Physical fitness in children with Marfan and Loeys-Dietz syndrome: associations between cardiovascular parameters, systemic manifestations, fatigue, and pain.

Children with Marfan (MFS) and Loeys-Dietz syndrome (LDS) report limitations in physical activities, sports, school, leisure, and work participation in daily life. This observational, cross-sectional, multicenter study explores associations between physical fitness and cardiovascular parameters, systemic manifestations, fatigue, and pain in children with MFS and LDS. Forty-two participants, aged 6-18 years (mean (SD) 11.5(3.7)), diagnosed with MFS (n = 36) or LDS (n = 6), were enrolled. Physical fitness was evaluated using the Fitkids Treadmill Test's time to exhaustion (TTE) outcome measure. Cardiovascular parameters (e.g., echocardiographic parameters, aortic surgery, cardiovascular medication) and systemic manifestations (systemic score of the revised Ghent criteria) were collected. Pain was obtained by visual analog scale. Fatigue was evaluated by PROMIS® Fatigue-10a-Pediatric-v2.0-short-form and PROMIS® Fatigue-10a-Parent-Proxy-v2.0-short-form. Multivariate linear regression analyses explored associations between physical fitness (dependent variable) and independent variables that emerged from the univariate linear regression analyses (criterion p < .05). The total group (MFS and LDS) and the MFS subgroup scored below norms on physical fitness TTE Z-score (mean (SD) -3.1 (2.9); -3.0 (3.0), respectively). Univariate analyses showed associations between TTE Z-score aortic surgery, fatigue, and pain (criterion p < .05). Multivariate analyses showed an association between physical fitness and pediatric self-reported fatigue that explained 48%; 49%, respectively, of TTE Z-score variance (F (1,18) = 18.6, p ≤ .001, r2 = .48; F (1,15) = 16,3, p = .01, r2 = .49, respectively).    Conclusions: Physical fitness is low in children with MFS or LDS and associated with self-reported fatigue. Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue, ultimately enhancing the physical activity and sports, school, leisure, and work participation of children with MFS and LDS. What is Known: • Marfan and Loeys-Dietz syndrome are heritable connective tissue disorders and share cardiovascular and systemic manifestations. • Children with Marfan and Loeys-Dietz syndrome report increased levels of disability, fatigue and pain, as well as reduced levels of physical activity, overall health and health-related quality of life. What is New: • Physical fitness is low in children with Marfan and Loeys-Dietz syndrome and associated with self-reported fatigue. • Our findings emphasize the potential of standardized and tailored exercise programs to improve physical fitness and reduce fatigue, ultimately enhancing the physical activity and sports, school, leisure, and work participation of children with Marfan and Loeys-Dietz syndrome.

Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients.

In patients born with anorectal malformations (ARM), additional congenital heart defects (CHD) can occur. We aimed to provide an overview on disease and treatment details of CHD identified in patients born with ARM, from a unique large cohort of a very rare disease. We performed a retrospective single-center cohort study between January 2000 and July 2023. All consecutive patients with ARM were included. Outcomes were the number of patients with CHD, and screening percentage and percentage of patients diagnosed with CHD over 3 time periods (2000-2006, 2007-2014, 2015-2023). We used uni- and multi-variable logistic regression analyses to search for associations between CHD present and baseline characteristics. In total, 281 patients were included. Some 241 (85.8%) underwent echocardiography, of whom 80 (33.2%) had CHD. Screening percentage with echocardiography increased (74.1% vs. 85.7% vs. 95.9%, p < 0.001) and percentage of patients diagnosed with CHD remained similar over time (30.2% vs. 34.5% vs. 34.0%, p = 0.836). Atrial and ventricular septal defects (n = 36, n = 29), and persistent left superior vena cava (n = 17) were most identified. The presence of VACTERL-association or a genetic syndrome was independently associated with the presence of CHD. CHD were present in 33% of patients with ARM that underwent echocardiography. Over time, the number of CHD identified through screening remained similar. Patients with the presence of VACTERL-association or a genetic syndrome had a higher risk of having CHD. Therefore, acknowledging the potential presence of CHD in patients with ARM remains important.

Structured Multidisciplinary Follow-Up After Pediatric Intensive Care: A Model for Continuous Data-Driven Health Care Innovation.

OBJECTIVES: Morbidity after PICU admission for critical illness is a growing concern. Sequelae may occur in various domains of functioning and can only appropriately be determined through structured follow-up. Here, we describe the process of designing and implementing a structured multidisciplinary follow-up program for patients and their parents after PICU admission and show the first results illustrating the significance of our program. DESIGN: Prospective observational cohort study. SETTING: Outpatient PICU follow-up clinic. PATIENTS: Patients 0-18 years old admitted to our PICU. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: In our structured multidisciplinary follow-up program, follow-up care is provided by a pediatric intensivist and psychologist and in addition, depending on patient's critical illness and received PICU treatment(s), by a pediatric pulmonologist, cardiologist, neurologist, and/or neuropsychologist. All consultations are scheduled consecutively. Collected data are stored in a hospital-wide data warehouse and used for yearly health care evaluation sessions as well as scientific research. Challenges in organizing this follow-up program include technological challenges, providing time-efficient care, participation rate, and completeness of questionnaires. In our experience, a dedicated team is essential to tackle these challenges. Our first results, obtained in 307 of 388 referred patients (79.1%), showed the diversity of problems arising after PICU discharge, including physical, neurocognitive, and psychosocial sequelae. In addition, our data also reflected the risk of psychosocial problems among parents. Within the limited operation time of our follow-up program, the program has evolved based on our experiences and the data collected. CONCLUSIONS: We successfully developed and implemented a structured multidisciplinary follow-up program for patients and their parents after PICU admission. This program may help to timely initiate appropriate interventions, improve the standard of care during and after PICU admission, and facilitate scientific research on outcome and prognosis after PICU admission.

Tissue Doppler imaging detects impaired biventricular performance shortly after congenital heart defect surgery.

Cardiac surgery with cardiopulmonary bypass is associated with the development of a systemic inflammatory response, which can lead to myocardial damage. However, knowledge concerning the time course of ventricular performance deterioration and restoration after correction of a congenital heart defect (CHD) in pediatric patients is sparse. Therefore, the authors perioperatively quantified left ventricular (LV) and right ventricular (RV) performance using echocardiography. Their study included 141 patients (ages 0-18 years) undergoing CHD correction and 40 control subjects. The study assessed LV systolic performance (fractional shortening) and diastolic performance (mitral Doppler flow) in combination with RV systolic performance [tricuspid annular plane systolic excursion (TAPSE)] and diastolic performance (tricuspid Doppler flow). Additionally, systolic (S') and diastolic (E', A', E/E') tissue Doppler imaging (TDI) measurements were obtained at the LV lateral wall, the interventricular septum, and the RV free wall. Echocardiographic studies were performed preoperatively, 1 day postoperatively, and at hospital discharge after 9 ± 5 days. Although all LV echocardiographic measurements showed a deterioration 1 day after surgery, only LV TDI measurements were impaired in patients at discharge versus control subjects (S': 5.7 ± 2.0 vs 7.1 ± 2.7 cm/s; E': 9.8 ± 3.9 vs 13.7 ± 5.1 cm/s; E/E': 12.2 ± 6.4 vs 8.8 ± 4.3; p < 0.05). In the RV, TAPSE and RV TDI velocities also were impaired in patients at discharge versus control subjects (TAPSE: 9 ± 3 vs 17 ± 5 mm; S': 5.2 ± 1.7 vs 11.4 ± 3.4 cm/s; E': 7.3 ± 2.5 vs 16.3 ± 5.2 cm/s; E/E': 12.5 ± 6.8 vs 4.8 ± 1.9; p < 0.05). Furthermore, longer aortic cross-clamp times were associated with more impaired postoperative LV and RV performance (p < 0.05). In conclusion, both systolic and diastolic biventricular performances were impaired shortly after CHD correction. This impairment was detected only by TDI parameters and TAPSE. Furthermore, a longer-lasting negative influence of cardiopulmonary bypass on myocardial performance was suggested.

Cardiac MRI in postoperative congenital heart disease patients.

The survival of patients with congenital heart disease (CHD) has greatly improved over the past decades. Nevertheless, lifelong follow-up is required in postoperative CHD patients, and noninvasive imaging plays an important role during follow-up. Cardiac MR (CMR) imaging enables comprehensive imaging of cardiac function and anatomy, and helps to detect patients who need re-intervention and to predict clinical outcome. Postoperative CHD patients who are frequently referred for CMR evaluation include those with coarctation of the aorta, tetralogy of Fallot, transposition of the great arteries, and single ventricle patients after the Fontan procedure. This article reviews the current clinical role of CMR in these various subgroups of postoperative CHD patients. Furthermore, an overview of novel CMR applications and their clinical value in CHD patients is provided.

Cardiac resynchronization therapy in paediatric and congenital heart disease patients.

The number of patients with congenital heart disease (CHD) has significantly increased over the last decades. The CHD population has a high prevalence of heart failure during late follow-up and this is a major cause of mortality. Cardiac resynchronization therapy (CRT) may be a promising therapy to improve the clinical outcome of CHD and paediatric patients with heart failure. However, the CHD and paediatric population is a highly heterogeneous group with different anatomical substrates that may influence the effects of CRT. Echocardiography is the mainstay imaging modality to evaluate CHD and paediatric patients with heart failure and novel echocardiographic tools permit a comprehensive assessment of cardiac dyssynchrony that may help selecting candidates for CRT. This article reviews the role of CRT in the CHD and paediatric population with heart failure. The current inclusion criteria for CRT as well as the outcomes of different anatomical subgroups are evaluated. Finally, echocardiographic assessment of mechanical dyssynchrony in the CHD and paediatric population and its role in predicting response to CRT is comprehensively discussed.

Growth of the aortic root in children and young adults with Marfan syndrome.

OBJECTIVES: The primary aim was to gain insight into the growth of the aortic root in children and young adults with Marfan syndrome (MFS). Furthermore, we aimed to identify a clinical profile of patients with MFS who require an aortic root replacement at a young age with specific interest in age, sex, height and fibrillin-1 (FBN1) genotype. METHODS: Aortic root dimensions of 97 patients with MFS between 0 year and 20 years and 30 controls were serially assessed with echocardiography. Trends were analysed using a linear mixed-effect model. Additionally, including only patients with MFS, we allowed trends to differ by sex, aortic root replacement and type of FBN1 mutation. RESULTS: Average aortic root dilatation in patients with MFS became more pronounced after the age of 8 years. In the MFS cohort, male patients had a significantly greater aortic root diameter than female patients, which was in close relationship with patient height. There was no difference in aortic root growth between children with dominant negative (DN) or haploinsufficient FBN1 mutations. However, DN-FBN1 variants resulting in loss of cysteine content were associated with a more severe phenotype. Eleven children needed an aortic root replacement. Compared with patients with MFS without aortic root surgery, these children had a significantly larger aortic root diameter from an early age. CONCLUSIONS: This study provides clinically useful longitudinal growth charts on aortic root growth in children and young adults with MFS. Children requiring prophylactic aortic root replacement during childhood can be identified at a young age. Our growth charts can help clinicians in decision making with regard to follow-up and prophylactic therapy. Loss of cysteine content in the FBN1 protein was associated with larger aortic root dimensions.

Corrected tetralogy of Fallot: comparison of tissue doppler imaging and velocity-encoded MR for assessment of performance and temporal activation of right ventricle.

PURPOSE: To compare velocity-encoded (VE) magnetic resonance (MR) imaging with tissue Doppler imaging to assess right ventricular (RV) peak systolic velocities and timing of velocities in patients with corrected tetralogy of Fallot and healthy subjects. MATERIALS AND METHODS: Local institutional review board approval was obtained; patients or their parents gave informed consent. Thirty-three patients (20 male, 13 female; median age, 12 years; interquartile range [IQR], 11-15 years; age range, 8-18 years) and 19 control subjects (12 male, seven female; median age, 14 years; IQR, 12-16 years; age range, 8-18 years) underwent VE MR imaging and tissue Doppler imaging. Peak systolic velocity and time to peak systolic velocity (percentage of cardiac cycle) were assessed at the RV free wall (RVFW) and RV outflow tract (RVOT). Data were analyzed by using linear regression, paired and unpaired tests, and Bland-Altman plots. RESULTS: Good correlation and agreement between the two techniques were observed. For peak systolic velocity at RVFW, r = 0.95 (mean difference, -0.4 cm/sec, P < .01), and at RVOT, r = 0.95 (mean difference, -0.4 cm/sec, P = .02). For timing at RVFW, r = 0.94 (mean difference, -0.2%, P = .44), and at RVOT, r = 0.89 (mean difference, -0.5%, P = .01). Peak systolic velocity was reduced in patients with corrected tetralogy of Fallot (at RVFW, median was 8.2 cm/sec [IQR, 6.4-9.7 cm/sec] vs 12.4 cm/sec [IQR, 10.8-13.8 cm/sec], P < .01; at RVOT, 4.7 cm/sec [IQR, 4.1-7.2 cm/sec] vs 10.2 cm/sec [IQR, 8.7-11.2 cm/sec], P < .01). The time delay between RVFW and RVOT was observed, which was significantly shorter in patients with corrected tetralogy of Fallot (median, 5.9% [IQR, 4.9%-7.4%] vs 8.4% [IQR, 6.6%-12.4%], P < .01). CONCLUSION: VE MR imaging and tissue Doppler imaging enable assessment of RV systolic performance and timing of velocities at the RVFW and RVOT in patients with corrected tetralogy of Fallot. Both techniques can be used interchangeably to clinically assess velocities and timing of velocities of the RV.

Tissue Doppler imaging in the left ventricle and right ventricle in healthy children: normal age-related peak systolic velocities, timings, and time differences.

AIMS: Tissue Doppler imaging (TDI) enables assessment of velocities and timings within the left (LV) and the right (RV) ventricle with high temporal resolution. Knowledge on normal age-related values of peak systolic velocities and timings in healthy children may optimize the benefit of device-based therapies in paediatric patients with heart failure. METHODS AND RESULTS: A total of 123 healthy children (from 1 month to 18 years old) underwent TDI evaluation of the RV and LV. Peak systolic velocity and time to peak systolic velocity were assessed at the basal LV lateral wall, inter-ventricular septum (IVS), RV free wall (RVFW), and at the RV outflow tract (RVOT). Intra-ventricular time differences were calculated. Regression analysis was performed to assess the age dependency of the ventricular mechanics. Median peak velocities were: LV lateral wall: 6.3 cm/s (inter-quartile range (IQR): 5.1-7.9 cm/s); IVS: 6.0 cm/s (5.4-6.7 cm/s); RVFW: 10.2 cm/s (8.9-11.3 cm/s); RVOT: 7.2 cm/s (6.0-8.2 cm/s). Timings of peak systolic velocities were: LV lateral wall: 101 ms (91-112 ms); IVS: 114 ms (100-128 ms); RVFW: 177 ms (157-194 ms); RVOT: 100 ms (88-113 ms). Timings and peak velocities significantly increased with age at both ventricles. No relevant time difference was observed within the LV, whereas a considerable time delay was observed within the RV between the RVFW and the IVS (62 ms, IQR: 45-74 ms) and between the RVFW and the RVOT (74 ms, IQR: 59-93 ms). CONCLUSION: The present evaluation provides TDI-derived physiological values on normal LV and RV mechanics of healthy children. Within the LV, no relevant time difference was observed, whereas a considerable mechanical delay is observed within the healthy RV.

Tetralogy of fallot: 3D velocity-encoded MR imaging for evaluation of right ventricular valve flow and diastolic function in patients after correction.

PURPOSE: To evaluate three-dimensional (3D) velocity-encoded (VE) magnetic resonance (MR) imaging, as compared with two-dimensional (2D) VE MR imaging, for assessment of pulmonary valve (PV) and tricuspid valve (TV) flow, with planimetry as the reference standard, and to evaluate diastolic function in patients with a corrected tetralogy of Fallot (TOF). MATERIALS AND METHODS: Local institutional review board approval was obtained, and patients or their parents gave informed consent. Twenty-five patients with a corrected TOF (12 male, 13 female; mean age, 13.1 years +/- 2.7 [standard deviation]; age range, 8-18 years) and 19 control subjects (12 male, seven female; mean age, 14.1 years +/- 2.4; age range, 8-18 years) underwent planimetric MR imaging, 2D VE MR imaging, and 3D VE MR imaging for TV and PV flow evaluation. For evaluation of diastolic function, PV and TV flow were summated. Data were analyzed by using linear regression analysis, paired and unpaired t testing, and Bland-Altman plots. RESULTS: Strong correlations between the 2D VE MR and 3D VE MR measurements of PV flow (for forward flow: r = 0.87, P < .01; for backward flow: r = 0.97, P < .01) were observed. With PV effective flow as a reference, 3D TV effective flow measurements were more accurate than 2D TV effective flow measurements: In patients, the mean 2D TV effective flow versus 2D PV effective flow difference was 17.6 mL +/- 11 (P < .001), and the mean 3D TV effective flow versus 3D PV effective flow difference was -1.2 mL +/- 4.7 (P = .22). Diastolic functional impairment in patients could be detected at 3D VE MR imaging diastolic assessment. CONCLUSION: Three-dimensional VE MR imaging is accurate for PV flow assessment and is more accurate than 2D VE MR imaging for TV flow evaluation. Assessment of diastolic function with 3D VE MR imaging can facilitate ongoing research of diastolic dysfunction in patients with a corrected TOF.

Assessment of intraventricular time differences in healthy children using two-dimensional speckle-tracking echocardiography.

BACKGROUND: Parameters describing intraventricular time differences are increasingly assessed in both adults and children. However, to appreciate the implications of these parameters in children, knowledge of the applicability of adult techniques in children is essential. Hence, the aim of this study was to assess the applicability of speckle-tracking strain-derived parameters in children, paying special attention to age and heart rate dependency. METHODS: One hundred eighty-three healthy subjects (aged 0-19 years) were included. Left ventricular global peak strain, time to global peak strain, and parameters describing intraventricular time differences were assessed using speckle-tracking strain imaging in the apical two-chamber, three-chamber, and four-chamber views (longitudinal strain) and the parasternal short-axis view (radial and circumferential strain). Parameters describing intraventricular time differences included the standard deviation of time to peak strain and differences in time to peak strain between two specified segments. Age and heart rate dependency were evaluated using regression analysis, and intraobserver and interobserver variability were tested. RESULTS: Acquisition and analysis of longitudinal six-segment time-strain curves was successful in 94.8% of subjects and radial and circumferential time-strain curves in 89.5%. No clinically significant linear relation was observed between age or heart rate and parameters describing intraventricular time differences. The coefficient of variation of time to global peak strain parameters was <10, while it was >10 for parameters describing intraventricular time differences. CONCLUSIONS: The feasibility of speckle-tracking strain analysis in children is relatively good. Furthermore, no linear relation was observed between age or heart rate and parameters describing intraventricular time differences. However, the limited reproducibility of some parameters describing intraventricular time differences will confine their applicability in clinical practice.

Disparity in right vs left ventricular recovery during follow-up after ventricular septal defect correction in children.

OBJECTIVES: Long-term prognosis after ventricular septal defect (VSD) correction in childhood is excellent. Nevertheless, decreased biventricular systolic performance has been described immediately following VSD surgery in children. In an effort to better understand this decrease and its time-course, we characterized biventricular systolic performance following VSD closure in paediatric patients up to 20 months postoperatively. METHODS: Thirty-nine children undergoing VSD surgery and 22 age-matched controls were included for echocardiographic follow-up of left (LV) and right ventricular (RV) systolic performance. LV fractional shortening and tricuspid annular plane systolic excursion (TAPSE) were assessed. Additionally, tissue Doppler imaging measurements were obtained at the basal LV lateral wall and RV free wall to assess both LV and RV systolic (S(')) performance. Studies were performed preoperatively, 1 day postoperatively, at discharge and 3-20 months postoperatively at medium-term follow-up. RESULTS: After an initial decrease in biventricular systolic performance, a significant recovery was observed within the first year after VSD surgery. After a medium-term follow-up of 8.4 ± 5.3 months, LV systolic performance parameters were normalized, while RV systolic performance parameters remained impaired in patients vs controls (TAPSE: 12.5 ± 1.2 vs 18.5 ± 3.2 mm, RV S('):8.9 ± 1.3 vs 12.5 ± 2.2 cm/s). CONCLUSIONS: Within the first year after VSD correction, LV systolic performance had normalized, while RV systolic performance remained significantly impaired up to 20 months after VSD closure. Both detrimental effects of open heart surgery with cardiopulmonary bypass and preoperative alterations may add to the observed postoperative impairment of specifically RV performance.

Enhanced characterization of ventricular performance after coarctation repair in neonates and young children.

BACKGROUND: Within the group of patients undergoing coarctectomy today, two subgroups can be identified: neonates with a critical coarctation and nonneonatal patients. We hypothesize that patients who have to undergo repair in the neonatal period will have more persistent impairment of ventricular performance postoperatively. Accordingly, we aimed to characterize biventricular performance after coarctectomy in neonatal and nonneonatal patients. METHODS: Children (aged 0 to 17 years) undergoing a coarctectomy were prospectively included and classified as neonatal (<1 month old) or nonneonatal patients. Age-matched controls were included for each measurement occasion. To evaluate left (LV) and right ventricular (RV) performance, fractional shortening, peak systolic (S') and early diastolic (E') tissue Doppler imaging velocities, and E/E' were assessed preoperatively, at discharge, and 1 year postoperatively (11.4 ± 8.3 months). RESULTS: In neonatal (n = 18) and nonneonatal (n = 19) patients LV performance significantly improved within the first postoperative year. Yet 1 year postoperatively, LV S' was still lower in neonatal patients vs controls (4.8 ± 1.1 vs 6.1 ± 1.6 cm/s; p = 0.036), whereas comparable results were observed in nonneonatal patients and controls. One year postoperatively, LV diastolic performance was impaired in neonatal (LV E' 8.7 ± 3.1 vs 13.2 ± 3.9 cm/s, p = 0.005) and nonneonatal patients (LV E' 12.1 ± 3.5 vs 15.1 ± 2.4 cm/s, p = 0.008) vs controls. In RV performance variables, no differences were observed 1 year postoperatively between neonatal and nonneonatal patients and controls. CONCLUSIONS: In both subgroups, LV diastolic performance does not recover to normal values within the first postoperative year. However, LV systolic performance remains more persistently impaired in patients who have to undergo repair in the neonatal period vs nonneonatal repair.

Relation of prolonged tissue Doppler imaging-derived atrial conduction time to atrial arrhythmia in adult patients with congenital heart disease.

Atrial arrhythmia (AA) is common in adult patients with congenital heart disease (CHD). To enable the prevention of AA or its complications, timely identification of adult patients with CHD at risk of AA is crucial. Long total atrial activation times have been related to AA. Tissue Doppler imaging (TDI) permits noninvasive evaluation of the total atrial conduction time (PA-TDI duration). The present study evaluated the association between the PA-TDI duration and the development of AA in adult patients with CHD. A total of 223 adult patients with CHD were followed up for the occurrence of AA after PA-TDI duration assessment. The PA-TDI duration was defined as the interval from the onset of the P wave on the electrocardiogram to the peak of the A' wave at the lateral atrial wall on TDI tracings. Among the various clinical and echocardiographic parameters, the association between the PA-TDI duration and AA occurrence was investigated. The median follow-up was 39 months (interquartile range 21 to 57). A PA-TDI duration of ≥126 ms was associated with AA during follow-up (log-rank, p <0.001). On multivariate analysis, a PA-TDI duration >126 ms (hazard ratio 2.25, 95% confidence interval 1.21 to 4.19) and history of AA (hazard ratio 4.89, 95% confidence interval 2.75 to 8.71) were independently associated with the occurrence of AA. In conclusion, PA-TDI duration and a history of AA were independently associated with the occurrence of AA in adult patients with CHD. The PA-TDI duration is a useful tool to identify patients with CHD at risk of AA during follow-up.

Mild residual pulmonary stenosis in tetralogy of fallot reduces risk of pulmonary valve replacement.

BACKGROUND: Current surgical strategies that aim at preventing pulmonary regurgitation in patients with corrected tetralogy of Fallot (cToF) may result in a certain grade of residual pulmonary stenosis (PS). The clinical implications of a postoperative residual PS in cToF patients remain unclear. Pulmonary valve replacement (PVR) is frequently needed during follow-up of cToF patients. The aim of the current study was to determine the role of residual PS in the need for PVR during follow-up in cToF patients. METHODS: cToF patients were included if clinical follow-up after primary surgical correction had taken place for a minimum of 5 years. Patient characteristics, surgical factors, and postoperative factors were reviewed, with a special focus on the transpulmonic systolic gradient. Cox proportional hazards regression analysis was performed to identify predictors of PVR. RESULTS: Of 171 cToF patients, 71 (41.5%) underwent PVR after 24.2 years (interquartile range, 16.8-31.6 years). Year of birth, older age at corrective operation, and patch use significantly predicted PVR during follow-up. By contrast, a mild residual PS in cToF patients (peak systolic gradient, 15-30 mm Hg) independently reduced the risk of PVR, as compared with patients without PS (hazard ratio, 0.47; p=0.02) and with moderate-to-severe PS (hazard ratio, 0.35; p=0.01). CONCLUSIONS: In addition to the known risks factors for PVR, a postoperative mild residual PS reduces the risk of PVR during follow-up of cToF patients. This finding provides clinical evidence for a conservative PS relief during correction of ToF.

Tissue-velocity magnetic resonance imaging and tissue Doppler imaging to assess regional myocardial diastolic velocities at the right ventricle in corrected pediatric Tetralogy of Fallot patients.

OBJECTIVES: In patients with corrected Tetralogy of Fallot (cToF), pulmonary regurgitation and subsequent increased right ventricular (RV) end-diastolic volume are diastolic parameters related to adverse outcome. In addition, abnormalities of the RV outflow tract (RVOT) independently promote RV dilatation in cToF patients. Tissue Doppler imaging (TDI) and tissue-velocity magnetic resonance imaging (TV-MRI) enable quantitative assessment of regional diastolic performance by measuring myocardial velocities. Assessment of regional diastolic velocities of the RV may provide insight into the relation between RVOT dysfunction and RV dilatation in cToF patients. The aim of the study was to perform a direct comparison of TV-MRI against TDI to assess regional RV diastolic velocities in cToF patients and control subjects. In addition, the relationship between regional RV diastolic velocities and RV dilatation was investigated. MATERIALS AND METHODS: Thirty-four cToF patients (8-18 years) and 19 controls were studied. Early (E') and late (A') peak diastolic velocities and E'/A' ratio were assessed with TDI and TV-MRI at the RV free wall and at the RVOT. RV volumes and pulmonary regurgitation were quantified with planimetric and 3-dimensional flow MRI, respectively. RESULTS: Good correlation and agreement were observed between TDI and TV-MRI at both regions of the RV (RV free wall: E': r = 0.92, mean bias: 0.5 cm/s, A': r = 0.92, mean bias: 0.4 cm/s; RVOT: E': r = 0.92, mean bias: -0.3 cm/s, A': r = 0.95, mean bias: 0.03 cm/s). With both imaging techniques, regional RV diastolic velocities were significantly reduced in cToF patients. The E'/A' ratio at the RVOT (assessed with both TDI and TV-MRI) was related to RV end-diastolic volume, even after correction for pulmonary regurgitation (TDI: P < 0.01, TV-MRI: P = 0.05). CONCLUSIONS: TDI and TV-MRI can be used interchangeably for the assessment of regional diastolic velocities and performance of the RV in cToF patients and in healthy controls. Regional diastolic velocities at the RVOT are reduced in cToF patients as compared with controls. In addition to pulmonary regurgitation, impaired diastolic performance at the RVOT is independently related to RV dilatation.

Relationship between temporal sequence of right ventricular deformation and right ventricular performance in patients with corrected tetralogy of Fallot.

OBJECTIVE: Right ventricular (RV) dysfunction is common in patients with corrected tetralogy of Fallot (cToF). Abnormalities in the temporal pattern of RV mechanical activation have been observed in patients with cToF, but the relationship with RV performance remains unclear. This study characterises RV performance and the temporal sequence of RV deformation in patients with cToF and healthy controls. STUDY DESIGN: 37 patients with cToF were compared with 18 controls. Using two-dimensional speckle tracking analysis, global RV strain was assessed. In addition, time to peak strain and the time difference between RV inlet and RV outlet (RV time delay) was assessed. MAIN OUTCOME MEASURE: The relation between RV performance and RV time delay was assessed with linear regression analysis. Results RV strain was reduced in patients compared with controls (-20.9 ± 4.3% vs -30.7 ± 3.4%, p<0.001). Time to peak strain at the RV inlet showed no differences between patients with cToF and controls (406 ± 55 ms vs 405 ± 67 ms, p=0.429), whereas time to peak strain at the RV outlet was significantly delayed in patients with cToF (339 ± 75 ms vs 262 ± 85 ms, p=0.003). Consequently, RV time delay was significantly shorter in patients with cToF than in controls (66 ± 48 ms vs 143 ± 53 ms, p<0.001). A close relation between RV time delay and RV strain was observed (r=-0.70, p<0.001). CONCLUSION: In patients with cToF, RV outlet deformation is delayed, causing a reduction in RV time delay which is significantly related to impairment in RV performance.

Real-time three-dimensional echocardiography: segmental analysis of the right ventricle in patients with repaired tetralogy of fallot.

BACKGROUND: The accurate assessment of right ventricular (RV) function and dimensions has important prognostic implications in patients with repaired tetralogy of Fallot (ToF). Three-dimensional imaging is the preferred methodology to evaluate RV function. Novel postprocessing software applications to evaluate three-dimensional data have provided insight into RV function and dimensions by analyzing the various RV components (inlet, apical trabecular, outlet). The aim of this study was to characterize regional RV function and dimensions with real-time three-dimensional echocardiography (RT3DE) in patients with repaired ToF. METHODS: Forty-one patients with repaired ToF (age range, 8-18 years) and 20 control subjects were enrolled. Global and segmental RV volumes and ejection fraction (EF) were assessed with RT3DE and compared between patients with repaired ToF and controls. RESULTS: Segmental analysis on RT3DE demonstrated that the apical trabecular region was the most remodeled RV component in patients with repaired ToF, with significantly increased end-diastolic volume and end-systolic volume compared with controls (59 ± 19 vs 41 ± 16 mL and 36 ± 13 vs 24 ± 8 mL, respectively; P = .001 for both). However, EF was preserved at that region. In contrast, EF was reduced at the RV inlet (53 ± 6% vs 58 ± 7%, P = .003) and outlet (44 ± 16% vs 52 ± 10% (P = .032). CONCLUSIONS: Patients with repaired ToF show characteristic RV remodeling as assessed with RT3DE. At the apical trabecular region, the largest volumes were observed compared with control patients, whereas EF at the inlet and outlet components was significantly impaired. RT3DE may facilitate future studies of segmental RV volumes and function in patients with repaired ToF.