Longitudinal systolic left ventricular function in preterm and term neonates: reference values of the mitral annular plane systolic excursion (MAPSE) and calculation of z-scores.

The mitral annular plane systolic excursion (MAPSE) is a quick and reliable echocardiographic tool for assessing longitudinal left ventricular (LV) systolic function in children and adults. Because this parameter is affected by the LV longitudinal dimension, pediatric and adult normal values are not suitable for preterm and term neonates. A prospective study investigated a large group of preterm and term neonates [gestational age (GA), 26/0-6 to 40/0-6; birth weight (BW), 670-4,140 g]. The growth- and BW-related changes in MAPSE were determined to establish normal z-score values for preterm and term neonates. The MAPSE ranged from a mean of 0.36 ± 0.05 cm in preterm neonates with a GA of 26/0-6 to 0.56 ± 0.08 cm in term neonates with a GA of 40/0-6. The findings showed MAPSE, GA, and BW to be moderately correlated. Pearson's correlation coefficient was 0.56 for GA (MAPSE; p < 0.001) and 0.58 for BW (MAPSE; p < 0.001). The normal MAPSE values did not differ significantly between females and males (p = 0.946). The absolute values and z-scores of normal MAPSE values in healthy preterm and term neonates within the first 48 h of life were calculated, and percentile charts were established. Determination of LV function using MAPSE might be useful for vulnerable infants for whom a prolonged examination is inappropriate and for neonates with suboptimal visualization of the endocardium.

Reference values of the mitral annular peak systolic velocity (Sm) in 690 healthy pediatric patients, calculation of Z-score values, and comparison to the mitral annular Plane systolic excursion (MAPSE).

BACKGROUND: The mitral annular peak systolic velocity (Sm) is an echocardiographic measurement using tissue Doppler imaging to assess longitudinal left ventricular (LV) systolic function in children and adults. We determined growth-related changes in Sm to establish reference values for the entire pediatric age group. METHODS AND RESULTS: A prospective study was conducted in a group of 690 healthy pediatric patients (age: 1 day-18 years). We determined the effects of age, sex, and body surface area (BSA) on the Sm values. Regression analysis was used to estimate Sm from age, BSA, and sex. In addition, a correlation of normal Sm with normal age-matched values of the M-mode parameter mitral annular plane systolic excursion (MAPSE) was measured. The Sm ranged from a mean of 5.8 cm/sec (Z-score ±2: 3.6-8.0 cm/sec) in the newborn to 11.8 cm/sec (Z-score ±2: 8.5-15.1 cm/sec) in the 18-year-old adolescent. The Sm values showed a positive correlation with age and BSA with a nonlinear course. There was no significant difference in Sm values between females and males. A significant correlation was found between Sm and MAPSE values. CONCLUSION: Z-scores of Sm values were calculated and percentile charts were established to serve as reference data in patients with congenital heart diseases.

Reference values and calculation of z-scores of echocardiographic measurements of the normal pediatric right ventricle.

Determination of right ventricular (RV) size and function has gained more interest in recent years in adults and children, especially in patients with congenital heart disease. Data on normal RV size parameters in children are scant. The aim of this study was to investigate growth-related changes in RV internal dimensions in a healthy pediatric cohort and the predictive value of RV parameters in identifying enlarged right ventricles in children with secundum-type atrial septal defects (ASD). A prospective study was conducted in a group of 576 healthy children (aged 1 day to 18 years) and 37 children (aged 1.4 to 17.7 years) with moderate-sized to large ASDs. The effects of age, body length, body weight, and body surface area were determined on the following RV parameters: end-diastolic basal diameter, end-diastolic midcavity diameter, end-diastolic length, end-systolic length, end-diastolic area, and end-systolic area. The predictive value of normal values stratified for age, body weight, body length, and body surface area was tested in children with ASDs. RV end-diastolic basal diameter, end-diastolic midcavity diameter, end-diastolic length, end-systolic length, end-diastolic area, and end-systolic area showed positive correlations with age, body length, body surface area, and body weight. In this population, RV z scores showed high specificity for detecting patients with ASDs, with sensitivity up to 89%, especially in children <8 years of age. In conclusion, the normal ranges of pediatric RV internal dimensions are provided. The z scores of these RV parameters were also calculated. Normal RV z scores might be important predictors in identifying enlarged right ventricles in patients with ASDs.

Reference values of the right ventricular outflow tract systolic excursion in 711 healthy children and calculation of z-score values.

OBJECTIVE: Quantitative determination of right ventricular (RV) function has gained more interest over the last years. The RV outflow tract systolic excursion (RVOT SE) has been recently proposed as an echocardiographic tool to assess RV systolic function in adults. We aimed to determine growth-related changes of RVOT SE in children and to establish references values. STUDY DESIGN: A prospective study was conducted in a group of 711 healthy paediatric patients (age: 1 day to 18 years). We determined the effects of age and body surface area (BSA) on RVOT SE values. RVOT SE values were further correlated with the established RV systolic function parameters tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular peak systolic velocity (S'). RESULTS: The RVOT SE ranged from a mean of 3.4 mm in neonates to 9.5 mm in 18-year-old adolescents. The RVOT SE values showed a positive correlation with age (r = 0.90, P < 0.001) and BSA (r = 0.91, P < 0.001). A significant positive correlation was seen between RVOT SE and TAPSE (r = 0.93, P < 0.001) and also between RVOT SE and S' (r = 0.86, P < 0.001) in our patients. CONCLUSION: RVOT SE provides a simple measure and, in combination with long-axis excursion parameters TAPSE and S', a comprehensive assessment of RV systolic function. Z-scores of RVOT SE values were calculated, and percentile charts were established to serve as reference data.

Remote monitoring leads to early recognition and treatment of critical arrhythmias in adults after atrial switch operation for transposition of the great arteries.

BACKGROUND: Adults with transposition of the great arteries (TGA) after atrial switch repair have an increased risk for arrhythmia and sudden cardiac death. We analyzed whether a remote monitoring (RM) system as part of an implantable cardiac device contributes to timely recognition and improved treatment of critical arrhythmias in these patients. METHODS AND RESULTS: All consecutive TGA patients (n=11) requiring a pacemaker or cardiac resynchronization therapy with or without implantable cardioverter defibrillator between 2008 and 2011 were included. RM-detected arrhythmia, abnormality of device integrity and reaction time from event transmission until acknowledgement via email and clinical decision making were analyzed and compared to a control group (n=21). In 10 patients (91%) 17 arrhythmias were detected, 8 patients (80%) indicated no symptoms. In the RM group time interval from transmission to acknowledgement was 2.4 days (range, 0-4.5 days). Clinical decision-making was advanced by a mean of 77.5 days (range, 10-197 days) compared with conventional follow-up and identified adaption of anti-arrhythmic medication in 8, electrical cardioversion in 2, overdrive pacing in 1 and radiofrequency ablation in 2 patients. A coronary sinus lead fracture was identified in 1 patient followed by successful replacement. CONCLUSIONS: RM enables early detection of tachyarrhythmia followed by optimization of medical treatment and potentially life-saving anti-tachycardic intervention in adults after atrial repair of TGA.

Longitudinal systolic ventricular interaction in pediatric and young adult patients with TOF: a cardiac magnetic resonance and M-mode echocardiographic study.

Aim of this prospective study was to evaluate longitudinal systolic left ventricular (LV)-right ventricular (RV) interaction using M-mode compared to magnetic resonance imaging (MRI) data in 146 pediatric and adults with operated tetralogy of Fallot (TOF). We determined biventricular measures of longitudinal M-mode echocardiography [i.e., tricuspid annular plane systolic excursion (TAPSE); the mitral annular plane systolic excursion (MAPSE)] compared to longitudinal function parameters using MRI. M-mode data were compared to established normal z-score values. We found a good correlation between MAPSE and LVEF values (r = 0.788; p < 0.001). Correlations between MRI derived MAPSE and M-mode guided MAPSE (r = 0.879, p < 0.001), and between MRI derived TAPSE and M-mode guided TAPSE were significant (r = 0.780, p < 0.001). While the LVEF was normal in patients with a normal RVEF, the LVEF was decreased in patients with significantly reduced RVEF. Patients with a significantly dilated RV (RVEDVi > 150 ml/m(2)) showed a significantly reduced mean MAPSE of 1.30 ± 0.26 cm. LV longitudinal function decreases below -2 SD of normal MAPSE z-score values after a mean of 22 postoperative years. Our data confirm progressive adverse RV-LV interaction in the long-term follow-up of TOF. We show that simple M-mode measurement of the longitudinal LV function (i.e. MAPSE) is a sufficient surrogate for estimation of LVEF. Therefore determination of the MAPSE is a helpful additional tool for LV systolic function assessment late after TOF repair.

Right ventricular performance in preterm and term neonates: reference values of the tricuspid annular peak systolic velocity measured by tissue Doppler imaging.

BACKGROUND: The tricuspid annular peak systolic velocity (S') is an echocardiographic measurement to assess systolic right ventricular function in adults and children. OBJECTIVE: We determined growth and birth weight-related changes of S' to establish reference values in preterm and term neonates. METHODS: A prospective study was conducted in a group of 290 preterm and term neonates (gestational age 26 + 0 to 40 + 6 weeks of gestation; birth weight 660-4,460 g). A correlation of S' values with established tricuspid annular plane systolic excursion (TAPSE) values was performed. RESULTS: The S' ranged from a mean of 4.5 cm/s (Z-score ± 2 SD: 3.6-5.5 cm/s) in preterm neonates in week 26(0-6) of gestation to 7.8 cm/s (Z-score ± 2 SD: 5.5-10.1 cm/s) in term neonates in week 40(0-6) of gestation. The S' values increased from 26 to 40 weeks of gestation. S' and week of gestation are strongly correlated: Pearson's correlation coefficient was 0.66 for week of gestation - S' (p < 0.001). A significant correlation was found between S' and TAPSE values (r = 0.67; p < 0.001). CONCLUSION: Z-scores of S' values were calculated and percentile charts were established to serve as reference data for preterm and term neonates with structurally normal hearts.

Permanent cardiac pacing in children: choosing the optimal pacing site: a multicenter study.

BACKGROUND: We evaluated the effects of the site of ventricular pacing on left ventricular (LV) synchrony and function in children requiring permanent pacing. METHODS AND RESULTS: One hundred seventy-eight children (aged <18 years) from 21 centers with atrioventricular block and a structurally normal heart undergoing permanent pacing were studied cross-sectionally. Median age at evaluation was 11.2 (interquartile range, 6.3-15.0) years. Median pacing duration was 5.4 (interquartile range, 3.1-8.8) years. Pacing sites were the free wall of the right ventricular (RV) outflow tract (n=8), lateral RV (n=44), RV apex (n=61), RV septum (n=29), LV apex (n=12), LV midlateral wall (n=17), and LV base (n=7). LV synchrony, pump function, and contraction efficiency were significantly affected by pacing site and were superior in children paced at the LV apex/LV midlateral wall. LV dyssynchrony correlated inversely with LV ejection fraction (R=0.80, P=0.031). Pacing from the RV outflow tract/lateral RV predicted significantly decreased LV function (LV ejection fraction <45%; odds ratio, 10.72; confidence interval, 2.07-55.60; P=0.005), whereas LV apex/LV midlateral wall pacing was associated with preserved LV function (LV ejection fraction ≥55%; odds ratio, 8.26; confidence interval, 1.46-47.62; P=0.018). Presence of maternal autoantibodies, gender, age at implantation, duration of pacing, DDD mode, and QRS duration had no significant impact on LV ejection fraction. CONCLUSIONS: The site of ventricular pacing has a major impact on LV mechanical synchrony, efficiency, and pump function in children who require lifelong pacing. Of the sites studied, LV apex/LV midlateral wall pacing has the greatest potential to prevent pacing-induced reduction of cardiac pump function.

Unique doubly connected left ventricular diverticulum.

This case report describes a unique form of a left ventricular diverticulum in a 17-year-old patient.Echocardiography, angiography, and magnetic resonance imaging including virtual endoscopy complete a detailed picture of the size and texture of a diverticulum, as well as the localisation of the two connections into the left ventricle.

Left ventricular hypertrabeculation/noncompaction with epilepsy, other heart defects, minor facial anomalies and new copy number variants.

BACKGROUND: Left ventricular hypertrabeculation/noncompaction (LVHT) is a cardiac abnormality of unknown etiology which has been described in children as well as in adults with and without chromosomal aberrations. LVHT has been reported in association with various cardiac and extracardiac abnormalities like epilepsy and facial dysmorphism. CASE PRESENTATION: A unique combination of LVHT, atrial septal defect, pulmonary valve stenosis, aortic stenosis, epilepsy and minor facial anomalies is presented in a 5.5 years old girl. Microarray-based genomic hybridization (array-CGH) detected six previously not described copy number variants (CNVs) inherited from a clinically unaffected father and minimally affected mother, thus, most likely, not clinically significant but rare benign variants. CONCLUSIONS: Despite this complex phenotype de novo microdeletions or microduplications were not detected by array CGH. Further investigations, such as whole exome sequencing, could reveal point mutations and small indels as the possible cause.

Left ventricular long-axis function: reference values of the mitral annular plane systolic excursion in 558 healthy children and calculation of z-score values.

BACKGROUND: Longitudinal myocardial function has gained more interest in the last years. The mitral annular plane systolic excursion (MAPSE) is an echocardiographic measurement to assess left ventricular (LV) long-axis function in adults. The aim of this study was to evaluate MAPSE values in a healthy pediatric population and to propose reference values. METHODS: A prospective study was conducted in a group of 558 healthy children and adolescents (age day 1 to 18 years) (body surface area [BSA] 0.18-2.21 m(2)). We determined the effects of age and BSA on MAPSE values and a possible correlation of MAPSE values with LV ejection fraction values. RESULTS: The MAPSE ranged from a mean of 0.57 cm (z-score ±2: 0.38-0.76 cm) in neonates to 1.63 cm (z-score ±2: 1.31-1.95 cm) in 18-year-old adolescents. The MAPSE values showed a positive correlation with age (r = 0.87, P < .001) and BSA (r = 0.89, P < .001) with a nonlinear course. There was no significant difference in MAPSE values between females or males. A positive correlation was found between MAPSE values and LV ejection fraction values (r = 0.28, P < .001). CONCLUSIONS: Z-scores of MAPSE values were calculated, and percentile charts were established to serve as reference data in patients with congenital heart disease or heart failure in the future.

Tricuspid annular peak systolic velocity (S') in children and young adults with pulmonary artery hypertension secondary to congenital heart diseases, and in those with repaired tetralogy of Fallot: echocardiography and MRI data.

BACKGROUND: Tricuspid annular peak systolic velocity (S'), as an echocardiographic index to assess right ventricular (RV) systolic function, has not been investigated thoroughly in children and young adults with repaired tetralogy of Fallot (TOF) and pulmonary artery hypertension secondary to congenital heart disease (PAH-CHD). METHODS: S' values in patients with TOF (n = 183) and PAH-CHD (n = 55) were compared with those in normal subjects. S' values were compared with RV ejection fraction and RV end-diastolic volume index (RVEDVi) determined by magnetic resonance imaging. RESULTS: S' values became significantly reduced in PAH-CHD patients after 10.4 years of age and after 13.6 years of age in patients with TOF compared with the lower boundary of the ±2-SD interval of normal subjects. Significant positive correlations between S' and RV ejection fraction were seen in patients with TOF (r = 0.66, P < .001) and those with PAH-CHD (r = 0.82, P < .001). Significant negative correlations between S' and RVEDVi were also seen in patients with repaired TOF (r = -0.29, P = .002) and in those with PAH-CHD (r = -0.59, P < .001). CONCLUSIONS: Although initially preserved, in this prospective study, impaired S' values with increasing age were found in patients with repaired TOF and PAH-CHD. Persistent pressure overload in patients with PAH-CHD as well as volume overload in those with repaired TOF might lead to systolic RV functional impairment and increased RVEDVi. The validity of S' data was supported by magnetic resonance imaging data (RVEDVi and RV ejection fraction).

Systolic right ventricular function in children and young adults with pulmonary artery hypertension secondary to congenital heart disease and tetralogy of Fallot: tricuspid annular plane systolic excursion (TAPSE) and magnetic resonance imaging data.

OBJECTIVE: The tricuspid annular plane systolic excursion (TAPSE), as echocardiographic index to assess right ventricular (RV) systolic function, has not been investigated thoroughly in children and young adults with tetralogy of Fallot (TOF) and pulmonary artery hypertension secondary to congenital heart disease (PAH-CHD). PATIENTS: TAPSE values of 49 patients with PAH-CHD and 156 patients with TOF were compared with age-matched normal subjects. TAPSE values were also compared with RV ejection fraction (RVEF) and RV indexed end-diastolic volume (RVEDVi) determined by magnetic resonance imaging in PAH-CHD and TOF patients. RESULTS: Patients with a PAH-CHD showed a positive correlation between TAPSE with RVEF (r= 0.81; P < 0.001) and a negative correlation between TAPSE with RVEDVi (r=-0.67; P < 0.001). Similarly, in our TOF patients, a positive correlation between TAPSE with RVEF (r= 0.65; P < 0.001) and a negative correlation between TAPSE with RVEDVi (r=-0.42; P < 0.001) was seen. CONCLUSIONS: Significant pressure overload in PAH-CHD patients and volume overload in TOF patients lead to a decreased systolic RV function, determined by TAPSE and magnetic resonance imaging and to increased RVEDVi values, determined by MRI, with time.

Hemodiafiltration in infants with complications during peritoneal dialysis.

End-stage renal disease (ESRD) in neonates still has a high mortality, particularly in the first year of life. We present the combination of peritoneal dialysis (PD) with intermittent hemodiafiltration (iHDF) in neonates with ESRD. Four infants younger than 28 days were treated with PD and iHDF. Renal diagnoses leading to ESRD were cortical necrosis, prune belly syndrome, neonatal hemolytic uremic syndrome, and autosomal recessive polycystic kidney disease. Initially, three patients were on iHDF until PD was started. At the time when complications occurred during PD, patients were switched back to iHDF. iHDF was used five times as a bridge to PD in case of abdominal surgery. Two of the four patients were switched to iHDF because of peritoneal ultrafiltration failure due to recurrent peritoneal leaks. Once, iHDF became necessary due to refractory peritonitis. All four patients survived the first year of life. Two patients were transplanted successfully at an age of 35 and 22 months, respectively. The others are on renal replacement therapy, one on PD at the age of 28 months and one on iHDF at the age of 25 months, respectively. In case of PD complications, iHDF may be an appropriate bridge to achieve long-term survival until kidney transplantation.

Reference values of tricuspid annular peak systolic velocity in healthy pediatric patients, calculation of z score, and comparison to tricuspid annular plane systolic excursion.

The tricuspid annular peak systolic velocity (TAPSV) is an echocardiographic measurement assessing right ventricular systolic function in children and adults. We determined the growth-related changes of the TAPSV to establish the references values for the entire pediatric age group. A prospective study was conducted of a group of 860 healthy pediatric patients (age 1 day to 18 years; body surface area [BSA] 0.14 to 2.30 m(2)). We determined the effects of age, gender, and BSA on the TAPSV values. Stepwise linear multiple regression analysis was used to estimate the TAPSV from the age, BSA, and gender. A correlation of normal TAPSV with normal tricuspid annular plane systolic excursion values was performed. The TAPSV ranged from a mean of 7.2 cm/s (z score ± 2: 4.8 to 9.5 cm/s) in the newborn to 14.3 cm/s (z score ± 2: 10.6 to 18.6 cm/s) in the 18-year-old adolescent. The TAPSV values showed a positive correlation with age and BSA, with a nonlinear course. No significant difference was found in the TAPSV values according to gender. A significant correlation was found between the TAPSV and tricuspid annular plane systolic excursion values in our pediatric population. In conclusion, the z scores of the TAPSV values were calculated, and percentile charts were established to serve as reference data for patients with congenital heart disease.

Impact of the permanent ventricular pacing site on left ventricular function in children: a retrospective multicentre survey.

BACKGROUND: Chronic right ventricular (RV) pacing is associated with deleterious effects on cardiac function. OBJECTIVE: In an observational multicentre study in children with isolated atrioventricular (AV) block receiving chronic ventricular pacing, the importance of the ventricular pacing site on left ventricular (LV) function was investigated. METHODS: Demographics, maternal autoantibody status and echocardiographic measurements on LV end-diastolic and end-systolic dimensions and volumes at age <18 years were retrospectively collected from patients undergoing chronic ventricular pacing (>1 year) for isolated AV block. LV fractional shortening (LVFS) and, if possible LV ejection fraction (LVEF) were calculated. Linear regression analyses were adjusted for patient characteristics. RESULTS: From 27 centres, 297 children were included, in whom pacing was applied at the RV epicardium (RVepi, n = 147), RV endocardium (RVendo, n = 113) or LV epicardium (LVepi, n = 37). LVFS was significantly affected by pacing site (p = 0.001), and not by maternal autoantibody status (p = 0.266). LVFS in LVepi (39 ± 5%) was significantly higher than in RVendo (33 ± 7%, p < 0.001) and RVepi (35 ± 8%, p = 0.001; no significant difference between RV-paced groups, p = 0.275). Subnormal LVFS (LVFS < 28%) was seen in 16/113 (14%) RVendo-paced and 21/147 (14%) RVepi-paced children, while LVFS was normal (LVFS ≥ 28%) in all LVepi-paced children (p = 0.049). These results are supported by the findings for LVEF (n = 122): LVEF was <50% in 17/69 (25%) RVendo- and in 10/35 (29%) RVepi-paced patients, while LVEF was ≥ 50% in 17/18 (94%) LVepi-paced patients. CONCLUSION: In children with isolated AV block, permanent ventricular pacing site is an important determinant of LV function, with LVFS being significantly higher with LV pacing than with RV pacing.

Differentiating inherited human herpesvirus type 6 genome from primary human herpesvirus type 6 infection by means of dried blood spot from the newborn screening card.

To differentiate active human herpesvirus type 6 (HHV-6) infection from inherited HHV-6 (iHHV-6), we analyzed dried blood spots from archived newborn screening cards in 3 patients with high HHV-6 DNA copy numbers. Two patients were positive for HHV-6 DNA as neonates suggesting iHHV-6. In 1 patient, the absence of HHV-6 DNA excluded iHHV-6.