What every radiologist should know about paediatric echocardiography.

Congenital heart defects (CHD) occur in less than one percent of all newborns. Echocardiography represents the imaging modality of choice for morphological and functional assessment. In childhood the different CHD types can be diagnosed trustfully and can be performed bedside. In the follow-up of CHD cross sectional imaging plays an important role and therefore it is essential for the radiologist to know the features, challenges and limitations of echocardiography. Within this review article a systematic approach for morphological and functional assessment of the heart will is given along with representative example images. In addition, typical echocardiographic findings in common CHD is presented. In older children, adolescents and grown-ups with CHD (GUCH) echocardiography suffers from limitations - partially due to skeletal deformations and lung emphysema. In particular right ventricular function assessment is not always possible by echocardiography. Therefore strengths and limitations of echocardiography will be discussed the role of cardiac magnetic resonance imaging (cMRI) and cardiac computed tomography (cCT) emphasized.

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.

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.

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).

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.

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.

Tricuspid annular plane systolic excursion and right ventricular ejection fraction in pediatric and adolescent patients with tetralogy of Fallot, patients with atrial septal defect, and age-matched normal subjects.

AIMS: To determine whether TAPSE is an accurate marker of right ventricular (RV) systolic function in patients with tetralogy of Fallot (TOF) and patients with small atrial septal defect (ASD). The tricuspid annular plane systolic excursion (TAPSE) values were measured and compared with RV ejection fraction (EF). METHODS AND RESULTS: A prospective study was conducted in pediatric and adolescent patients with TOF (n = 110), with isolated small secundum ASD (n = 200), and age-matched patients with normally structured heart. The TAPSE values showed a positive correlation with age in both patients with ASD and normal subjects. No significant difference of TAPSE values was seen in control subjects and age-matched ASD patients. The TAPSE was not decreased compared to normal subjects in eight infant TOF patients before corrective surgery. A reduction of TAPSE values with increasing time interval following corrective surgery was seen. After a mean of 7 years TAPSE values become significantly reduced compared to age-matched controls, being below the lower bound of the -2 SD. CONCLUSION: In ASD patients the systolic RV function was preserved over the pediatric age group when compared to normal subjects. In contrast, although initially preserved, we found an impaired TAPSE with increasing postoperative period in our TOF patients.

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.

Campylobacter jejuni infection associated with myopericarditis in adolescents: report of two cases.

Two cases of myopericarditis associated with Campylobacter jejuni infection in male adolescents are presented. C. jejuni is the most common cause of bacterial gastroenteritis worldwide; however, cardiac complications are rare, even in adults. To our knowledge, these are the first reported cases of campylobacter-related myopericarditis in adolescents.

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.

Therapeutic experience with infliximab in a patient with polyarticular juvenile idiopathic arthritis and uveitis.

A pediatric patient with prolonged seronegative polyarticular juvenile idiopathic arthritis (JIA) and concomitant aggressive, anterior uveitis refractory to any conventional antirheumatic therapy was treated with infliximab. Arthritis and C-reactive protein (CRP) values showed prompt positive effects but, after 6 weeks, returned gradually to initial values despite ongoing therapy. In contrast, a more sustained therapeutic effect was observed on the uveitis, with increased visual acuity and reduced inflammatory signs of the affected eye. However, this benefit was also lost at week 30, after which infliximab had to be discontinued due to side effects. To conclude, in polyarticular seronegative JIA, infliximab showed a transient beneficial effect which was more pronounced on uveitis than arthritis.

Effects of inhaled gentamicin prophylaxis on acquisition of Pseudomonas aeruginosa in children with cystic fibrosis: a pilot study.

Inhaled antibiotics are an established treatment for chronic Pseudomonas aeruginosa (PA) infection in patients with cystic fibrosis (CF). However, inhaled antibiotics might also have prophylactic potential to delay acquisition of PA in early stages of the disease. From 1986-1999, all CF patients at this center who experienced defined risk situations for acquisition of PA (28 patients) received inhaled gentamicin (80 mg BID for those < 12 months; 120 mg BID for those > 12 months) for a minimum of 3 years. Twelve patients had repeated risk situations and continued this prophylaxis without interruption during the entire study period (group 1). In the remaining 16 patients, inhaled antibiotics were discontinued at various times for a variety of reasons (group 2). None of the patients in group 1, but 7 in group 2, became chronically infected with PA (P = 0.01). Lung function and chest X-ray scores were significantly worse in those 7 infected patients, when compared to the noninfected ones in both groups. This suggests that long-term-prophylaxis with inhaled gentamicin can effectively delay acquisition of PA and decrease disease progression in children with CF.