Flow Dynamics in Children With Bicuspid Aortic Valve: A Blood Speckle Tracking Study.

OBJECTIVE: Bicuspid aortic valve (BAV) is associated with progressive aortic dilation. Although the etiology is complex, altered flow dynamics is thought to play an important role. Blood speckle tracking (BST) allows for visualization and quantification of complex flow, which could be useful in identifying patients at risk of root dilation and could aid in surgical planning. The aims of this study were to assess and quantify flow in the aortic root and left ventricle using BST in children with bicuspid aortic valves. METHODS AND RESULTS: A total of 38 children <10 y of age were included (24 controls, 14 with BAV). Flow dynamics were examined using BST in the aortic root and left ventricle. Children with BAV had altered systolic flow patterns in the aortic root and higher aortic root average vorticity (25.9 [23.4-29.2] Hz vs. 17.8 [9.0-26.2] Hz, p < 0.05), vector complexity (0.17 [0.14-0.31] vs. 0.05 [0.02-0.13], p < 0.01) and rate of energy loss (7.9 [4.9-12.1] mW/m vs. 2.7 [1.2-7.4] mW/m, p = 0.01). Left ventricular average diastolic vorticity (20.9 ± 5.8 Hz vs. 11.4 ± 5.2 Hz, p < 0.01), kinetic energy (0.11 ± 0.05 J/m vs. 0.04 ± 0.02 J/m, p < 0.01), vector complexity (0.38 ± 0.1 vs. 0.23 ± 0.1, p < 0.01) and rate of energy loss (11.1 ± 4.8 mW/m vs. 2.7 ± 1.9 mW/m, p < 0.01) were higher in children with BAV. CONCLUSION: Children with BAV exhibit altered flow dynamics in the aortic root and left ventricle in the absence of significant aortic root dilation. This may represent a substrate and potential predictor for future dilation and diastolic dysfunction.

Advanced Imaging Technologies for Assessing Tetralogy of Fallot: Insights Into Flow Dynamics.

Tetralogy of Fallot is the most common cyanotic congenital heart defect requiring surgical repair. Although surgical interventions have significantly reduced mortality, postrepair complications, such as pulmonary valve regurgitation and stenosis, may lead to adverse outcomes, including right ventricular dysfunction and increased risks of morbidity and mortality. This review explores the potential of advanced imaging technologies, including 4-dimensional-flow magnetic resonance imaging and high-frame-rate echocardiography, in providing valuable insights into blood flow dynamics and energy parameters. Quantitative measures, such as energy loss and vorticity, along with qualitative flow analysis, can provide additional insights into adverse haemodynamics at a potentially earlier and more reversible stage. Furthermore, personalized patient-specific information from these imaging modalities aids in guiding treatment decisions and monitoring postoperative interventions effectively. By characterizing flow patterns, these advanced imaging techniques hold great promise in improving the assessment and management of tetralogy of Fallot, providing tailored insights. However, further research and longitudinal studies are required to fully establish their clinical utility and potential impact on patient care.

La tétralogie de Fallot (TF) est la malformation cardiaque congénitale cyanogène la plus fréquente et elle requiert une correction chirurgicale. Bien que les interventions chirurgicales aient permis de diminuer considérablement la mortalité liée à la TF, des complications après ces interventions, comme la régurgitation de la valve pulmonaire (RP) ou la sténose, peuvent entraîner des issues cliniques défavorables, y compris la dysfonction ventriculaire droite et l'augmentation des risques de morbidité et de mortalité. Dans le présent article de synthèse, nous explorons le potentiel des techniques d'imagerie de pointe, y compris l'IRM de flux 4D et l'échocardiographie ultrarapide, pour obtenir des renseignements utiles sur la dynamique du débit sanguin et les paramètres énergétiques. Des mesures quantitatives, comme la perte d'énergie et la vorticité, pourraient permettre de mieux comprendre les paramètres hémodynamiques défavorables à un stade plus précoce où ils pourraient être plus facilement réversibles. De plus, des renseignements personnalisés obtenus par ces modalités d'imagerie peuvent contribuer à orienter les décisions thérapeutiques et à surveiller les interventions postopératoires pour chaque patient. La caractérisation des profils de débit sanguin par ces techniques d'imagerie s'annonce prometteuse pour améliorer l'évaluation et la prise en charge de la TF grâce à des renseignements personnalisés. Toutefois, des recherches supplémentaires et des études longitudinales devront être menées pour statuer sur leur utilité clinique et leurs répercussions possibles sur les soins offerts aux patients.

Outcome of Antibody-Mediated Fetal Heart Disease With Standardized Anti-Inflammatory Transplacental Treatment.

Background Transplacental fetal treatment of immune-mediated fetal heart disease, including third-degree atrioventricular block (AVB III) and endocardial fibroelastosis, is controversial. Methods and Results To study the impact of routine transplacental fetal treatment, we reviewed 130 consecutive cases, including 108 with AVB III and 22 with other diagnoses (first-degree/second-degree atrioventricular block [n=10]; isolated endocardial fibroelastosis [n=9]; atrial bradycardia [n=3]). Dexamethasone was started at a median of 22.4 gestational weeks. Additional treatment for AVB III included the use of a ß-agonist (n=47) and intravenous immune globulin (n=34). Fetal, neonatal, and 1-year survival rates with AVB III were 95%, 93%, and 89%, respectively. Variables present at diagnosis that were associated with perinatal death included an atrial rate <90 beats per minute (odds ratio [OR], 258.4; 95% CI, 11.5-5798.9; P<0.001), endocardial fibroelastosis (OR, 28.9; 95% CI, 1.6-521.7; P<0.001), fetal hydrops (OR, 25.5; 95% CI, 4.4-145.3; P<0.001), ventricular dysfunction (OR, 7.6; 95% CI, 1.5-39.4; P=0.03), and a ventricular rate <45 beats per minute (OR, 12.9; 95% CI, 1.75-95.8; P=0.034). At a median follow-up of 5.9 years, 85 of 100 neonatal survivors were paced, and 1 required a heart transplant for dilated cardiomyopathy. Cotreatment with intravenous immune globulin was used in 16 of 22 fetuses with diagnoses other than AVB III. Neonatal and 1-year survival rates of this cohort were 100% and 95%, respectively. At a median age of 3.1 years, 5 of 21 children were paced, and all had normal ventricular function. Conclusions Our findings reveal a low risk of perinatal mortality and postnatal cardiomyopathy in fetuses that received transplacental dexamethasone±other treatment from the time of a new diagnosis of immune-mediated heart disease.

Pulmonary Hypertension in Children is Associated With Abnormal Flow Patterns in the Main Pulmonary Artery as Demonstrated by Blood Speckle Tracking.

Background: Paediatric pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance resulting in increased pulmonary artery (PA) and right ventricular pressure (RV). This is associated with disturbed flow dynamics in the PA and RV that are not well characterized. We aimed to compare flow dynamics in children with PAH compared with healthy controls using blood speckle tracking echocardiography. Methods: Patients <10 years of age with PAH and healthy controls were included. We examined flow dynamics in the main PA (MPA) and right ventricle based on acquisition blood speckle tracking images obtained from the RV and PA. Qualitative and quantitative analyses were performed. Results: Eighteen subjects were included in each group. A diastolic vortex in the MPA was identified in 16 of the patients with PAH, but not in controls. Significantly higher MPA systolic (4.84 vs 2.42 mW/m; P = 0.01) and diastolic (0.69 vs 0.14 mW/m; P = 0.01) energy loss, as well as increased vector complexity (systole: 0.21 vs 0.04, P = 0.003; diastole: 0.13 vs 0.05, P = 0.04) and diastolic vorticity (15.2 vs 4.4 Hz; P = 0.001), were noted in PAH compared with controls. Conclusion: This study demonstrates the presence of abnormal flow patterns in the MPA with diastolic vortex formation in most patients with PAH. This diastolic vortex likely results from reflected waves from the distal pulmonary bed. Our data indicate that the diastolic vortex could potentially be used in the diagnosis of PAH. The clinical significance of the energy loss findings warrants further investigation in a larger cohort of patients with PAH.

Contexte: L'hypertension artérielle pulmonaire (HTAP) pédiatrique est caractérisée par une résistance vasculaire pulmonaire accrue qui donne lieu à une augmentation de la pression dans l'artère pulmonaire (AP) et dans le ventricule droit (VD). Ce phénomène s'accompagne de perturbations de la dynamique des débits dans l'AP et le VD, qui n'ont pas encore été bien caractérisées. Nous avons cherché à comparer la dynamique des débits chez des enfants atteints d'HTAP avec celle de témoins en bonne santé en utilisant l'échocardiographie de suivi des marqueurs acoustiques du sang. Méthodologie: Des patients de moins de 10 ans atteints d'HTAP et des témoins en bonne santé ont participé à l'étude. La dynamique des débits du tronc pulmonaire (TP) et du ventricule droit a été examinée à partir d'images de suivi des marqueurs acoustiques du sang de l'AP et du VD. Des analyses qualitatives et quantitatives ont aussi été réalisées. Résultats: Dix-huit sujets ont été inclus dans chacun des groupes. Un vortex diastolique du TP a été observé chez 16 des patients atteints d'HTAP, mais n'était présent chez aucun des témoins. Une perte d'énergie significativement plus élevée dans le TP a été notée pour la systole (4,84 vs 2,42 mW/m; P = 0,01) et la diastole (0,69 vs 0,14 mW/m; P = 0,01) des patients atteints d'HTAP; de plus, une complexité vectorielle accrue (systole : 0,21 vs 0,04, P = 0,003; diastole : 0,13 vs 0,05, P = 0,04) et une vorticité diastolique accrue (15,2 vs 4,4 Hz; P = 0,001) ont été notées chez les patients atteints d'HTAP comparativement aux témoins. Conclusion: Notre étude fait état d'un profil circulatoire anormal caractérisé par la formation d'un vortex diastolique dans le TP chez la plupart des patients atteints d'HTAP. Ce vortex découle probablement d'ondes réfléchies du lit pulmonaire distal. Les données que nous avons obtenues indiquent que le vortex diastolique pourrait possiblement être utilisé dans le diagnostic de l'HTAP. Par contre, la signification clinique des résultats concernant la perte d'énergie nécessite d'autres études auprès d'une cohorte plus importante de patients atteints d'HTAP.

Prenatal Diagnosis of Vascular Ring: Evaluation of Fetal Diagnosis and Postnatal Outcomes.

BACKGROUND: The impact of fetal echocardiography on the diagnosis and outcomes of vascular ring has not been well examined. We hypothesized that prenatal detection of vascular ring has improved over time and that prenatal diagnosis of vascular ring is associated with earlier intervention and favorable outcomes. METHODS: This is a single-center, retrospective study of the evolution and outcomes of prenatal diagnosis of vascular ring from 2000 to 2020. We compared clinical presentation, timing of surgical intervention, and outcomes between the prenatally and postnatally diagnosed cases during the same study period. RESULTS: A total of 170 patients were included: 50 with prenatal and 120 with postnatal diagnosis of vascular ring. Prenatal diagnoses included 42 patients (84%) with right aortic arch (RAA), aberrant left subclavian artery (ALSCA), and a left-sided ductus arteriosus and eight (16%) patients with double aortic arch (DAA). The postnatal cohort consisted mainly of 90 patients (75%) with DAA and 22 (18%) with RAA-ALSCA. None of the postnatally diagnosed cases had undergone a fetal echocardiogram. Numbers (percentage) of prenatally diagnosed cases of vascular ring compared with the postnatal cases improved from 4/31 (13%), to 10/29 (34%), to 14/25 (56%), and to 22/35 (69%), respectively, during 2000-2005, 2005-10, 2010-15, and 2015-20 (P = .032). Vascular ring was an isolated abnormality in 84% and 85% of the prenatal and postnatal cohorts, respectively. Compared with the prenatal cohort, postnatally diagnosed patients with an isolated vascular ring were more frequently symptomatic (66% vs 48%, P < .03) and underwent cross-sectional imaging (69% vs 44%, P = .009) and surgery more frequently (79% vs 48%, P = .003). Surgery was performed at a later patient age (18 [2-147] months vs 4.8 [0.5-42] months, P = .01) and was more often associated with residual symptoms (27/81 [33%] vs 1/20 [5%], P = .01) in the postnatal cohort than in the prenatal cohort. CONCLUSIONS: The diagnosis of vascular ring by fetal echocardiography has improved over time. A significantly higher incidence of RAA-ALSCA in the prenatal compared with the postnatal cohort suggests that patients with this form of vascular ring often do not present to medical attention with significant symptoms postnatally. Prenatal diagnosis of vascular ring was associated with a lower incidence of symptoms, less frequent use of cross-sectional imaging, earlier age at surgical intervention, and lower likelihood of residual symptoms.

Right Ventricular Flow Dynamics in Dilated Right Ventricles: Energy Loss Estimation Based on Blood Speckle Tracking Echocardiography-A Pilot Study in Children.

Using blood speckle tracking (BST) based on high-frame-rate echocardiography (HFRE), we compared right ventricle (RV) flow dynamics in children with atrial septal defects (ASDs) and repaired tetralogy of Fallot (rTOF). Fifty-seven children with rTOF with severe pulmonary insufficiency (PI) (n = 21), large ASDs (n = 11) and healthy controls (CTL, n = 25) were included. Using a flow phantom, we studied the effects of imaging plane and smoothing parameters on 2-D energy loss (EL). RV diastolic EL was similar in ASD and rTOF, but both were greater than in CTL. Locations of high EL were similar in all groups in systole, occurring in the RV outflow tract and around the tricuspid valve leaflets in early diastole. An additional apical early diastolic area of EL was noted in rTOF, corresponding to colliding tricuspid inflow and PI. The flow phantom revealed that EL varied with imaging plane and smoothing settings but that the EL trend was preserved if kept consistent.

Effect of anthracycline therapy on myocardial function and markers of fibrotic remodelling in childhood cancer survivors.

AIMS: Anthracyclines are a cornerstone of paediatric cancer treatment. We aimed to quantify myocardial cardiac magnetic resonance (CMR) native T1 (NT1) and extracellular volume fraction (ECV) as markers of fibrosis in a cohort of childhood cancer survivors (CCS). METHODS AND RESULTS: A cohort of CCS in remission underwent CMR T1 mapping. Diastolic function was assessed by echocardiography. Results were compared to a cohort of normal controls of similar age and gender. Fifty-five CCS and 46 controls were included. Both groups had similar mean left ventricular (LV) NT1 values (999 ± 36 vs. 1007 ± 32 ms, P = 0.27); ECV was higher (25.6 ± 6.9 vs. 20.7 ± 2.4%, P = 0.003) and intracellular mass was lower (37.5 ± 8.4 vs. 43.3 ± 9.9g/m2, P = 0.02) in CCS. The CCS group had lower LV ejection fraction (EF) and LV mass index with otherwise normal diastolic function in all but one patient. The proportion of subjects with elevated ECV compared to controls did not differ between subgroups with normal or reduced LV EF (22% vs. 28%; P = 0.13) and no correlations were found between LVEF and ECV. While average values remained within normal range, mitral E/E' (6.6 ± 1.6 vs. 5.9 ± 0.9, P = 0.02) was higher in CCS. Neither NT1 nor ECV correlated with diastolic function indices or cumulative anthracycline dose. CONCLUSIONS: There is evidence for mild diffuse extracellular volume expansion in some asymptomatic CCS; myocyte loss could be part of the mechanism, accompanied by subtle changes in systolic and diastolic function. These findings suggest mild myocardial damage and remodelling after anthracycline treatment in some CCS which requires continued monitoring.

Novel Z Scores to Correct Biases Due to Ventricular Volume Indexing to Body Surface Area in Adolescents and Young Adults.

BACKGROUND: Reference values for cardiac magnetic resonance imaging (cMRI) in children and young adults are scarce. This leads to risk stratification of patients with congenital heart diseases being based on volumes indexed to body surface area (BSA). We aimed to produce cMRI Z score equations for ventricular volumes in children and young adults and to test whether indexing to BSA resulted in an incorrect assessment of ventricular dilation according to sex, body composition, and growth. METHODS: We retrospectively included 372 subjects aged < 26 years with either normal hearts or conditions with no impact on ventricular volumes (reference group), and 205 subjects with repaired tetralogy of Fallot (TOF) aged < 26 years. We generated Z score equations by means of multivariable regression modelling. Right ventricular dilation was assessed with the use of Z scores and compared with indexing to BSA in TOF subjects. RESULTS: Ventricular volume Z scores were independent from age, sex, and anthropometric measurements, although volumes indexed to BSA showed significant residual association with sex and body size. In TOF subjects, indexing overestimated dilation in growing children and underestimated dilation in female compared with male subjects, and in overweight compared with lean subjects. CONCLUSIONS: Indexed ventricular volumes measured with cMRI did not completely adjust for body size and resulted in a differential error in the assessment of ventricular dilation according to sex and body size. Our proposed Z score equations solved this problem. Future studies should evaluate if ventricular volumes expressed as Z scores have a better prognostic value than volumes indexed to BSA.

Unique foetal diagnosis of aorto-pulmonary collaterals in right atrial isomerism.

Right atrial isomerism is associated with complex cardiac malformations, particularly single-ventricle lesions; right atrial isomerism is rarely associated with aorto-pulmonary collateral arteries. We report a foetal diagnosis of right atrial isomerism, with an unbalanced atrioventricular septal defect, pulmonary stenosis, total anomalous venous drainage, and significant aorto-pulmonary collaterals diagnosed at 22 weeks' gestation.

Progressive right ventricular outflow tract fibrosis after repair of tetralogy of Fallot.

OBJECTIVES: The objective of this study was to determine the evolution of fibrosis over time and its association with clinical status. METHODS: Children with repaired tetralogy of Fallot who had undergone at least two cardiac magnetic resonance examinations including T1 mapping at least 1 year apart were included. RESULTS: Thirty-seven patients (12.7 ± 2.6 years, 61% male) were included. Right ventricular free wall T1 increased (913 ± 208 versus 1023 ± 220 ms; p = 0.02). Baseline cardiac magnetic resonance parameters did not predict a change in imaging markers or exercise tolerance. The right ventricular free wall per cent change correlated with left ventricular T1% change (r = 0.51, p = 0.001) and right ventricular mass Z-score change (r = 0.51, p = 0.001). T1 in patients with late gadolinium enhancement did not differ from the rest. CONCLUSION: Increasing right ventricular free wall T1 indicates possible progressive fibrotic remodelling in the right ventricular outflow tract in this pilot study in children and adolescents with repaired tetralogy of Fallot. The value of T1 mapping both at baseline and during serial assessments will need to be investigated in larger cohorts with longer follow-up.

Preliminary Experience Using Motion Compensated CINE Magnetic Resonance Imaging to Visualise Fetal Congenital Heart Disease.

BACKGROUND: Recent advances in cardiovascular magnetic resonance (CMR) imaging have facilitated CINE imaging of the fetal heart. In this work, a preliminary investigation of the utility of multislice CINE CMR for assessing fetal congenital heart disease is performed and compared with echocardiography. METHODS AND RESULTS: Multislice CINE CMR and echocardiography images were acquired in 25 pregnant women wherein the fetus had a suspected congenital heart defect based on routine obstetric ultrasound. Pathognomonic images were identified for each subject for qualitative comparison of CMR and echocardiography. Quantitative comparison of CMR and echocardiography was then performed by 2 reviewers using a binary scoring of 9 fetal cardiac anatomic features (identifiable/not-identifiable). Pathognomonic images demonstrated the ability of CMR to visualize a variety of congenital heart defects. Overall CMR was able to identify the majority of the 9 assessed fetal cardiac anatomic features (reviewer 1, 7.1±2.1; reviewer 2, 6.7±2.3). Although both reviewers identified more anatomic features with echocardiography (reviewer 1, 7.8±2.3; reviewer 2, 7.5±2.4; P=0.01), combining information from both modalities enabled identification of additional anatomic features across subjects (reviewer 1, 8.4±1.3; reviewer 2, 8.4±1.2). The primary limiting factor for CMR was inadequate coverage of the fetal cardiac anatomy or noncontiguous slices because of gross fetal movement. CONCLUSIONS: CINE CMR enables visualization of fetal congenital heart disease. This work demonstrates the potential of CMR for diagnosing congenital heart disease in utero in conjunction with echocardiography during late gestation.

Recent Advances and Trends in Pediatric Cardiac Imaging.

Cardiac imaging is central to today's pediatric cardiology practice not only to diagnose structural congenital defects and delineate cardiac and extracardiac anatomy but also for determining the hemodynamic impact of the structural defects and acquired pediatric diseases. Not so long ago, clinicians had to heavily rely on angiography as the main cardiac imaging modality to visualize the heart. Particularly, the development of echocardiography in the 1970s and 1980s together with the development of magnetic resonance imaging (MRI) and computed tomography (CT) resulted in a non-invasive diagnostic revolution with diagnostic catheterization becoming obsolete apart for very specific indications. The continuous improvements in non-invasive imaging modalities allow an unprecedented level of understanding of cardiac morphology and function. Over the last few years, the specific roles of the three imaging modalities and their complementary roles in diagnosis and treatment have become well established resulting in a multimodality approach to specific congenital lesions. Recently, multimodality guidelines were published for postoperative tetralogy of Fallot and patients with transposition of the great arteries (Cohen et al. J Am Soc Echocardiogr. 2016;29(7):571-621, Valente et al. J Am Soc Echocardiogr. 2014;27(2):111-41). In this paper, we aim to highlight some of the most significant advances and highlight some emerging trends in pediatric cardiac imaging.

Percutaneous Fetal Atrial Balloon Septoplasty for Simple Transposition of the Great Arteries With an Intact Atrial Septum.

We report the first ultrasonographically guided percutaneous balloon atrial septoplasty (BAS), to our knowledge, in a fetus with transposition of the great arteries and an intact ventricular and atrial septum (37 + 2 weeks). After vaginal delivery at 38 weeks, the infant had an elective septostomy (day 1) and an arterial switch procedure (day 7), with an uneventful postoperative course. For centres with experience in fetal cardiac interventions, fetal BAS is a superior management option compared with the alternatives for this high-risk physiology.

The continuing challenge of evaluating diastolic function by echocardiography in children: developing concepts and newer modalities.

PURPOSE OF REVIEW: Assessment of diastolic function by echocardiography is challenging but important. Left ventricular filling has been more extensively studied than the right ventricle, and predominantly in adult populations. Although multiple parameters exist to assess diastolic function, they all have limitations, including load and heart rate dependency, which make assessment of diastolic function particularly challenging. The purpose of this article is to review evolving concepts and modalities for echo assessment of diastolic function in children. RECENT FINDINGS: The paradigm whereby diastolic dysfunction severity progresses in a staged fashion from impaired relaxation to increasing ventricular stiffness, may not apply in children. In addition, previous adult guidelines are not readily applicable to children with cardiomyopathy and the applicability of the newly revised adult guidelines needs to be evaluated in children. It is unlikely that any one single echocardiographic diastolic parameter will adequately reflect diastolic function. Hence, parameters derived from atrioventricular valve inflow, pulmonary venous, and tissue Doppler need to be integrated. Newer modalities such as diastolic strain rate and rotation mechanics may be useful as more sensitive markers of early ventricular dysfunction but have important limitations and require more evaluation before routine use in practice. Assessment of systolic-diastolic coupling may enhance assessment of diastolic function. SUMMARY: Diastolic function impacts outcomes and should be part of routine echocardiographic assessment of function. An integrative approach combining different parameters, possibly with contribution of newer modalities in the future, is required.

Characteristics of premature ventricular contractions in healthy children and their impact on left ventricular function.

BACKGROUND: There are few data regarding the characteristics of premature ventricular contractions (PVCs) in healthy children and their impact on left ventricular (LV) function. OBJECTIVE: The purpose of this study was to assess the prevalence of LV systolic dysfunction in children with frequent PVCs (≥10%) and determine whether it is associated with PVC characteristics (e.g., proportion, coupling interval, width, and/or morphology). METHODS: We conducted a single-center cohort study of children with structurally normal hearts and PVC burden ≥10% by 24-hour Holter monitoring performed between 2008 and 2012. Clinical, arrhythmic, and echocardiographic data were reviewed at baseline and during follow-up. RESULTS: A total of 47 children (22 female [47%], mean age 8.2 ± 6.5 years) had a mean PVC burden of 20.9 ± 11.9% at baseline. The PVC coupling interval averaged 430 ± 110 ms, with a PVC width of 118 ± 27 ms. PVCs were monomorphic in 44 patients (94%). Although no patient had severe cardiomyopathy, 7 (15%) had reduced shortening fraction (Z-score <-2). A strong association was observed between PVC coupling interval and LV shortening fraction Z-score <-2.0 (area under the curve 0.95 ± 0.03, P <.001). A cutoff value <365 ms yielded the greatest discriminatory ability (Youden J-statistic 0.72, sensitivity 85.7%, specificity 86.5%). PVC proportion, width, and morphology were not significantly associated with LV shortening fraction. During 4.0 ± 2.8 years of follow-up, the PVC burden decreased from a median of 18% to 1.5% (P<.001). CONCLUSION: PVCs in children with structurally normal hearts are associated with a relatively benign course, with spontaneous resolution in most children. Mild LV systolic dysfunction, observed in 15%, is strongly correlated with a shorter coupling interval (<365 ms).

Exercise-induced ventricular re-polarisation changes in moderate congenital aortic valve stenosis.

UNLABELLED: Introduction Pressure overload increases in patients with moderate aortic valvular stenosis during exercise. In the absence of symptoms, it remains difficult, however, to discriminate patients for surgery based only on pressure overload. Other parameters, such as the dispersion of ventricular re-polarisation (d-QT), which reportedly increases with the transvalvular pressure gradient, have not been fully studied in this condition. OBJECTIVE: To determine the pattern of QT and d-QT response to exercise testing in children with moderate aortic valve stenosis in order to evaluate the impact of pressure overload from an electrophysiological perspective. Materials and methods In all, 15 patients were compared with 15 controls paired for age (14.8±2.5 versus 14.2±1.5 years old) and gender (66.7% male). All the patients underwent exercise stress testing with 12-lead electrocardiograph recording. QT was measured from the onset of QRS to the apex (QTa) at rest, at peak exercise, and at 1 and 3 minutes upon recovery. QT was corrected using the Fridericia equation, and d-QT was calculated. RESULTS: Resting QTc was similar among the study groups, but increased significantly in study patients compared with the control group at maximal effort (p=0.004) and after 1 (p<0.001) and 3 (p<0.001) minutes of recovery. A significant association was identified between groups for d-QT (p=0.034), and post-hoc tests revealed a significant difference only at rest (p=0.001). CONCLUSIONS: Ventricular re-polarisation abnormalities can be unmasked and highlighted by the assessment of electrical re-polarisation during exercise challenge in patients with asymptomatic moderate aortic valve stenosis. Using QT response to exercise could be beneficial for better optimisation of risk stratification in these patients.

Timing of Dynamic NT-proBNP and hs-cTnT Response to Exercise Challenge in Asymptomatic Children with Moderate Aortic Valve Regurgitation or Moderate Aortic Valve Stenosis.

Patients with congenital aortic valve stenosis (AVS) can remain asymptomatic but may develop progressive and often underestimated exercise intolerance. The risk of increased left ventricular (LV) wall stress, irreversible myocardial fibrosis and sudden death in untreated patients warrants earlier intervention. The timing for curative therapy for severe AVS is clear, but optimal timing for moderate stenosis (modAS) is unknown. AVS often coexists with aortic regurgitation, which adds a volume overload to an already pressure-overloaded LV, adding an additional challenge to the estimation of disease severity. We investigated the possible value of N-terminal pro-brain natriuretic peptide (NT-proBNP) and high-sensitivity cardiac troponin T (hs-cTnT) upon treadmill exercise challenge in children with asymptomatic modAS versus moderate regurgitation (modAR). The aim was to determine optimal timing of peak biochemical response. Blood samples were obtained at rest, and then at 20, 40 and 60 min after peak exercise comparing modAS and modAR to healthy controls. Exercise performance was equivalent in all groups, with no difference for biomarker levels at rest. The increase in NT-proBNP was significant in modAR at 40 min (99.2 ± 48.6 ng/L; p = 0.04) and 60 min into recovery (100.0 ± 53.7 ng/L; p = 0.01), but not in modAS. The increase in hs-cTnT was significant only at 60 min into recovery for modAS and modAR. NT-proBNP and hs-cTnT following exercise challenge are possible discriminant biomarkers of modAR from modAS and controls at 60 min into recovery despite comparable exercise performance. This offers a promising avenue for future stratification of aortic valve disease and optimal timing of intervention.