Biventricular responses to exercise and their relation to cardiorespiratory fitness in pediatric pulmonary hypertension.

Despite exercise intolerance being predictive of outcomes in pulmonary arterial hypertension (PAH), its underlying cardiac mechanisms are not well described. The aim of the study was to explore the biventricular response to exercise and its associations with cardiorespiratory fitness in children with PAH. Participants underwent incremental cardiopulmonary exercise testing and simultaneous exercise echocardiography on a recumbent cycle ergometer. Linear mixed models were used to assess cardiac function variance and associations between cardiac and metabolic parameters during exercise. Eleven participants were included with a mean age of 13.4 ± 2.9 yr old. Right ventricle (RV) systolic pressure (RVsp) increased from a mean of 59 ± 25 mmHg at rest to 130 ± 40 mmHg at peak exercise (P < 0.001), whereas RV fractional area change (RV-FAC) and RV-free wall longitudinal strain (RVFW-Sl) worsened (35.2 vs. 27%, P = 0.09 and -16.6 vs. -14.6%, P = 0.1, respectively). At low- and moderate-intensity exercise, RVsp was positively associated with stroke volume and O2 pulse (P < 0.1). At high-intensity exercise, RV-FAC, RVFW-Sl, and left ventricular longitudinal strain were positively associated with oxygen uptake and O2 pulse (P < 0.1), whereas stroke volume decreased toward peak (P = 0.04). In children with PAH, the increase of pulmonary pressure alone does not limit peak exercise, but rather the concomitant reduced RV functional reserve, resulting in RV to pulmonary artery (RV-PA) uncoupling, worsening of interventricular interaction and LV dysfunction. A better mechanistic understanding of PAH exercise physiopathology can inform stress testing and cardiac rehabilitation in this population.NEW & NOTEWORTHY In children with pulmonary arterial hypertension, there is a marked increase in pulmonary artery pressure during physical activity, but this is not the underlying mechanism that limits exercise. Instead, right ventricle-to-pulmonary artery uncoupling occurs at the transition from moderate to high-intensity exercise and correlates with lower peak oxygen uptake. This highlights the more complex underlying pathological responses and the need for multiparametric assessment of cardiac function reserve in these patients when feasible.

Abnormal vascular thickness and stiffness in young adults with type 1 diabetes: new insights from cutting-edge ultrasound modalities.

BACKGROUND: Cardiovascular disease (CVD) remains the leading cause of morbidity and mortality in patients with Type 1 Diabetes (T1D). Early markers of CVD include increased carotid intima-media thickness (CIMT) and pulse wave velocity (PWV), but these existing ultrasound technologies show limited spatial and temporal resolution in young adults. The purpose of this study is to evaluate the utility of high-resolution ultrasound modalities, including high frequency ultrasound CIMT (hfCIMT) and ultrafast ultrasound PWV (ufPWV), in young adults with Type 1 Diabetes. METHODS: This is a prospective single-center observational cohort study including 39 participants with T1D and 25 age and sex matched controls. All participants underwent hfCIMT and ufPWV measurements. hfCIMT and ufPWV measures of T1D were compared with controls and associations with age, sex, BMI, A1c, blood pressure, and lipids were studied. RESULTS: Mean age was 24.1 years old in both groups. T1D had a greater body mass index (27.7 [5.7] vs 23.1 [3.2] kg/m2), LDL Cholesterol, and estimated GFR, and had a mean A1c of 7.4 [1.0] % (57 mmol/mol) and diabetes duration of 16.1 [3.7] years with 56% using insulin pumps. In T1D, hfCIMT was significantly increased as compared to controls (0.435 ± 0.06 mm vs 0.379 ± 0.06 mm respectively, p < 0.01). ufPWV measures were significantly increased in T1D (systolic foot PWV: 5.29 ± 0.23 m/s vs 5.50 ± 0.37 m/s, p < 0.01; dicrotic notch PWV = 7.54 ± 0.46 m/s vs 7.92 ± 0.41 m/s, p < 0.01). Further, there was an impact of A1c-measured glycemia on hfCIMT, but this relationship was not seen with ufPWV. No significant statistical correlations between hfCIMT and ufPWV measures in either T1D or healthy controls were observed. CONCLUSION: Young adults with T1D present with differences in arterial thickness and stiffness when compared with controls. Use of novel high-resolution ultrasound measures describe important relationships between early structural and vascular pathophysiologic changes and are promising tools to evaluate pre-clinical CVD risk in youth with T1D. TRIAL REGISTRATION: ISRCTN91419926.

Interatrial Communications: Prevalence and Subtypes in 12,385 Newborns-a Copenhagen Baby Heart Study.

The prevalence of interatrial communications in newborns, i.e., patent foramen ovale or atrial septal defect, was previously reported to be between 24 and 92%, but the area has been impeded by lack of a universal classification method. A recently published novel echocardiographic diagnostic algorithm for systematic classification of interatrial communications had inter-and intraobserver agreements superior to standard expert assessment. This study aimed to determine the prevalence of subtypes of interatrial communications on transthoracic echocardiography in newborns. Echocardiograms of newborns aged 0-30 days were prospectively collected in the population-based cohort study Copenhagen Baby Heart Study in 2017-2018 and analyzed according to the new diagnostic algorithm, classifying interatrial communications into three subtypes of patent foramen ovale and three subtypes of atrial septal defects. Echocardiograms from 15,801 newborns were analyzed; 3416 (21.6%) were excluded due to suboptimal image quality or severe structural heart disease (n = 3), leaving 12,385 newborns (aged 12 [interquartile range 8; 15] days, 48.2% female) included in the study. An interatrial communication was detected in 9766 (78.9%) newborns. According to the algorithm, 9029 (72.9%) had a patent foramen ovale, while 737 (6.0%) fulfilled criteria for an atrial septal defect, further divided into subtypes. An interatrial communication was seen on echocardiography in almost 80% of newborns aged 0-30 days. Patent foramen ovale was 12 times more frequent than atrial septal defects. The observed prevalence of atrial septal defects was higher than previously reported. Follow up studies could distinguish which interatrial communications require follow-up or intervention. ClinicalTrial.gov, NCT02753348, posted April 27, 2016, [ https://classic.clinicaltrials.gov/ct2/show/NCT02753348 ].

Impact of cardiopulmonary bypass on cerebrovascular autoregulation assessed by ultrafast ultrasound imaging.

Newborns with congenital heart disease undergoing cardiac surgery are at risk of neurodevelopmental impairment with limited understanding of the impact of intra-operative cardiopulmonary bypass (CPB), deep hypothermia and selective cerebral perfusion on the brain. We hypothesized that a novel ultrasound technique, ultrafast power Doppler (UPD), can assess variations of cerebral blood volume (CBV) in neonates undergoing cardiac surgery requiring CPB. UPD was performed before, during and after surgery in newborns with hypoplastic left heart syndrome undergoing a Norwood operation. We found that global CBV was not significantly different between patients and controls (P = 0.98) and between pre- and post-surgery (P = 0.62). UPD was able to monitor changes in CBV throughout surgery, revealing regional differences in CBV during hypothermia during which CBV correlated with CPB flow rate (R2  = 0.52, P = 0.021). Brain injury on post-operative magnetic resonance imaging was observed in patients with higher maximum variation in CBV. Our findings suggest that UPD can quantify global and regional brain perfusion variation during neonatal cardiac surgery with this first intra-operative application demonstrating an association between CBV and CPB flow rate, suggesting loss of autoregulation. Therefore, the measurement of CBV by UPD could enable optimization of cerebral perfusion during cardiac surgery in neonates. KEY POINTS: The impact of cardiopulmonary bypass (CPB) on the neonatal brain undergoing cardiac surgery is poorly understood. Ultrafast power Doppler (UPD) quantifies cerebral blood volume (CBV), a surrogate of brain perfusion. CBV varies throughout CPB surgery and is associated with variation of the bypass pump flow rate during deep hypothermia. Association between CBV and bypass pump flow rate suggests loss of cerebrovascular autoregulatory processes. Quantitative monitoring of cerebral perfusion by UPD could provide a direct parameter to optimize CPB flow rate.

Growth of targeted neonatal echocardiography in Chinese neonatal intensive care units: gaps in practice and training.

To evaluate clinical practice, neonatologists' attitudes, and the extent of training and accreditation regarding targeted neonatal echocardiography (TnEcho) among Chinese neonatologists. A web-based questionnaire was emailed to 331 neonatologists across China who completed training in subspecialty neonatology. The survey covered various aspects of TnEcho, including the characteristics of clinical practice, attitudes towards its usefulness, and perceived barriers to implementation and training methods. Survey response rate was 68.0% (225/331). Seventy-nine (35.1%) respondents stated that TnEcho was utilized in their NICUs. Most respondents reported the use of echocardiography to evaluate hemodynamic significance of the patent ductus arteriosus (PDA, 94.9%). The eyeballing technique was most used to evaluate left (82.3%) and right (77.2%) ventricular function. Most respondents (87.3-96.2%) positively valued the role of TnEcho in providing timely and longitudinal hemodynamic information to guide cardiovascular care. Access to TnEcho was more likely in centers with on-site pediatric cardiology service (p = .003), larger bed capacity (p = .004), or level IV status (p = .003). Lack of experienced practitioners with echocardiography expertise (88.9%) and accredited training programs (85.8%) was perceived to be the major barrier to implementation. Of concern, most practitioners with TnEcho skills received training in an informal manner through workshops (60.8%) or self-directed learning (54.4%). Conclusions: The use of TnEcho for longitudinal evaluation of infants with hemodynamic instability is growing within Chinese NICUs. There is an urgent need to develop standardized training programs and accreditation for TnEcho which are adapted to the Chinese context.

A novel algorithm for classification of interatrial communications within the oval fossa in the newborn.

BACKGROUND: An interatrial communication is present in most neonates. The majority are considered the "normal" patency of the oval foramen, while a minority are abnormal atrial septal defects. Differentiation between the two with transthoracic echocardiography may be challenging, and no generally accepted method of classification is presently available. We aimed to develop and determine the reliability of a new classification of interatrial communications in newborns. METHODS AND RESULTS: An algorithm was developed based on echocardiographic criteria from 495 newborns (median age 11[8;13] days, 51.5% females). The algorithm defines three main categories: patency of the oval foramen, atrial septal defect, and no interatrial communication as well as several subtypes. We found an interatrial communication in 414 (83.6%) newborns. Of these, 386 (93.2%) were categorised as patency of the oval foramen and 28 (6.8%) as atrial septal defects.Echocardiograms from another 50 newborns (median age 11[8;13] days, 36.0% female), reviewed by eight experts in paediatric echocardiography, were used to assess the inter- and intraobserver variation of classification of interatrial communications into patency of the oval foramen and atrial septal defect, with and without the use of the algorithm. Review with the algorithm gave a substantial interobserver agreement (kappa = 0.66), and an almost perfect intraobserver agreement (kappa = 0.82). Without the use of the algorithm, the interobserver agreement between experienced paediatric cardiologists was low (kappa = 0.20). CONCLUSION: A new algorithm for echocardiographic classification of interatrial communications in newborns produced almost perfect intraobserver and substantial interobserver agreement. The algorithm may prove useful in both research and clinical practice.

Comparison between Del Nido and conventional blood cardioplegia in pediatric open-heart surgery.

BACKGROUND: Del Nido cardioplegia (DNc) was designed for superior myocardial protection during cardiopulmonary bypass (CPB). We conducted a retrospective review to explore if DNc was associated with increase in systemic ventricle dysfunction (sVD) following pediatric CPB. METHODS AND RESULTS: This single-center, retrospective study included 1534 patients undergoing CPB between 2013 and 2016, 997 prior to center-wide conversion to DNc and 537 following. The primary outcome was new postoperative ≥moderate sVD by echocardiogram. Secondary outcomes included sVD of any severity and right ventricular dysfunction. Data was evaluated by interrupted time-series analysis. Groups had similar cardiac diagnoses and surgical complexity. Del Nido cardioplegia was associated with longer median (IQR) CPB [117 (84-158) vs 108 (81-154), p = 0.04], and aortic cross-clamp [83 (55-119) vs 76 (53-106), p = 0.03], and fewer cardioplegia doses [2 (1-2) vs 3 (2-4), p < 0.0001]. Mortality was similar in both groups. Frequency of sVD was unchanged following DNc, including predetermine subgroups (neonates, infants, and prolonged cross-clamp). Logistic regression showed a significant rise in right ventricular dysfunction (OR 5.886 [95% CI: 0.588, 11.185], p = 0.03) but similar slope. CONCLUSIONS: Use of DNc was not associated with increased in reported sVD, and provided similar myocardical protection to the systemic ventricle compared to conventional cardioplegia but may possibly impact right ventricular function. Studies evaluating quantitative systolic and diastolic function are needed.

Challenges in diagnosis and management of coarctation of the aorta.

PURPOSE OF REVIEW: Coarctation of the aorta remains a controversial topic with uncertainties in long-term outcomes. RECENT FINDINGS: Recent advances in fetal imaging including echocardiography and MRI offer novel opportunities for better detection and prediction of the need for neonatal intervention.New imaging techniques are providing novel insights about the impact of arch geometry and size on flow dynamics and pressure gradients. The importance of arch size rather than shape for optimal hemodynamics has been identified. Long-term outcome data suggest a significant increase in mortality risk in coarctation patients beyond the third decade when compared with the general population. Hypertension is highly prevalent not only in adult patients following repair of coarctation but also in normotensive patients presenting with LV diastolic dysfunction and adverse remodelling, indicating that abnormal vascular properties are important. Patients with coarctation undergoing neonatal repair are at risk for adverse neurodevelopmental outcomes and patients could benefit from timely neurocognitive evaluation and intervention. SUMMARY: Optimizing aortic arch size, prevention and aggressive treatment of hypertension and vascular stiffening are important to improve long-term outcomes.

Retraining an Artificial Intelligence Algorithm to Calculate Left Ventricular Ejection Fraction in Pediatrics.

OBJECTIVES: Identifying patients with low left ventricular ejection fraction (LVEF) and monitoring LVEF responses to treatment are important clinical goals. Can a deep-learning algorithm predict pediatric LVEF within clinically acceptable error? DESIGN: The study authors wanted to fine-tune an adult deep-learning algorithm to calculate LVEF in pediatric patients. A priori, their objective was to refine the algorithm to perform LVEF calculation with a mean absolute error (MAE) ≤5%. SETTING: A quaternary pediatric hospital PARTICIPANTS: A convenience sample (n = 321) of echocardiograms from newborns to 18 years old with normal cardiac anatomy or hemodynamically insignificant anomalies. Echocardiograms were chosen from a group of healthy controls with known normal LVEF (n = 267) and a dilated cardiomyopathy patient group with reduced LVEF (n = 54). INTERVENTIONS: The artificial intelligence model EchoNet-Dynamic was tested on this data set and then retrained, tested, and further validated to improve LVEF calculation. The gold standard value was LVEF calculated by clinical experts. MEASUREMENTS AND MAIN RESULTS: In a random subset of subjects (n = 40) not analyzed prior to selection of the final model, EchoNet-Dynamic calculated LVEF with a MAE of 8.39%, R2 = 0.47 without, and MAE 4.47%, R2 = 0.87 with fine-tuning. Bland-Altman analysis suggested that the model slightly underestimates LVEF (bias = -2.42%). The 95% limits of agreement between actual and calculated values were -12.32% to 7.47%. CONCLUSIONS: The fine-tuned model calculates LVEF in a range of pediatric patients within clinically acceptable error. Potential advantages include reducing operator error in LVEF calculation and supporting independent LVEF assessment by inexperienced users.

Cardiac Function and Ventricular Interactions in Persistent Pulmonary Hypertension of the Newborn.

OBJECTIVES: The aim of this study was to use a comprehensive imaging protocol to identify echocardiographic correlations of right and left ventricular size, function, and hemodynamics in neonates with persistent pulmonary hypertension of newborn and describe their relationship with key clinical variables. DESIGN: Retrospective case-control echocardiography-based study of persistent pulmonary hypertension of newborn. SETTING: A tertiary neonatal ICU in Canada. PATIENTS: Forty-nine neonates (gestational age ≥ 35 wk old) diagnosed with persistent pulmonary hypertension of newborn within first 3 days after birth and 50 age-matched controls. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The echocardiographic measurements comprised of right ventricular and left ventricular functional markers, including tricuspid annular plane systolic excursion, fractional area change, tissue Doppler imaging, and deformation imaging. Sample size was based on detecting an intergroup difference of 10% in tricuspid annular plane systolic excursion, which was considered the primary outcome. Linear correlations between the right and left ventricular indices, as well as their association with the outcome of death or extracorporeal membrane oxygenation were evaluated. Persistent pulmonary hypertension of newborn was associated with lower tricuspid annular plane systolic excursion (6.81 ± 1.92 vs 9.25 ± 1.30 mm), right-ventricular global longitudinal strain (16.9% ± 5.4% vs -21.6% ± 4.6%); left ventricular ejection fraction (49% ± 7% vs 55% ± 6%), left ventricular global longitudinal strain (-16.7% ± 3.3% vs -21.4% ± 2.0%) (all p < 0.01). Right and left ventricular diastolic and global function was also lower in persistent pulmonary hypertension of newborn, with more pronounced changes seen for the right ventricle. Moderate-to-strong linear correlations were observed between the right and left ventricular functional markers, with right ventricular global longitudinal strain and left ventricular global longitudinal strain being the strongest (r = 0.8). Within persistent pulmonary hypertension of newborn group, hypoxic ischemic encephalopathy was associated with lower right and left ventricular systolic and right ventricular diastolic performance. Tricuspid annular plane systolic excursion (p =0.08) and left ventricular systolic velocity (p = 0.09) tended to be lower in patients who subsequently died/needed extracorporeal membrane oxygenation. CONCLUSIONS: Persistent pulmonary hypertension of newborn is characterized by global cardiac dysfunction, involving both the right and left ventricles, with significant interventricular functional correlation. Cardiac dysfunction early in disease course may identify patients at highest risk of adverse outcome.

Calibration of an Electrical Analog Model of Liver Hemodynamics in Fontan Patients.

Fontan associated liver disease is a common complication in patients with Fontan circulation, who were born with a single functioning heart ventricle. The hepatic venous pressure gradient (HVPG) is used to assess liver health and is a surrogate measure of the pressure gradient across the entire liver (portal pressure gradient (PPG)). However, it is thought to be inaccurate in Fontan patients. The main objectives of this study were (1) to apply an existing detailed lumped parameter model (LPM) of the liver to Fontan patients using patient-specific clinical data and (2) to determine whether HVPG is a suitable measurement of PPGs in these patients. An existing LPM of the liver blood circulation was applied and tuned to simulate patient-specific liver hemodynamics. Geometries were collected from seven adult Fontan patients and used to evaluate model parameters. The model was solved and tuned using waveform measurements of flows, inlet and outlet pressures. The predicted ratio of portal to hepatic venous pressures is comparable to in vivo measurements. The results confirmed that HVPG is not suitable for Fontan patients, as it would underestimate the portal pressures gradient by a factor of 3 to 4. Our patient-specific liver model provides an estimate of the pressure drop across the liver, which differs from the clinically used metric HVPG. This work represents a first step toward models suitable to assess liver health in Fontan patients and improve its long-term management.

Right Ventricular Remodeling in Hypoplastic Left Heart Syndrome is Minimally Impacted by Cardiopulmonary Bypass: A Comparison of Norwood vs. Hybrid.

Right ventricular (RV) remodeling in hypoplastic left heart syndrome (HLHS) begins prenatally and continues through staged palliations. However, it is unclear if the most marked observed remodeling post-Norwood is secondary to cardiopulmonary bypass (CPB) exposure or if it is an adaptation intrinsic to the systemic RV. This study aims to determine the impact of CPB on RV remodeling in HLHS. Echocardiograms of HLHS survivors undergoing stage 1 Norwood (n = 26) or Hybrid (n = 20) were analyzed at pre- and post-stage 1, pre- and post-bidirectional cavo-pulmonary anastomosis (BCPA), and pre-Fontan. RV fractional area change (FAC), vector velocity imaging for longitudinal & derived circumferential deformation (global radial shortening (GRS) = peak radial displacement/end-diastolic diameter), and deformation ratio (longitudinal/ circumferential) were assessed. Both groups had similar age, clinical status and functional parameters pre-stage 1. No difference in RV size and sphericity at any stage between groups. RVFAC was normal (> 35%) throughout for both groups. Both Norwood and Hybrid patients had increased GRS (p = 0.0001) post-stage 1 and corresponding unchanged longitudinal strain, resulting in decreased deformation ratio (greater relative RV circumferential contraction), p = 0.0001. Deformation ratio remained decreased in both groups in subsequent stages. Irrespective of timing of the first CPB exposure, both Norwood and Hybrid patients underwent similar RV remodeling, with relative increase in circumferential to longitudinal contraction soon after stage 1 palliation. The observed RV remodeling in HLHS survivors were minimally impacted by CPB.

The Cardiac Genome Clinic: implementing genome sequencing in pediatric heart disease.

PURPOSE: This study investigated the diagnostic utility of nontargeted genomic testing in patients with pediatric heart disease. METHODS: We analyzed genome sequencing data of 111 families with cardiac lesions for rare, disease-associated variation. RESULTS: In 14 families (12.6%), we identified causative variants: seven were de novo (ANKRD11, KMT2D, NR2F2, POGZ, PTPN11, PURA, SALL1) and six were inherited from parents with no or subclinical heart phenotypes (FLT4, DNAH9, MYH11, NEXMIF, NIPBL, PTPN11). Outcome of the testing was associated with the presence of extracardiac features (p = 0.02), but not a positive family history for cardiac lesions (p = 0.67). We also report novel plausible gene-disease associations for tetralogy of Fallot/pulmonary stenosis (CDC42BPA, FGD5), hypoplastic left or right heart (SMARCC1, TLN2, TRPM4, VASP), congenitally corrected transposition of the great arteries (UBXN10), and early-onset cardiomyopathy (TPCN1). The identified candidate genes have critical functions in heart development, such as angiogenesis, mechanotransduction, regulation of heart size, chromatin remodeling, or ciliogenesis. CONCLUSION: This data set demonstrates the diagnostic and scientific value of genome sequencing in pediatric heart disease, anticipating its role as a first-tier diagnostic test. The genetic heterogeneity will necessitate large-scale genomic initiatives for delineating novel gene-disease associations.

Impaired Right Ventricular Performance Is Associated with Adverse Outcome after Hypoxic Ischemic Encephalopathy.

Rationale: Asphyxiated neonates with hypoxic ischemic encephalopathy (HIE) are at risk of myocardial dysfunction; however, echocardiography studies are limited and little is known about the relationship between hemodynamics and brain injury.Objectives: To analyze the association between severity of myocardial dysfunction and adverse outcome as defined by the composite of death and/or abnormal magnetic resonance imaging.Methods: Neonates with HIE undergoing therapeutic hypothermia were enrolled. Participants underwent echocardiography at 24 hours, 72 hours (before rewarming), and 96 hours (after rewarming). Cerebral hemodynamics were monitored by near-infrared spectroscopy and middle cerebral artery Doppler.Measurements and Main Results: Fifty-three patients with a mean gestation and birthweight of 38.8 ± 2.0 weeks and 3.33 ± 0.6 kg, respectively, were recruited. Thirteen patients (25%) had mild encephalopathy, 27 (50%) had moderate encephalopathy, and 13 (25%) had severe encephalopathy. Eighteen patients (34%) had an adverse outcome. Severity of cardiovascular illness (P < 0.001) and severity of neurologic insult (P = 0.02) were higher in neonates with adverse outcome. Right ventricle (RV) systolic performance at 24 hours was substantially lower than published normative data in all groups. At 24 hours, lower tricuspid annular plane systolic excursion (P = 0.004) and RV fractional area change (P < 0.001), but not pulmonary hypertension, were independently associated with adverse outcome on logistic regression. High brain regional oxygen saturation (P = 0.007) and low middle cerebral artery resistive index (P = 0.04) were associated with RV dysfunction on post hoc analysis.Conclusions: RV dysfunction is associated with the risk of adverse outcome in asphyxiated patients with HIE undergoing hypothermia. Echocardiography may be a valuable diagnostic and prognostic tool in this vulnerable population.

Intraoperative echocardiographic coronary artery imaging in congenital and acquired heart disease.

Transoesophageal and epicardial echocardiography are indispensible intraoperative imaging modalities to guide paediatric heart disease surgeries and influence surgical decision-making. A less well-described role of intraoperative imaging is its utility in evaluating coronary artery patency and flow. Focused two-dimensional, colour, and spectral Doppler imaging of the coronary arteries should be performed during surgeries involving coronary manipulation or re-implantation, or in cases where there is unexpected ventricular dysfunction or electrographic signs concerning for ischaemia. Intraoperative imaging allows for any anatomical issues to be detected and addressed promptly in the operating room. Imaging of the coronary arteries should identify unobstructed coronary ostia and proximal course without kinking, angulation, narrowing, or significant calibre change to suggest stenosis or extrinsic compression from neighbouring structures. The aim of this review is to highlight the usefulness of transoesophageal and epicardial echocardiography in evaluating coronary artery patency and flow, provide a how-to guide for optimal imaging, and to introduce a practical guideline to achieve best clinical practice.

Right ventricular function in patients with pulmonary regurgitation with versus without tetralogy of Fallot.

BACKGROUND: Right ventricular (RV) dilation from pulmonary valve regurgitation (PR) is common after intervention(s) for pulmonary stenosis (PS) or atresia and intact ventricular septum (PA/IVS). It is not well established whether PR and RV dilation have similar effects on RV function and exercise capacity in these patients compared to patients after repair of tetralogy of Fallot (rToF). The aims of this study were to compare exercise tolerance, RV function and myocardial mechanics in non-ToF versus rToF children with significantly increased and comparable RV volumes. METHODS: Thirty PS or PA/IVS children after intervention(s) with significant PR and RV dilation (non-ToF group) were retrospectively matched for RV end-diastolic volume index (RVEDVi) and age with 30 rToF patients. Clinical characteristics, RV function by echocardiography and CMR, ECG and exercise capacity were compared between groups. RESULTS: The groups were well matched for RVEDVi and age. Global RV function (RVEF: 48.7 ±â€¯6.4% vs. 48.5 ±â€¯7.2%, P = .81) and exercise capacity (% predicted peak VO2:82.5 ±â€¯17.7% vs. 75.6 ±â€¯20.4%, P = .27) were similarly reduced between groups. RVEDVi correlated inversely with RVEF in both groups (non-ToF:r = -0.39, P = .04, rToF:r = -0.40, P = .03). QRS duration was wider in rToF patients, and in both groups inversely correlated with RVEF (non-ToF:r = -0.77, P < .001, rToF:r = -0.69, P < .001). In contrast to global function, longitudinal RV strain was lower in rTOF vs non-TOF (-20.1 ±â€¯3.9 vs.-25.7 ±â€¯4.4, P < .001). CONCLUSIONS: Global RV function and exercise capacity are similarly reduced in non-ToF and rToF patients with severely dilated RV, after matching by RVEDVi, suggesting a comparable impact of RV dilation on RV global function. The significance of reduced RV longitudinal function and worse dyssynchrony in rToF patients require further exploration.

Prevalence of anthracycline-related cardiac dysfunction in long-term survivors of adult-onset lymphoma.

BACKGROUND: Anthracycline-related heart failure is a leading cause of morbidity in survivors of adult-onset lymphoma. There is a paucity of information on screening for late-occurring preclinical disease, which, in turn, has limited guidelines for early detection and intervention. The objectives of this study were to examine the prevalence and risk of cardiac dysfunction, as measured by echocardiography (abnormal left ventricular systolic/diastolic function or strain), in lymphoma survivors who received treatment with anthracyclines and to evaluate the diagnostic yield of blood biomarkers in the asymptomatic setting. METHODS: Lymphoma survivors who underwent hematopoietic cell transplantation (HCT) (n = 78) or received conventional therapy (non-HCT; n = 77) were compared with each other and with a group of matched controls (n = 51); the study was limited to lymphoma survivors who were >5 years from diagnosis. RESULTS: At a median follow-up of 9.4 years after diagnosis, 1 in 5 (20.6 %) lymphoma survivors had cardiac dysfunction; the odds of having cardiac dysfunction were 6.6-fold greater (odds ratio [OR], 6.6; P = .01) among lymphoma survivors compared with matched controls. There was a dose-dependent risk of cardiac dysfunction according to the cumulative anthracycline dose (controls [referent group], 1-249 mg/m2 [OR, 4.7; P = .05], and ≥250 mg/m2 [OR, 7.6; P < .01]), but there was no difference in the prevalence of cardiac dysfunction between conventionally treated and HCT survivors. The diagnostic accuracy of cardiac blood biomarkers in the asymptomatic setting was quite poor. CONCLUSIONS: In these long-term survivors, there was a high rate of cardiac dysfunction that was independent of HCT status. The growing number of lymphoma survivors makes it imperative to identify reliable and cost-effective strategies to decrease the burden of heart failure in this population. Cancer 2018;124:850-7. © 2017 American Cancer Society.

Pediatric Outcome after Maternal Cancer Diagnosed during Pregnancy.

BACKGROUND: Data on the long-term outcome of children who are exposed to maternal cancer with or without treatment during pregnancy are lacking. METHODS: In this multicenter case-control study, we compared children whose mothers received a diagnosis of cancer during the pregnancy with matched children of women without a cancer diagnosis. We used a health questionnaire and medical files to collect data regarding neonatal and general health. All children were prospectively assessed (by means of a neurologic examination and the Bayley Scales of Infant Development) at 18 months, 36 months, or both. A cardiac assessment was performed at 36 months. RESULTS: A total of 129 children (median age, 22 months; range, 12 to 42) were included in the group whose mother had cancer (prenatal-exposure group) with a matching number in the control group. During pregnancy, 96 children (74.4%) were exposed to chemotherapy (alone or in combination with other treatments), 11 (8.5%) to radiotherapy (alone or in combination), 13 (10.1%) to surgery alone, 2 (1.6%) to other drug treatments, and 14 (10.9%) to no treatment. Birth weight was below the 10th percentile in 28 of 127 children (22.0%) in the prenatal-exposure group and in 19 of 125 children (15.2%) in the control group (P=0.16). There was no significant between-group difference in cognitive development on the basis of the Bayley score (P=0.08) or in subgroup analyses. The gestational age at birth was correlated with the cognitive outcome in the two study groups. Cardiologic evaluation among 47 children at 36 months of age showed normal cardiac findings. CONCLUSIONS: Prenatal exposure to maternal cancer with or without treatment did not impair the cognitive, cardiac, or general development of children in early childhood. Prematurity was correlated with a worse cognitive outcome, but this effect was independent of cancer treatment. (Funded by Research Foundation-Flanders and others; ClinicalTrials.gov number, NCT00330447.).

In Situ Growth and Size Regulation of Single Gold Nanoparticles in Composite Microgels.

Herein, a novel method for the in situ growth of single gold nanoparticles (AuNPs) in microgel (MG) networks is presented. The key feature in this approach is the localization of ß-diketone groups capable of both complexation and reduction of aurate ions in the MGs' core, which allows localization of the nucleation and growth of single AuNPs. The MG synthesis is carried out via precipitation polymerization in water with N-vinylcaprolactam as the main monomer and with the two comonomers acetoacetoxyethyl methacrylate (AAEM) and acrylic acid (AAc), where AAEM is mainly located in the MGs' core and AAc in their shell. For the synthesis of AuNPs, a certain amount of chloroauric acid (HAuCl4 ) is added to the dispersion, followed by fast reduction with sodium borohydride (NaBH4 ). In situ synthesized AuNPs in MGs possess a spherical shape, with a diameter of 8.1 ± 0.8 nm, being localized in the center of every MG. In addition, these AuNPs embedded into MG networks can be used as seeds that grow in their size after the addition of HAuCl4 up to 46.0 ± 9.5 nm under mild reaction conditions (room temperature, aqueous dispersion) and without the use of any additional reducing and stabilizing agents.

Echocardiography Grading for Pulmonary Arteriovenous Malformation Screening in Children with Hereditary Hemorrhagic Telangiectasia.

Transthoracic contrast echocardiography (TTCE) has high sensitivity but low specificity in screening for pulmonary arteriovenous malformations (pAVMs) in children with hereditary hemorrhagic telangiectasia (HHT). Here we describe characteristics of TTCE that might be used to reduce the need for confirmatory computed tomography scans in children with HHT.