Bert Nagel

Right ventricular function in infants, children and adolescents: reference values of the tricuspid annular plane systolic excursion (TAPSE) in 640 healthy patients and calculation of z score values.

BACKGROUND Tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement to assess right ventricular systolic function in adults. The aim of this study was to determine growth-related changes in TAPSE to establish references values. METHODS A prospective study was conducted in a group of 640 healthy pediatric patients (age range, 1 day to 18 years; body surface area range, 0.12-2.25 m(2)). The effects of age and body surface area on TAPSE were determined. RESULTS TAPSE ranged from a mean of 0.91 cm (z score +/- 3, 0.56-1.26 cm) in neonates to 2.47 cm (z score +/- 3, 1.84-3.10 cm) in 18-year-olds. TAPSE values showed positive correlations with age and body surface area. There was no significant difference in TAPSE values between female or male children. CONCLUSION In this study, z scores of TAPSE values were calculated and percentile charts were established to serve as reference data for ready application in patients with congenital heart disease in the future.

Systolic Right Ventricular Function in Preterm and Term Neonates: Reference Values of the Tricuspid Annular Plane Systolic Excursion (TAPSE) in 258 Patients and Calculation of Z-Score Values

Background: The tricuspid annular plane systolic excursion (TAPSE) is an echocardiographic measurement to assess right ventricular systolic function in adults and children. Objective: We determined growth- and birth weight-related changes of TAPSE to establish reference values in preterm and term neonates. Methods: A prospective study was conducted in a group of 258 preterm and term neonates (age: 25+0 to 40+6 weeks of gestation, birth weight: 530–4,200 g). Results: The TAPSE ranged from a mean of 0.44 cm (Z-score ±2: 0.30–0.59 cm) in preterm neonates in the 26th week of gestation to 1.03 cm (Z-score ±2: 0.85–1.21 cm) in term neonates in the 41st week of gestation. The TAPSE values increased in a linear way from the 26th to 41st week of gestation. TAPSE, week of gestation and weight are strongly correlated: Pearson’s correlation coefficient was 0.93 for week of gestation – TAPSE (p < 0.001), 0.93 for week of gestation – birth weight (p < 0.001), and 0.89 for birth weight – TAPSE (p < 0.001). There was no statistically significant difference of normal TAPSE values between female and male patients (p = 0.987). Conclusion: Z-scores of TAPSE values were calculated and percentile charts were established to serve as reference data for ready application in preterm and term neonates with structurally normal hearts and with congenital heart disease in the future.

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.

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.

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.

Systolic right ventricular function in pediatric and adolescent patients with tetralogy of Fallot: echocardiography versus magnetic resonance imaging.

OBJECTIVE The tricuspid annular plane systolic excursion (TAPSE) as an echocardiographic index to assess right ventricular (RV) systolic function has not been investigated thoroughly in pediatric patients and adolescents with tetralogy of Fallot (TOF) after surgical repair. METHODS TAPSE was determined in 131 patients with TOF and 252 age-matched normal subjects. TAPSE values were compared with RV ejection fraction (EF) and indexed RV end-diastolic volume (EDVi) determined by magnetic resonance imaging in a cross-sectional study design. TAPSE values were also correlated to QRS duration (QRSd) determined by electrocardiogram. RESULTS The TAPSE values showed a positive correlation with age in normal subjects. The TAPSE was not decreased in infants and young children with TOF compared with normal subjects. A significant reduction of TAPSE values with increasing time after surgical repair was observed. After a mean of 7 years after surgical repair, the TAPSE values become significantly reduced compared with age-matched controls, being below the lower bound of -2 standard deviations. A positive correlation between TAPSE with RVEF and a negative correlation between TAPSE with RVEDVi were observed. A significant positive correlation was found between QRSd and RVEDVi, and a significant negative correlation was found between QRSd and RVEF. CONCLUSION Although TAPSE was initially preserved, impaired TAPSE was observed with increasing time after surgical repair in pediatric patients with TOF.

Splenic state in surviving patients with visceral heterotaxy.

AIM To identify patients with visceral heterotaxy who are at risk from fulminant sepsis. METHODS We studied 38 patients, 37 having undergone abdominal ultrasound, all 38 having examination of blood films to establish presence of Howell-Jolly bodies, and all 38 documented to have had pneumococcal vaccination and prophylaxis with penicillin. We checked whether the parents were aware of the splenic state of their child, and when possible, we compared current results of blood films with those obtained postnatally. RESULTS Two of the 17 patients with multiple spleens, all 11 without a detectable spleen, and 1 of 9 patients with a normal spleen, showed Howell-Jolly bodies in their blood films. In 5 of 23 patients with serial blood films, Howell-Jolly bodies had not been seen postnatally, but could now be detected in current blood films. Of these patients, 2 had multiple spleens, 1 did not have a spleen, and 1 had a solitary spleen of normal size. In the other patient, ultrasound could not be performed. Only one of these patients was receiving penicillin prophylactically, and had received pneumococcal vaccination. Of the 15 patients in whom Howell-Jolly bodies were present in the blood, only 8 parents knew about the potential risk for infection. Another 7 parents were sure that their child was taking penicillin regularly, and had received pneumococcal vaccination. CONCLUSIONS Howell-Jolly bodies can be found in the blood of patients with visceral heterotaxy independent of the anatomical state of the spleen. As Howell-Jolly bodies can be encountered in the blood of such patients with increasing age, those with multiple and solitary spleens should be monitored regularly to identify those at risk. Parental knowledge of the splenic state, and compliance for prophylaxis using penicillin, and pneumococcal vaccination, were unsatisfactory in our cohort.

Congestive heart failure in childhood. An epidemiologic study

ZusammenfassungHintergrund:Obwohl es zahlreiche epidemiologische Studien über die Herzinsuffizienz (HI) bei Erwachsenen gibt, liegen bis jetzt keine Daten für Kinder vor.Methodik:Retrospektiv wurden alle Kinder untersucht, die von 1989 bis 1998 mit folgenden Diagnosen in der Universitäts-Kinderklinik Essen stationär und nachfolgend ambulant behandelt worden waren (herausgesucht über ICD-9-Schlüssel): angeborene und erworbene Herzfehler, Kardiomyopathien, Rhythmusstörungen, arterielle und pulmonale Hypertonie und sonstige kardiovaskuläre Fehlbildungen. Die Diagnose einer HI wurde über die typischen Symptome definiert, Beginn und Ende über das An- und Absetzen der Therapie mit ACE-Hemmern und/oder Diuretika und/oder Digitalis, auch im Verlauf der postoperativen Therapie. Als Bezugsgruppe fungieren sowohl die Kinder mit o. g. kardiologischen Diagnosen (1 755 herzkranke Kinder) als auch alle stationären Kinder der gesamten Kinderklinik während des Beobachtungszeitraums (25 227).Ergebnisse:587 (33,4%) der herzkranken Kinder (n = 1 755) und 507 (39,1%) der Kinder mit angeborenem Herzfehler (n = 1 297) wurden innerhalb der 10 Jahre herzinsuffizient. Ohne die Fälle postoperativer HI wurden 23,7% der Kinder mit angeborenen Herzfehlern herzinsuffizient.Kumulative Inzidenz der HI bei herzkranken Kindern: 334 auf 1 000 herzkranke und 23,3 auf 1 000 aller stationär behandelten Kinder; bei Kindern mit angeborenen Herzfehlern: 289 auf 1 000 Kinder mit kardiologischer Diagnose und 20,1 auf 1 000 stationäre Kinder.Prävalenz der HI bei herzkranken Kindern: 279 auf 1 000 herzkranke und 17,3 auf 1 000 stationäre Kinder; bei Kindern mit angeborenen Herzfehlern: 261 auf 1 000 herzkranke und 16,1 auf 1 000 stationäre Kinder. Die HI trat zu 70,6% im 1. Lebensjahr auf und dauerte durchschnittlich 15 Monate. Bei 78% der Patienten mit angeborenen Herzfehlern endete sie mit einer Operation.Im 10-jährigen Beobachtungszeitraum starben 111 der 1 755 herzkranken Kinder, 81 von ihnen an der HI. Das bedeutet eine Gesamtmortalität von 6,3%, 18% nach einer Herzoperation oder -katheterisierung, 74% mit den Zeichen der HI. Von den Kindern mit angeborenen Herzfehlern starben 6,2%. Umgekehrt starben von den 587 Kindern mit HI 81 (14%); 67% der Todesfälle ereigneten sich im 1. Lebensjahr, bei Patienten mit angeborenen Herzfehlern sogar 71%.Schlussfolgerung:Die HI bei Kindern ist insgesamt selten, spielt aber eine entscheidende Rolle beim Schicksal der herzkranken Kinder, vor allem bei angeborenen Herzfehlern. Sie weist im Gegensatz zur HI der Erwachsenen eine deutlich bessere Prognose auf, weil häufig die Möglichkeit einer operativen/interventionellen Therapie besteht. Eine prospektive Registererfassung ist dringend notwendig.AbstractBackground:In contrast to the adult age group epidemiologic studies on congestive heart failure (CHF) in infancy and childhood are lacking.Methods:Retrospective study of all patients admitted to the University Children’s Hospital Essen, Germany, between 1989 and 1998 with the ICD 9 code for congenital and acquired heart diseases, cardiomyopathies, arrhythmias, arterial und pulmonary hypertension, and other cardiovascular anomalies. CHF was defined by the typical symptoms, start and end of CHF by commencement and end of anticongestive therapy (ACE inhibitors and/or diuretics and/or digoxin). Prevalence and incidence of CHF were determined and related to all patients with congenital and acquired heart diseases and to all general pediatric inpatients at the Children’s Hospital from 1989 to 1998.Within 10 years 1,755 children with heart diseases were admitted, 918 boys and 837 girls. 1,297 children suffered from congenital heart diseases, 167 from rhythm disturbances, 110 from cardiomyopathies and acquired heart diseases, while prematures and patients with systemic or pulmonary hypertension are not furthermore topic of this study.Results:CHF occurred in 587 (33.4%) out of 1,755 children with all congenital and acquired heart diseases, and in 507 (39.1%) out of 1,297 children with congenital heart defects (CHD). When postoperative CHF was excluded, CHF occurred in 23.7% of children with CHD.Cumulative incidence of CHF was 334 out of 1,000 patients with all heart diseases and 233 out of 1,000 general pediatric inpatients. For patients with CHD the incidence of CHF was 289 out of 1,000 patients with all heart diseases and 20.1 out of 1,000 general pediatric inpatients.Prevalence of CHF for children with heart diseases was 279 in 1,000 and 17.3 in 1,000 general pediatric inpatients, for children with CHD 261 in 1,000 and 16.1 in 1,000 general pediatric inpatients. CHF occurred in 70.6% during the 1st year of life and lasted for a mean of 15 months. Only in patients with cardiomyopathies and acquired heart diseases the incidence in infancy was not so pronounced. In 78% of patients with CHD CHF ends after an operation.Mortality: during the 10-year interval 111 out of 1,755 patients with heart diseases died, 81 of them for CHF. That gives an overall mortality of 6.3%, 18% following heart surgery or cardiac catheterization, 74% with signs of CHF. In patients with CHD mortality was 6.2%. Out of the 587 children with CHF, 81 (14%) died. 67% of deaths occurred during the 1st year of life, in patients with CHD even in 71%.Conclusion:CHF is uncommon in infants and children with congenital or acquired heart disease, but has considerable mortality. As surgical or interventional therapy is well established in nearly all patients with CHD, prognosis is much better compared to adults. A prospective evaluation by a nationwide registry is necessary.

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 260-6 of gestation to 7.8 cm/s (Z-score ± 2 SD: 5.5-10.1 cm/s) in term neonates in week 400-6 of gestation. The S′ values increased from 26 to 40 weeks of gestation. S′ and week of gestation are strongly correlated: Pearsons 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.