (62%-67%), and RV GLS -22.8% (-20.5% to -24.5%). Further, statistically significant improvement of LV function was observed after 6 months-LVEF 63% (62%-65%) and LV GLS -22.55% (-21.05% to -24.25%; P < .05); however, RV function remained unchanged. The group with severe cardiac involvement showed LV function recovery pattern with no significant improvement between 6 weeks and 3 months after MIS-C, while still improving between 3 and 6 months after discharge. CONCLUSIONS: LV and RV function is within normal limits 6 weeks after MIS-C regardless of severity of cardiovascular involvement; LV function improves further between 6 weeks and 6 months after the disease. The long-term prognosis is optimistic with full recovery of cardiac function.

Assessment of the physical performance in children with asymptomatic pre-excitation.

AIMS: Pre-excitation syndrome can lead to recurrent supraventricular tachycardias (SVTs) and carries a risk of sudden cardiac death (SCD). However, an underestimated consequence of antegrade conduction through an accessory pathway is fusion of intrinsic and accessory conduction that causes asynchronous activation and myocardial contraction that could be a cause for cardiac dysfunction and dilation. It is not known to what extent pre-excitation affects myocardial and physical performance in those patients. The aim of the study was to assess to what degree ventricular pre-excitation affects physical performance in children, using cardio-pulmonary exercise testing (CPET). METHODS AND RESULTS: The study group consisted of 30 asymptomatic children, aged 8-17 years, with pre-excitation and no history or documentation of SVT compared to 31 healthy controls matched according to sex and age. All patients underwent routine cardiology assessment and then CPET. Echocardiography showed there were no differences in the left ventricular size and function between the study and control group. During the CPET both, patients and controls achieved maximal effort. Patients in the study group showed significantly lower values of VO2max and anaerobic threshold when compared to controls. The most affected subgroup was patients with persistent pre-excitation throughout the exercise. CONCLUSIONS: Physical performance is affected in children with pre-excitation. This effect is stronger in patients with persistent delta wave observed throughout the exercise.

Children With Acute Myocarditis Often Have Persistent Subclinical Changes as Revealed by Cardiac Magnetic Resonance.

BACKGROUND: Many children presenting with myocarditis may not fully recover and have long-term complications, including dilated cardiomyopathy. Magnetic resonance imaging (MRI) has a potential for early detection of persistent changes with long-term implications, but is not performed routinely in the monitoring of myocarditis. PURPOSE: To monitor adolescents who present with acute myocarditis using MRI and routine diagnostic tests over the short- to mid-term. STUDY TYPE: Prospective. POPULATION: Eighteen consecutive adolescents (median age 15.5, interquartile range 14.8-16.9 years, 78% male) with acute myocarditis. FIELD STRENGTH: A 3T scanner including cine steady-state free precession (SSFP), dark-blood T2 -weighted (T2 W) images, and late gadolinium enhancement (LGE). ASSESSMENT: The diagnosis of acute myocarditis was based on clinical symptoms and signs and MRI criteria (cine, T2 -W images, LGE). Follow-up MRI was performed after median 7 months (range 6-9 months). Other routine diagnostic tests included electrocardiogram (ECG), high-sensitivity troponin levels, transthoracic echocardiography, and Holter monitoring. STATISTICAL TESTS: Fisher's exact test, Wilcoxon test for paired samples, Mann-Whitney test for independent samples, Kruskal-Wallis test. RESULTS: At baseline, 17 patients (94%) had elevated troponin levels and/or ST-T changes on resting ECG; ECG showed depressed left ventricular ejection fraction (LVEF<50%) in four patients (22%). At follow-up there was a complete recovery in 16 patients (89%) observed with routinely performed tests, with two cases of persistent ventricular arrhythmia. Despite normal left ventricular volume and LVEF, MRI disclosed ongoing active inflammation in five patients (28%), healed myocarditis with persistent scars in eight patients (44%), and complete resolution of initially observed changes in five patients (28%). DATA CONCLUSION: In children with acute myocarditis, despite normalization of other routinely assessed parameters (including LVEF), there is a high prevalence of persistent MRI changes showing ongoing disease or remnant scars at follow-up. MRI may allow early detection and prevention of long-term complications of myocarditis in the follow-up care of children with acute myocarditis. LEVEL OF EVIDENCE: 2 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2020. J. Magn. Reson. Imaging 2020;52:488-496.

Serum Klotho is correlated to cardiovascular complications of chronic kidney disease in children.

AIM: Aim of the study was to investigate soluble Klotho (sKl), fibroblast growth factor 23 (FGF23) concentrations, and their correlations with cardiovascular complications in children with CKD. MATERIALS AND METHODS: 38 children with CKD stages 2 - 5 were compared to 38 healthy controls in terms of: plasma FGF23, serum sKl, peripheral and central blood pressure, arterial stiffness (pulse wave velocity - (PWV)), carotid intima media thickness (cIMT), left ventricular mass index (LVMI), and diastolic function. Correlations between FGF23, sKl, and cardiovascular parameters were investigated. RESULTS: The CKD group was characterized by higher FGF23, lower sKl concentrations, higher peripheral and central blood pressure, arterial stiffness, cIMT, left ventricular mass index, and decreased E/A ratio compared to the control group. In CKD children, sKl correlated negatively with diastolic blood pressure (DBP), mean arterial pressure (MAP), central systolic, diastolic, and mean blood pressure, PWV, and LVMI. In multivariate analysis, higher sKl was a significant predictor of lower peripheral and central DBP and lower LVMI and E/A, whereas higher FGF23 was a predictor of higher of LVMI. CONCLUSION: (1) In children with CKD, decreased sKl might be a marker of elevated central blood pressure. (2) Both sKl decrease and FGF23 increase could possibly contribute to left ventricular hypertrophy in this group of patients.

[Pulmonary embolism in a girl with nephrotic syndrome and factor V Leiden - case report]. / Zatorowosc plucna u dziewczynki z zespolem nerczycowym i czynnikiem V Leiden ­ opis przypadku.

Thromboembolic complications are found in 2-3% of children with nephrotic syndrome (NS); this increased risk is caused by hypovolemia, hemoconcentration, increased number and activity of platelets, hyperfibrinogenemia and loss of coagulation inhibitors. Risk is even higher in case of additional factors e.g. congenital thrombophilia. CASE REPORT: Girl with NS aged 17 11/12 years was admitted to hospital due to respiratory tract infection with cough and back pain. NS started 9 months earlier and she had two bouts of disease, and was treated only with prednisone (current dose - 60 mg/48h). On admission she was without any abnormalities on auscultation, with BP 111/65 mmHg, HR 80 bpm, satO2 99%. Lab results showed the increase of WBC 18.3×103/µL, D-dimers 23038 µg/L and proteinuria 900 mg/dL. Other values of examined parameters were in normal limits. Chest X-ray and ECG were also normal. Presumptive diagnosis of pulmonary embolism was made and the patient was given 1000IU of antithrombin III and nadroparine (2x90IU/kg/24h s.c.). In ECHO the occlusion of left pulmonary artery and preserved blood flow in right were revealed. In angioCT clot nearly filling lumen of left pulmonary artery, clot in intermediate part of right pulmonary artery, and focus of pulmonary infarction in 10th segment of left lung were found. Doppler USG of lower limb veins did not reveal thrombi or perforator vein incompetence. Treatment with nadroparine was continued, and rapid improvement of clinical condition and disappearance of pain and cough were observed. Mycophenolate mofetil was added, which resulted in subsidence of proteinuria. Rivaroxaban was used in prophylaxis of recurrences of thromboembolism. Tests for thrombophilia revealed factor V Leiden in patient.

SETD5 loss-of-function mutation as a likely cause of a familial syndromic intellectual disability with variable phenotypic expression.

Loss-of-function de novo mutations in the SETD5 gene, encoding a putative methyltransferase, are an important cause of moderate/severe intellectual disability as evidenced by the results of sequencing large patient cohorts. We present the first familial case of a SETD5 mutation contributing to a phenotype of congenital heart defects and dysmorphic features, with variable expression, in two siblings and their father. Interestingly, the father demonstrated only mild intellectual impairment. Family based exome sequencing combined to careful parental phenotyping may reveal a more complex clinical picture in newly recognized syndromes. © 2016 Wiley Periodicals, Inc.

Evidence for troponin C (TNNC1) as a gene for autosomal recessive restrictive cardiomyopathy with fatal outcome in infancy.

Restrictive cardiomyopathy is a rare form of pediatric cardiac disease, for which the known genes include MYH7, TNNT2, TNNI3, ACTC1, and DES. We describe a pediatric proband with fatal restrictive cardiomyopathy associated with septal hypertrophy and compound heterozygosity for TNNC1 mutations (NM_003280: p.A8V [c.C23T] and p.D145E [c.C435A]). This association between restrictive cardiomyopathy and TNNC1 mutations was strengthened by prospective observations on the second pregnancy in the family which revealed, in the presence of the same TNNC1 genotype, prenatally diagnosed hypertrophic cardiomyopathy which evolved into restrictive cardiomyopathy, heart failure and death at the age of 9 months. Contrary to previous reports, family and population analyses showed that each of the TNNC1 variants was not pathogenic when present alone. Our results (i) confirm that genetic backgrounds of hypertrophic cardiomyopathy and restrictive cardiomyopathy overlap and (ii) indicate that TNNC1 is a likely novel gene for autosomal recessive restrictive cardiomyopathy. © 2016 Wiley Periodicals, Inc.

Ceftriaxone-associated acute gallbladder enlargement - an unexpected diagnosis in the child with urinary tract infection.

UNLABELLED: Biliary sludge and/or biliary pseudolithiasis occur in patients treated with ceftriaxone with prevalence of 3-57%. Biliary obstruction can be the cause of the acute gallbladder enlargement. It is a minor complication, that usually does not give clinical symptoms and resolves once the drug is discontinued. The authors present a case of a 5-month old boy treated for the acute pyelonephritis. Routine ultrasound, performed on the 5th day of treatment with ceftriaxone, showed gallbladder enlargement. In the consecutive studies small gallblader sludge was visible. Patient had no symptoms related to the gallbladder enlargement. Ultrasound performed 6 weeks from the drug discontinuation was completely normal. CONCLUSIONS: Patients treated with ceftroiaxone should be monitored for biliary sludge and pseudolithiasis.

Heart rate dynamics and asymmetry during sympathetic activity stimulation and post-stimulation recovery in ski mountaineers-a pilot exploratory study.

There is a lack of studies on non-linear heart rate (HR) variability in athletes. We aimed to assess the usefulness of short-term HR dynamics and asymmetry parameters to evaluate the neural modulation of cardiac activity based on non-stationary RR interval series by studying their changes during sympathetic nervous system activity stimulation (isometric handgrip test) and post-stimulation recovery in professional ski mountaineers. The correlation between the changes in the parameters and the respiratory rate (RespRate) and also the duration of the career was analyzed. Short-term (5 min) and ultra-short-term (1 min) rates of patterns with no variations (0V), number of acceleration runs of length 1 (AR1), and short-term Porta's Index were greater, whereas Guzik's Index (GI) was smaller during sympathetic stimulation compared to rest. GI increased and the number of AR1 decreased during recovery. Greater increases in GI and RMSSD were associated with greater decreases in RespRate during recovery. Greater increases in RespRate from rest to short-term sympathetic stimulation were associated with greater increases in 0V (Max-min method) and AR1 but also with greater decreases in decelerations of short-term variance and accelerations and decelerations of long-term variance. Greater increases in 0V (Max-min method) and number of AR1 during sympathetic stimulation were associated with a shorter career duration. Greater decreases in these parameters during recovery were associated with a longer career duration. Changes in measures of HR dynamics and asymmetry, calculated based on short-term non-stationary RRi time series induced by sympathetic stimulation and post-stimulation recovery, reflected sympathovagal shift and were associated with condition-related alterations in RespRate and career duration in athletes who practice ski mountaineering.

A MYH7 variant in a five-generation-family with hypertrophic cardiomyopathy.

Background: Hypertrophic cardiomyopathy (HCM) is a genetic condition with a prevalence of 1:500-1:3 000. Variants in genes encoding sarcomeric proteins are mainly responsible for the disease. MYH7 gene encoding a myosin heavy chain beta, together with MYPBC3 gene are the two most commonly affected genes. The clinical presentation of this disease varies widely between individuals. This study aims to report a variant of MYH7 responsible for HCM in a five-generation family with a history of cardiac problems. Methods: The diagnosis was established according to the European Society of Cardiology HCM criteria based on two-dimensional Doppler echocardiography or cardiovascular magnetic resonance. Genetic analysis was performed using next-generation-sequencing and Sanger method. Results: The medical history of the presented family began with a prenatal diagnosis of HCM in the first child of a family with previously healthy parents. Five generations of the family had a long history of sudden cardiac death and cardiac problems. A NM_000257.4:c.2342T>A (p.Leu781Gln) variant was detected in the MYH7 gene. It was heterozygous in the proband and in all affected individuals in a large family. The variant was present in 10 affected members of the family, and was absent in 7 members. The clinical course of the disease was severe in several members of the family: three family members died of sudden cardiac death, one patient required heart transplantation, three underwent septal myectomy, and three required implantable cardioverter defibrillator (ICD) implantation. Conclusion: Herein, we report a MYH7 variant responsible for HCM. Familial HCM is inherited primarily in autosomal dominant mode, which is in accordance with our study. However, the presented family showed a broad clinical spectrum of HCM. Out of 10 family members with positive genetic testing 8 had severe presentation of the disease and 2 had a mild phenotype. This suggests that the severity of the disease may depend on other factors, most likely genetic.

Left ventricular noncompaction (LVNC) and low mitochondrial membrane potential are specific for Barth syndrome.

Barth syndrome (BTHS) is an X-linked mitochondrial defect characterised by dilated cardiomyopathy, neutropaenia and 3-methylglutaconic aciduria (3-MGCA). We report on two affected brothers with c.646G > A (p.G216R) TAZ gene mutations. The pathogenicity of the mutation, as indicated by the structure-based functional analyses, was further confirmed by abnormal monolysocardiolipin/cardiolipin ratio in dry blood spots of the patients as well as the occurrence of this mutation in another reported BTHS proband. In both brothers, 2D-echocardiography revealed some features of left ventricular noncompaction (LVNC) despite marked differences in the course of the disease; the eldest child presented with isolated cardiomyopathy from late infancy, whereas the youngest showed severe lactic acidosis without 3-MGCA during the neonatal period. An examination of the patients' fibroblast cultures revealed that extremely low mitochondrial membrane potentials (mtΔΨ about 50 % of the control value) dominated other unspecific mitochondrial changes detected (respiratory chain dysfunction, abnormal ROS production and depressed antioxidant defense). 1) Our studies confirm generalised mitochondrial dysfunction in the skeletal muscle and the fibroblasts of BTHS patients, especially a severe impairment in the mtΔΨ and the inhibition of complex V activity. It can be hypothesised that impaired mtΔΨ and mitochondrial ATP synthase activity may contribute to episodes of cardiac arrhythmia that occurred unexpectedly in BTHS patients. 2) Severe lactic acidosis without 3-methylglutaconic aciduria in male neonates as well as an asymptomatic mild left ventricular noncompaction may characterise the ranges of natural history of Barth syndrome.

Diastolic function evaluation in children with ventricular arrhythmia.

Premature ventricular contractions (PVC) are frequently seen in children. We evaluated left ventricular diastolic function in PVC children with normal left ventricular systolic function to detect whether diastolic function disturbances affect physical performance. The study group consisted of 36 PVC children, and the control group comprised 33 healthy volunteers. Echocardiographic diastolic function parameters such as left atrial volume index (LAVI), left atrial strains (AC-R, AC-CT, AC-CD), E wave, E deceleration time (Edt), E/E' ratio, and isovolumic relaxation time (IVRT) were measured. In the cardiopulmonary exercise test (CPET), oxygen uptake (VO2 max) was registered. Evaluation of diastolic function parameters revealed statically significant differences between the patients and controls regarding Edt (176.58 ± 54.8 ms vs. 136.94 ± 27.8 ms, p < 0.01), E/E' (12.6 ± 3.0 vs. 6.7 ± 1.0, p < 0.01), and IVRT (96.6 ± 19.09 ms. vs. 72.86 ± 13.67 ms, p < 0.01). Left atrial function was impaired in the study group compared to controls: LAVI (25.3 ± 8.2 ml/m2 vs. 19.2 ± 7.5 ml/m2, p < 0.01), AC-CT (34.8 ± 8.6% vs. 44.8 ± 11.8%, p < 0.01), and AC-R-(6.0 ± 4.9% vs. -11.5 ± 3.5%, p < 0.01), respectively. VO2 max in the study group reached 33.1 ± 6.2 ml/min/kg. A statistically significant, moderate, negative correlation between VO2 max and E/E' (r = -0.33, p = 0.02) was found. Left ventricular diastolic function is impaired and deteriorates with the arrhythmia burden increase in PVC children. Ventricular arrhythmia in young individuals may be related to the filling pressure elevation and drive to exercise capacity deterioration.

Cardiopulmonary capacity is reduced in children with ventricular arrhythmia.

BACKGROUND: Premature ventricular contractions (PVCs) are frequently seen in children and are considered benign. A substantial group of adolescents with PVCs complain about a broad range of clinical symptoms, including low exertion tolerance. OBJECTIVE: The purpose of this study was to evaluate prospectively whether ventricular arrhythmia affects physical performance in adolescents with normal left ventricular function, using a cardiopulmonary exercise test (CPET) and evaluating the electrocardiographic (ECG) characteristics of patients with PVCs with regard to exercise capacity. METHODS: The study group consisted of 49 children with PVCs and normal left ventricular function. The control group consisted of 36 healthy volunteers. Standard ECG, 24-hour Holter ECG, and CPET were performed. PVCs were analyzed for QRS duration, bundle branch block pattern, QRS axis, and coupling interval (CInt). For CPET, heart rate (HR), oxygen uptake (VO2max), predicted VO2max, and VO2max expressed as a percentage of the predicted value (%VO2) were measured. RESULTS: In 37 patients (76%), arrhythmia subsided during exercise. Patients achieved lower VO2max (32.9 ± 6.3 mL/min/kg) than controls (40.4 ± 6.7 mL/min/kg; P <.01). %VO2 was 71.0 ± 13.7 in patients and 79.3 ± 12.2 in controls (P <.01). Exercise HR at which PVCs subsided correlated with VO2max (r = 0.3; P = .07). Patients with persisting arrhythmia performed worse than those in whom arrhythmia subsided during exercise (VO2max, P <.01; %VO2,P <.01). No correlation between QRS and CInt parameters and VO2max was observed. CONCLUSION: Patients with PVCs have lower aerobic capacity than their healthy peers. Further worsening of exercise capacity is present when PVCs are preserved during effort.

Inter-Rater Reliability of the Polish Version of the Alberta Infant Motor Scale in Children with Heart Disease.

There is an urgent need for the systematic monitoring of motor and cognitive neurodevelopment and the evaluation of motor skill development in infants and children with heart disease. Familiarizing students and early graduates with the developmental care needed by these patients may help in the system-wide implementation of early motor screening in this population. The purpose of this study was to investigate the agreement between a last-year physiotherapy student and an experienced pediatric physiotherapist when applying the Polish version of the Alberta Infant Motor Scale (AIMS) to a heterogenous group of children with congenital heart defects. Agreement between raters was verified based on the observation of 80 (38 females) patients with heart disease aged 1-18 months using a Bland-Altman plot with limits of agreement and an intraclass correlation coefficient. The bias between raters for the total score for four age groups (0-3 months, 4-7 months, 8-11 months and 12-18 months) was between -0.17 and 0.22 (range: -0.54-0.78), and the ICC was between 0.875 and 1.000. Thus, a reliable assessment of motor development or motor skills using the Polish version of the AIMS can be performed in pediatric patients with heart defects by clinically inexperienced last-year physiotherapy students who are familiarized with the AIMS manual.

Validity of the Pneumonitor for RR intervals acquisition for short-term heart rate variability analysis extended with respiratory data in pediatric cardiac patients.

BACKGROUND: Breathing pattern alterations change the variability and spectral content of the RR intervals (RRi) on electrocardiogram (ECG). However, there is no method to record and control participants' breathing without influencing its natural rate and depth in heart rate variability (HRV) studies. AIM: This study aimed to assess the validity of the Pneumonitor for acquisition of short-term (5 minutes) RRi in comparison to the reference ECG method for analysis of heart rate (HR) and HRV parameters in the group of pediatric patients with cardiac disease. METHODS: Nineteen patients of both sexes participated in the study. An ECG and Pneumonitor were used to record RRi in 5-minute static rest conditions, the latter also to measure the relative tidal volume and respiratory rate. The validation comprised Student's t-test, Bland-Altman analysis, intraclass correlation coefficient, and Lin's concordance correlation. The possible impact of respiratory activity on the agreement between ECG and the Pneumonitor was also assessed. RESULTS: An acceptable agreement for the number of RRi, mean RR, hazard ratio (HR), and HRV measures calculated based on RRi acquired using the ECG and Pneumonitor was presented. There was no association between the breathing pattern and RRi agreement between devices. CONCLUSIONS: The Pneumonitor might be considered appropriate for cardiorespiratory studies in the group of pediatric cardiac patients in rest condition.

The discovery of a data-driven causal diagram of sport participation in children and adolescents with heart disease: a pilot study.

The interventions aimed at improving the levels of physical activity (PA) in children and adolescents diagnosed with heart disease did not produce the expected outcomes. Safe participation in sport activities proposed based on actual recommendations could be a solution to promote PA in this population. The aims of this study were to discover a causal diagram of sport participation in children and youth with heart disease and establish the factors that affect and are affected thereof through the use of questionnaires. Furthermore, the study aims to qualitatively assess the reliability of the constructed diagram in comparison with existing medical knowledge. The Greedy Fast Causal Inference method was employed to conduct a data-driven search of the directed acyclic graph that represents the causal relationships within the provided observational data. This causal discovery was performed using the Tetrad software. The analysis involved a cohort of 121 Caucasian patients (50 females) diagnosed with heart disease. The age range of the patients included in the study was 8-17 years. The study findings indicate that the participants engaged in sports presented significantly higher values of health-related quality of life (QoL) and motives for participating in physical and leisure activities. Age appears to be a cause of sport participation. Sport participation appears to be a cause of participation in physical education classes, which in turn appears to be a cause of higher enjoyment. Higher enjoyment appears to be a cause of other motives for participating in physical and leisure activities, as well as a higher score in terms of physical health. The causal diagram provided a graphical representation of the causal relationship between sport participation and better QoL with potential confounders for children and adolescents with heart disease that nearly coincided with the existing literature. Clinical trials should be designed to validate clinical utility of the presented causal diagram.

Heart Rate Variability in Individuals with Down Syndrome: A Scoping Review with Methodological Considerations.

Individuals with Down syndrome (DS) present similar heart rate variability (HRV) parameters at rest but different responses to selected movement maneuvers in comparison to individuals without DS, which indicates reduced vagal regulation. The present study undertakes a scoping review of research on HRV in individuals with DS, with special attention paid to the compliance of the studies with standards and methodological paper guidelines for HRV assessment and interpretation. A review was performed using PubMed, Web of Science and CINAHL databases to search for English language publications from 1996 to 2020 with the MESH terms "heart rate variability" and "down syndrome", with the additional inclusion criteria of including only human participants and empirical investigations. From 74 studies, 15 were included in the review. None of the reviewed studies met the recommendations laid out by the standards and guidelines for providing the acquisition of RR intervals and necessary details on HRV analysis. Since authors publishing papers on this research topic do not adhere to the prescribed standards and guidelines when constructing the methodology, results of the research papers on the topic are not directly comparable. Authors need to design the study methodology more robustly by following the aforementioned standards, guidelines and recommendations.

Prediction of the hypertension risk in teenagers.

BACKGROUND: Creation of a hypertension risk stratification model and development of an algorithm to detect hypertension in teenagers. METHODS: The study group consisted of 690 middle and high school students, aged 15-17 years, from the metropolitan area of Warsaw, Poland. Information concerning family history and presence of risk factors for cardiovascular disease was gathered. Three-time blood pressure measurements were taken during at least two separate visits, which were at least a week apart, using the auscultatory method, according to standard procedures. Anthropometric measurements included: body weight, height, arm, hip and abdominal circumference, skin-fold thickness measured on the rear surface of an arm, below the inferior angle of the scapula and at the belly. Following indexes were determined: body mass index, waist to hip ratio (WHR), waist to height ratio, hip to height ratio. RESULTS: A logistic regression model, describing the risk of hypertension in adolescents aged 15-17 was invented. p(x) = (e^g(x))/(1+e^g(x)) where g(x) = -0.097 × height+0.085 × weight+7.764 × WHR+1.312 × family hypertension. Family hypertension means presence of hypertension among members of the closest family. The formula was created, allowing the pre-selection of adolescents at risk of hypertension during screening. Next an algorithm for the detection of hypertension for practical use was proposed. CONCLUSIONS: Body weight, WHR and incidence of hypertension in the family are the strongest predictors of hypertension in teenagers. Proposed screening algorithm can be a useful tool for selecting teenagers at risk of hypertension and in need of specialized diagnostics and care.

Electrocardiographic Parameters Associated with Adverse Outcomes in Children with Cardiomyopathies.

Cardiomyopathies have a low prevalence in children and thus may lead to malignant ventricular arrhythmias or the progression of heart failure, resulting in death. In adults, the QRS-T angle derived from ECG has been associated with adverse outcomes in patients with hypertrophic and dilated cardiomyopathies. We aimed to assess the electrocardiographic parameters, including QRS-T angle, associated with adverse cardiac events in children with cardiomyopathies. Forty-two children with cardiomyopathies were included in this study: 19 with dilated cardiomyopathy, 17 with hypertrophic cardiomyopathy, and 6 with left ventricular non-compaction. Additionally, 19 control subjects were recruited. In terms of ECG parameters, the QRS-T angle was significantly greater among patients with adverse outcomes compared to patients without the end points of the study (133° vs. 65°, p < 0.001). On Kaplan−Meier survival curves, QRS-T angle > 120°, increased serum concentrations of NT-proBNP and troponin I levels as well as greater NYHA or Ross scale were associated with the greatest risk of unfavorable outcome. The QRS-T angle appears to be a valuable component of 12-lead ECG interpretation, and might be helpful in outlining patients with the greatest cardiovascular risk. Additionally, serum biomarkers such as NT-proBNP (p = 0.003) and troponin (p < 0.001) are useful in outlining patients with the worst survival.

Image quality and radiation doses of volumetric 320-row computed tomography angiography with prospective electrocardiogram-gating in the assessment of coronary arteries in neonates and infants.

BACKGROUND: Diagnostic imaging of coronary arteries is required in neonates and infants suspected of congenital or acquired coronary artery anomalies and in pre- and postoperative assessment of complex congenital heart diseases (CHD). AIM: Our study aimed to evaluate the image quality of volumetric 320-row computed tomography angiography (CTA) with prospective electrocardiogram (ECG)-gating for coronary arteries in neonates and infants with heart diseases, analyze factors influencing image quality and assess a radiation dose related to the procedure. METHODS: The study included 110 CTA performed in neonates and infants with CHD. RESULTS: CTA was performed in 37 girls and 73 boys at a median (interquartile range [IQR]) age of 3.0 (0.5-5.0) months, median (IQR) body weight of 5 (3.66-6.5) kg, and median heart rate (HR) of 133 (92-150) beats per minute. The orifices of the left coronary artery were visible in 100% of CTA, the orifices of the right coronary arteries were visible in 96%, whereas all coronary segments were assessable in 45% of CTA. Patients with non-diagnostic segments were significantly younger, median (IQR) age of 2.0 (0.21-5.00) months, had lower body weight of 4.6 (3.45-6.07) kg and faster HR of 136.5 (120-150) beats per minute (P <0.05) than patients with diagnostic image quality in all segments (4.0, 2-6 months, 6.0, 4.2-7 kg, and 130; 110-150 beats per minute, respectively; P <0.05). CONCLUSIONS: CTA performed with volumetric 320-row prospective ECG-gating allows for good visibility of the coronary arteries with an acceptable radiation dose. Children aged >15 days, with body weight >4.85 kg and HR <130 beats per minute are good candidates for excellent quality non-invasive CTA of all segments of coronary arteries.