Pediatric Congenital Heart Disease Patients Research Articles

Acute neurological injury in pediatric patients with single-ventricle congenital heart disease.

Single-ventricle congenital heart disease (CHD) in pediatric patients with Glenn and Fontan physiology represents a unique physiology requiring the surgical diversion of the systemic venous return from the superior vena cava (Glenn) and then the inferior vena cava (Fontan) directly to the pulmonary arteries. Because many of these patients are on chronic anticoagulation therapy and may have right-to-left shunts, arrhythmias, or lymphatic disorders that predispose them to bleeding and/or clotting, they are at risk of experiencing neurological injury requiring intubation and positive pressure ventilation, which can significantly hamper pulmonary blood flow and cardiac output. The aim of this study was to describe the complex neurological and cardiopulmonary interactions of these pediatric patients after acute central nervous system (CNS) injury. The authors retrospectively analyzed the records of pediatric patients who had been admitted to a quaternary children's hospital with CHD palliated to bidirectional Glenn (BDG) or Fontan circulation and acute CNS injury and who had undergone intubation and mechanical ventilation. Patients who had been admitted from 2005 to 2019 were included in the study. Clinical characteristics, surgical outcomes, cardiovascular and pulmonary data, and intracranial pressure data were collected and analyzed. Nine pediatric single-ventricle patients met the study inclusion criteria. All had undergone the BDG procedure, and the majority (78%) were status post Fontan palliation. The mean age was 7.4 years (range 1.3-17.3 years). At the time of acute CNS injury, which included traumatic brain injury, intracranial hemorrhage, and cerebral infarct, the median time interval from the most recent cardiac surgical procedure was 3 years (range 2 weeks-11 years). Maintaining normocarbia to mild hypercarbia for most patients during intubation periods did not cause neurological deterioration, and hemodynamic profiles were more favorable as compared to periods of hypocarbia. Hypocarbia was associated with unfavorable hemodynamics but was necessary to decrease intracranial hypertension. Most patients were managed using low mean airway pressure (MAWP) in order to minimize the impact on preload and cardiac output. The authors highlight the complex neurological and cardiopulmonary interactions with respect to partial pressure of arterial CO2 (PaCO2) and MAWP when pediatric CHD patients with single-ventricle physiology require mechanical ventilation. The study data demonstrated that tight control of PaCO2 and minimizing MAWP with the goal of early extubation may be beneficial in this population. A multidisciplinary team of pediatric critical care intensivists, cardiac intensivists and anesthesiologists, and pediatric neurosurgeons and neurologists are recommended to ensure the best possible outcomes.

Early Experience with High-density Electroanatomical Mapping Using the Rhythmia™ Mapping System in Congenital and Pediatric Heart Disease.

The Rhythmia™ system (Boston Scientific, Natick, MA, USA) facilitates the rapid acquisition of high-resolution electroanatomical and activation maps. However, there are limited data on its efficacy and safety in pediatric and adult congenital heart disease (CHD) patients. In a retrospective, observational cohort study, adult CHD and pediatric patients followed by pediatric cardiology underwent electrophysiologic study using the Rhythmia™ electroanatomic mapping system. Variables examined included the number of electroanatomical maps required, acquisition time, procedure time, fluoroscopy time, radiation dosage, and rate of recurrent arrhythmia. Twelve consecutive patients, including six male patients (50%), were included with an average age of 27.7 years (range: 11–64 years). Seven (58%) of these patients had a diagnosis of CHD [moderate complexity in two (17%) and great complexity in five patients (42%)] and 10 (83%) patients underwent ablation. A total of 37 high-density maps were created in 12 procedures, with a median of 8,140 mapping points, taking a median of 631 seconds. The median procedure time was 189.5 minutes. The median fluoroscopy time was 0.9 minutes, with eight (67%) patients receiving no fluoroscopy at all. Recurrence occurred in one patient (8%) over a median follow-up duration of 16 months (interquartile range: 12.8–17.3 months). No adverse periprocedural events were recorded. This study suggests the use of high-density electroanatomic mapping in adult CHD patients showed potential for rapid acquisition of highly detailed maps with minimal fluoroscopy time or risk of periprocedural events in the studied population.

B-PO02-005 THE EFFECTS OF CARDIAC IMPLANTABLE ELECTRONIC DEVICE PERSONAL PROTECTIVE EQUIPMENT USE IN PATIENTS WITH PEDIATRIC AND ADULT CONGENITAL HEART DISEASE

Pediatric and adult congenital heart disease (ACHD) patients encounter physical and emotional barriers. Cardiac implantable electronic devices (CIEDs), including pacemakers and implantable cardiac defibrillators (ICD) often compound these issues. Damage to the CIED system may lead to urgent/emergent medical care and additional surgeries to resolve. Patient fear associated with damaging the CIED system can lead to avoidance of physical activity and reduced quality of life (QOL). CIED personal protective equipment (PPE) is a potential treatment for decreasing this fear. To determine the effects of CIED PPE use in the pediatric and ACHD population. Patients 5 years or older with a CIED at a single pediatric and ACHD heart rhythm center were prospectively enrolled. Demographic data and baseline assessments of self-reported physical activity and concerns regarding activity were obtained. QOL was assessed using the PedsQLTM 4.0 SF 15. Each participant received a Vital Beat (Tienhoven, The Netherlands) CIED protector and customized athletic shirt to support the device at the correct location. Repeat assessment after 6-12 months was completed. Statistical analysis with Wilcoxon signed-rank test was performed to compare pre/post-intervention responses. Of the 77 patients enrolled, 26 completed repeat assessment. The mean age at enrollment was 15.6 years (range 5 - 36) with a mean device age of 2.2 years (<1 - 10 years). Pacemakers were present in 77% and 23% had ICDs. Leads were epicardial with abdominal placement in 65% and transvenous with chest placement in 35%. There was structural heart disease in 77% and 31% were device dependent. The PPE was used in 92% and there were no reports of PPE malfunction. Fear associated with physical activity was significantly reduced, z = -4, p< .001, with a large effect size (r = .55). Increase in physical activity occurred in 23% though not statistically significant. Total quality of life scores improved, z = -2.771, P<.05, with a medium effect size (r = .4). Providing CIED PPE to pediatric and ACHD patients may decrease their fear of damaging their device system, increasing physical activity levels, and improving quality of life.

Pre-operative assessment of pediatric congenital heart disease patients in the COVID-19 era: lessons learned.

Equal to COVID-19 patients, non-COVID-19 patients are affected by the medical and social drawbacks of the COVID-19 pandemic. A significant reduction in elective life-changing surgeries has been witnessed in almost all affected countries. This study discusses an applicable and effective pre-operative assessment protocol that can be applied during the COVID-19 era. Our study is a descriptive retrospective observational study that involves children with CHD requiring open-heart surgeries at our tertiary care centre between March and November, 2020. We reviewed the charts of eligible patients aged 18 years and below. We identified the total numbers of scheduled, performed, and postponed surgeries, respectively. A thorough description of the clinical and physical presentation of the postponed cases, who tested positive for SARS-CoV-2, is provided. Sixty-eight open-heart surgeries were scheduled at our centre between March and November, 2020. Three surgeries (4%) were postponed due to COVID-19. The three patients were asymptomatic COVID-19 cases detected on routine SARS-CoV-2 polymerase chain reaction testing. No symptoms of cough, chest pain, dyspnea, rhinorrhea, diarrhea, abdominal pain, anosmia, and ageusia were reported by our patients. All patients were afebrile and hemodynamically stable. Owing to the pre-operative assessment protocol that was implemented after the first case was detected, only three healthcare workers were at risk of COVID-19 transmission and were imposed to infectious evaluation and home quarantine. Adopting our discussed preoperative COVID-19 assessment protocol for CHD patients is an effective method to detect COVID-19 infections, optimise patient care, and ensure healthcare workers' safety.

Exploring the Differences in Macrocognition Between Experts and Non-CHD Experts Managing Congenital Heart Disease (CHD)

Background and aims: Children with congenital heart disease (CHD) are at risk of deterioration in the face of common childhood illnesses, and their resuscitation and acute management is often best achieved with the guidance of CHD experts. Access to such expertise may be limited outside specialty heart centers and the fragility of these patients is cause for discomfort among many emergency medicine physicians. An understanding of the differences in macrocognition of these clinicians could shed light on some of the causes of discomfort and facilitate the development of a sociotechnological solution to this problem. Methods: Cardiac intensivists (CHD experts) and pediatric emergency medicine physicians (non-CHD experts) in a major academic cardiac center were interviewed using the critical decision method. Interview transcripts were coded deductively based on Klein’s macrocognitive framework and inductively to allow for new or modified characterization of dimensions. Results: While both CHD-experts and non-CHD experts relied on the macrocognitive functions of sensemaking, naturalistic decision making and detecting problems, the specific data and mental models used to understand the patients and course of therapy differed between CHD-experts and non-CHD experts. Conclusion: Characterization of differences between the macrocognitive processes of CHD experts and non-CHD experts can inform development of sociotechnological solutions to augment decision making pertaining to the acute management of pediatric CHD patients.

Neurocognitive disorders amongst patients with congenital heart disease undergoing procedures in childhood

BackgroundAmongst children with congenital heart disease (CHD), earlier age of repair improves cardiovascular outcomes. The effects of early intervention on neurodevelopment remains unclear. We assessed the association between early life repair, cardiopulmonary bypass (CPB) and the incidence of neurocognitive disorders (NCD) amongst CHD patients. MethodsWe created two retrospective cohorts from the Quebec CHD Database; with data from 1988 to 2010. Complexity of reparative procedures for CHD lesions were used as the proxy of CPB exposure with more complex procedure means longer exposure to CPB. Study Population 1 included pediatric patients with a single reparative procedure and compared patients with complex (long CBP) versus isolated shunt (short CBP) lesions. To assess the effects of CPB alone in Study Population 2 we compared patients with isolated atrial septal defects (ASD) who had surgical (short CBP) versus percutaneous (no CBP) repairs. The primary endpoint for both cohorts was development of an NCD. ResultsIn Study population 1, 1174 patients underwent complex surgical repair and 1620 had a shunt closure. The incidence of NCDs was 2.45/100 person-years in the complex surgery group, and 2.08/100 person-years in the shunt closure group. The following were associated with increased risk of developing a NCD: surgical complexity (Hazard Ratio [HR] = 1.20, 95% Confidence Interval [CI]: 1.01–1.42), younger age at intervention (AAI) (HR = 1.20, 95% CI: 1.16–1.25), male sex (HR = 1.91, 95% CI: 1.61–2.27), and later calendar year at intervention (HR = 1.06, 95% CI: 1.04–1.07). Study population 2 had 527 isolated ASD patients; 202 underwent surgical repair and 325 had percutaneous closure. The incidence of NCDs was not statistically different between groups. Male sex (HR = 1.77, 95% CI: 1.08–2.89) and younger AAI (HR = 1.15, 95% CI: 1.06–1.25) were associated with increased NCD risk. ConclusionIncreased surgical complexity, male sex and younger AAI were associated with increased risk of NCDs in pediatric CHD patients. Surveillance protocols should be considered to identify NCDs in CHD patients after cardiac intervention.

Impact of hard lockdown on interventional cardiology procedures in congenital heart disease: a survey on behalf of the Italian Society of Congenital Heart Disease.

The Coronavirus disease 2019 (COVID-19) pandemic has thoroughly and deeply affected the provision of healthcare services worldwide. In order to limit the in-hospital infections and to redistribute the healthcare professionals, cardiac percutaneous intervention in Pediatric and Adult Congenital Heart Disease (ACHD) patients were limited to urgent or emergency ones. The aim of this article is to describe the impact of the COVID-19 pandemic on Pediatric and ACHD cath laboratory activity during the so-called 'hard lockdown' in Italy. Eleven out of 12 Italian institutions with a dedicated Invasive Cardiology Unit in Congenital Heart Disease actively participated in the survey. The interventional cardiology activity was reduced by more than 50% in 6 out of 11 centers. Adolescent and ACHD patients suffered the highest rate of reduction. There was an evident discrepancy in the management of the hard lockdown, irrespective of the number of COVID-19 positive cases registered, with a higher reduction in Southern Italy compared with the most affected regions (Lombardy, Piedmont, Veneto and Emilia Romagna). Although the pandemic was brilliantly addressed in most cases, we recognize the necessity for planning new, and hopefully homogeneous, strategies in order to be prepared for an upcoming new outbreak.

Application of CRISPR-Cas9 gene editing for congenital heart disease.

Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9 (CRISPR-Cas9) is an ancient prokaryotic defense system that precisely cuts foreign genomic DNA under the control of a small number of guide RNAs. The CRISPR-Cas9 system facilitates efficient double-stranded DNA cleavage that has been recently adopted for genome editing to create or correct inherited genetic mutations causing disease. Congenital heart disease (CHD) is generally caused by genetic mutations such as base substitutions, deletions, and insertions, which result in diverse developmental defects and remains a leading cause of birth defects. Pediatric CHD patients exhibit a spectrum of cardiac abnormalities such as septal defects, valvular defects, and abnormal chamber development. CHD onset occurs during the prenatal period and often results in early lethality during childhood. Because CRISPR-Cas9-based genome editing technology has gained considerable attention for its potential to prevent and treat diseases, we will review the CRISPR-Cas9 system as a genome editing tool and focus on its therapeutic application for CHD.

Free-breathing high resolution modified Dixon steady-state angiography with compressed sensing for the assessment of the thoracic vasculature in pediatric patients with congenital heart disease

BackgroundCardiovascular magnetic resonance angiography (CMRA) is a non-invasive imaging modality of choice in pediatric patients with congenital heart disease (CHD). This study was aimed to evaluate the diagnostic utility of a respiratory- and electrocardiogram-gated steady-state CMRA with modified Dixon (mDixon) fat suppression technique and compressed sensing in comparison to standard first-pass CMRA in pediatric patients with CHD at 3 T.MethodsIn this retrospective single center study, pediatric CHD patients who underwent CMR with first-pass CMRA followed by mDixon steady-state CMRA at 3 T were analyzed. Image quality using a Likert scale from 5 (excellent) to 1 (non-diagnostic) and quality of fat suppression were assessed in consensus by two readers. Blood-to-tissue contrast and quantitative measurements of the thoracic vasculature were assessed separately by two readers. CMRA images were reevaluated by two readers for additional findings, which could be identified only on either one of the CMRA types. Paired Student t test, Wilcoxon test, and intraclass correlation coefficients (ICCs) were used for statistical analysis.Results32 patients with CHD (3.3 ± 1.7 years, 13 female) were included. Overall image quality of steady-state mDixon CMRA was higher compared to first-pass CMRA (4.5 ± 0.5 vs. 3.3 ± 0.5; P < 0.001). Blood-to-tissue contrast ratio of steady-state mDixon CMRA was comparable to first-pass CMRA (7.85 ± 4.75 vs. 6.35 ± 2.23; P = 0.133). Fat suppression of steady-state mDixon CMRA was perfect in 30/32 (94%) cases. Vessel diameters were greater in first-pass CMRA compared to steady-state mDixon CMRA with the greatest differences at the level of pulmonary arteries and veins (e.g., right pulmonary artery for reader 1: 10.4 ± 2.4 vs. 9.9 ± 2.3 mm, P < 0.001). Interobserver agreement was higher for steady-state mDixon CMRA for all measurements compared to first-pass CMRA (ICCs > 0.92). In 9/32 (28%) patients, 10 additional findings were identified on mDixon steady-state CMRA (e.g., partial anomalous venous return, abnormalities of coronary arteries, subclavian artery stenosis), which were not depicted using first-pass CMRA.ConclusionsSteady-state mDixon CMRA offers a robust fat suppression, a high image quality, and diagnostic utility for the assessment of the thoracic vasculature in pediatric CHD patients.

Tissue engineering: Relevance to neonatal congenital heart disease

Congenital heart disease (CHD) represents a large clinical burden, representing the most common cause of birth defect-related death in the newborn. The mainstay of treatment for CHD remains palliative surgery using prosthetic vascular grafts and valves. These devices have limited effectiveness in pediatric patients due to thrombosis, infection, limited endothelialization, and a lack of growth potential. Tissue engineering has shown promise in providing new solutions for pediatric CHD patients through the development of tissue engineered vascular grafts, heart patches, and heart valves. In this review, we examine the current surgical treatments for congenital heart disease and the research being conducted to create tissue engineered products for these patients. While much research remains to be done before tissue engineering becomes a mainstay of clinical treatment for CHD patients, developments have been progressing rapidly towards translation of tissue engineering devices to the clinic.

Multi-institution assessment of the use and risk of cardiovascular computed tomography in pediatric patients with congenital heart disease

BackgroundCardiac computed tomography (CT) is increasingly used in pediatric patients with congenital heart disease (CHD). Variability of practice and of comprehensive diagnostic risk across institutions is not known. MethodsFour centers prospectively enrolled consecutive pediatric CHD patients <18 years of age undergoing cardiac CT from January 6, 2017 to 1/30/2020. Patient characteristics, cardiac CT data and comprehensive diagnostic risk were compared by age and institutions. Risk categories included sedation and anesthesia use, vascular access, contrast exposure, cardiovascular medication, adverse events (AEs), and estimated radiation dose. ResultsCardiac CT was performed in 1045 pediatric patients at a median (interquartile range, IQR) age of 1.7 years (0.3, 11.0). The most common indications were arterial abnormalities, suspected coronary artery anomalies, functionally single ventricle heart disease, and tetralogy of Fallot/pulmonary atresia. Sedation was used in 8% and anesthesia in 11% of patients. Peripheral vascular access was utilized for 93%. Median contrast volume was 2 ​ml/kg. Beta blockers were administered in 11% of cases and nitroglycerin in 2% of cases. The median (IQR) total procedural dose length product (DLP) was 20 ​mGy∗cm (10, 50). Sedation, vascular access, contrast exposure, use of cardiovascular medications and radiation dose estimates varied significantly by institution and age (p ​< ​0.001). Seven minor adverse events (0.7%) and no major adverse events were reported. ConclusionCardiac CT for CHD is safe in pediatric patients when appropriate CT technology and expertise are available. Scans can be acquired at relatively low radiation exposure with few minor adverse events.

Efficacy and safety of tolvaptan after pediatric congenital heart disease surgery.

It is not clear whether tolvaptan is safe and effective irrespective of various underlying clinical conditions including the functional ventricle morphology, chromosomal abnormalities, and renal function after complex pediatric congenital heart disease surgery. Also, the appropriate dose of tolvaptan in these patients has not been previously identified. We retrospectively assessed the urine volume, body weight, patient clinical characteristics, laboratory data, and vital signs before and on days 1 and 7 of the tolvaptan administration after congenital heart disease surgery. Also, we assessed the relationship between the tolvaptan dose and its effects. A total of 86 patients were included the study. The mean time from the surgery to the tolvaptan administration was 23.5 ± 3.7days. After administering tolvaptan, the urine volume significantly increased and body weight significantly decreased from baseline by days 1 and 7 (p < 0.0001). The urine volume significantly increased more in the survivors than the deceased. Of the 22 patients who had low serum sodium concentrations at baseline, 20 had an increased serum sodium concentration on day 7. The clinical effect of tolvaptan was not affected by the functional ventricle morphology, chromosomal abnormalities, or renal function. There was a positive correlation between the tolvaptan dose and change in the urine volume until a tolvaptan dose of up to 0.3mg/kg/day but not at more than 0.3mg/kg/day. Tolvaptan administration is safe and effective after congenital heart disease surgery irrespective of various underlying clinical conditions. Though the urine volume tends to increase until a tolvaptan dose of up to 0.3mg/kg/day in pediatric congenital heart disease patients, there was no further benefit with more than 0.3mg/kg/day.

The benefit of exercise in children with congenital heart disease

Children and adolescents with congenital heart disease (CHD) often have decreased exercise capacity and muscle mass. Exercise has numerous health benefits and can improve exercise capacity and muscle mass. As such, guidelines recommend 60 min of daily physical activity in the majority of pediatric CHD patients. Despite these known benefits and guidelines, children and adolescents with CHD often do not exercise regularly. The purpose of this review is to summarize the current literature on exercise in this population. With recent advancements in the field, clinical focus in pediatric cardiology has expanded from improving mortality to improving long-term outcomes and quality of life. As part of this paradigm shift, there is ongoing research on safety, impact, and effective delivery of exercise to this population with the overarching goal of promoting an active lifestyle. Exercise interventions are generally safe, feasible, and beneficial in children and adolescents with CHD. There is growing evidence demonstrating the benefits and utility of exercise as a prescription rather than restriction in pediatric CHD patients. Novel approaches are needed for effective delivery of exercise in this population. Further studies are warranted for assessment of long-term benefits of exercise in pediatric CHD patients.

In silico features of ADAMTS13 contributing to plasmatic ADAMTS13 levels in neonates with congenital heart disease

IntroductionRisk factors contributing to heightened thrombosis in pediatric congenital heart disease (CHD) patients are not fully understood. Among the neonatal CHD population, those presenting with single ventricular physiology are at the highest risk for perioperative thrombosis. The von Willebrand factor and ADAMTS13 interactions have emerged as causative risk factors for pediatric stroke and could contribute to heightened thrombosis in CHD neonates. MethodsThis study investigates a cohort of children with single ventricle physiology and undergoing cardiac surgery, during which some patients developed thrombosis. In this cohort, we analyzed the relationship of several molecular features of ADAMTS13 with the plasma and activity levels in patients at risk of thrombosis. Additionally, in light of the natural antithrombotic activity of ADAMTS13, we have sequenced the ADAMTS13 gene for each patient and evaluated the role of genetic variants in determining the plasma ADAMTS13 levels using a series of in silico tools including Hidden Markov Models, EVmutation, and Rosetta. ResultsLower ADAMTS13 levels were found in patients that developed thrombosis. A novel in silico analysis to assess haplotype effect of co-occurring variants identified alterations in relative surface area and solvation energy as important contributors. Our analysis suggested that beneficial or deleterious effect of a variant can be reasonably predicted by comprehensive analysis of in silico assessment and in vitro and/or in vivo data. ConclusionFindings from this study add to our understanding the role of genetic features of ADAMTS13 in patients at high risk of thrombosis related to an imbalanced relation between VWF and ADAMTS13.

Assessment of gadolinium deposition in the brain tissue of pediatric and adult congenital heart disease patients after contrast enhanced cardiovascular magnetic resonance

BackgroundContrast enhanced magnetic resonance imaging (MRI) is an important tool for the assessment of extracardiac vasculature and myocardial viability. Gadolinium (Gd) brain deposition after contrast enhanced MRI has recently been described and resulted in a warning issued by the United States Food and Drug Administration. However, the prevalence of brain deposition in children and adults with congenital heart disease (CHD) undergoing cardiovascular magnetic resonance (CMR) is unclear. We hypothesized that Gd exposure as part of one or more CMRs would lead to a low rate of brain deposition in pediatric and adult CHD patients.MethodsWe queried our institutional electronic health record for all pediatric and adult CHD patients who underwent contrast enhanced CMR from 2005 to 2018 and had a subsequent brain MRI. Cases were age- and gender-matched to controls who were never exposed to Gd and underwent brain MRIs. The total number of contrast enhanced MRIs, type of Gd, and total Gd dose were determined. Brain MRIs were reviewed by a neuroradiologist for evidence of Gd deposition using qualitative and quantitative assessment. Quantitative assessment was performed using the dentate nucleus to pons signal intensity ratio (dp-SIR) on T1 weighted imaging. Continuous variables were analyzed using Mann–Whitney U and Spearman rank correlation tests. Normal SIR was defined as the 95% CI of the control population dp-SIR.ResultsSixty-two cases and 62 controls were identified. The most contrast enhanced MRIs in a single patient was five and the largest lifetime dose of Gd that any patient received was 0.75 mmol/kg. There was no significant difference in the mean dp-SIR of cases and controls (p = 0.11). The dp-SIR was not correlated with either the lifetime dose of Gd (rs = 0.21, p = 0.11) or the lifetime number of contrast enhanced studies (rs = 0.21, p = 0.11). Two cases and 2 controls had dp-SIRs above the upper bound of the 95% confidence interval for the control group. One case had qualitative imaging-based evidence of Gd deposition in the brain but had a dp-SIR within the normal range.ConclusionIn our cohort of pediatric and adult CHD patients undergoing contrast enhanced CMR, there was a low incidence of qualitative and no significant quantitative imaging-based evidence of Gd brain deposition.

Perioperative Echocardiographic Hemodynamic Parameters and Postoperative Outcome in Pediatric Congenital Heart Disease: A Descriptive Observational Prospective Pilot Study Protocol

Background: We have elaborated a randomized controlled trial (RCT) protocol in pediatric congenital heart disease patients scheduled for surgical repair. In this RCT protocol trans-thoracic echocardiography will be realized perioperatively to guide fluid and hemodynamic therapy in these patients. This RCT will determine the impact of goal directed therapy with echocardiography on postoperative outcome in terms of morbidity, length of intensive care unit stay (LOSICU), length of mechanical ventilation (LMV), length of hospital stay (LOS), fluid therapy and vasopressor-inotropic therapy. There are no trials in this population which have identified echocardiographic hemodynamic parameters predictive of postoperative outcome in terms of morbidity, LOSICU, LMV and LOS. The objective of this pilot observational prospective trial protocol is to describe the study which will determine echocardiographic hemodynamic parameters predictive of postoperative outcomes. These hemodynamic parameters will be integrated in the RCT which has the objective to determine the impact of goal directed fluid and hemodynamic therapy guided by trans-thoracic echocardiography on postoperative adverse outcome. Methods: Patients aged less than 18 years with congenital heart disease admitted for surgical repair will be included. Trans-thoracic echocardiography will be realized to measure different hemodynamic parameters preoperatively and perioperatively after weaning from cardiopulmonary bypass until discharge from the ICU in included patients. Primary outcome will be postoperative morbidity, secondary outcomes will be LOSICU, LMV and LOS; tertiary outcomes will be fluid therapy, vasopressor and inotropic therapy. Primary outcome measure will be the presence of postoperative organ dysfunction. Secondary outcome measures will be the number of postoperative days spent in the intensive care unit (ICU), number of postoperative days spent on invasive or non-invasive mechanical ventilation and the number of postoperative days spent in the conventional hospitalization ward. Tertiary outcome measures will be the quantity of fluid administered and the vasopressor-inotropic score (VIS). The study will be monocentric. XLSAT 2018.3 or plus will be the software for statistic analysis. Results are expected in the first semester of 2021. Conclusion: This pilot study will identify echocardiographic hemodynamic parameters predictive of postoperative adverse outcome which will be integrated in the second RCT where goal directed fluid and hemodynamic therapy will be guided with echocardiography.

Third-generation dual-source dual-energy CT in pediatric congenital heart disease patients: state-of-the-art.

Cardiovascular computer tomography (CT) in pediatric congenital heart disease (CHD) patients is often challenging. This might be due to limited patient cooperation, the high heart rate, the complexity and variety of diseases and the need for radiation dose minimization. The recent developments in CT technology with the introduction of the third-generation dual-source (DS) dual-energy (DE) CT scanners well suited to respond to these challenges. DSCT is characterized by high-pitch, long anatomic coverage and a more flexible electrocardiogram-synchronized scan. DE provides additional clinical information about vascular structures, myocardial and lung perfusion and allows artifacts reduction. These advances have increased clinical indications and modified CT protocol for pediatric CHD patients. In our hospital, DSCT with DE technology has rapidly become an important imaging technique for both pre- and postoperative management of pediatric patients with CHDs. The aim of this article is to describe the state-of-the-art in DSCT protocol with DE technology in pediatric CHD patients, providing some case examples of our experience over an 18-month period.

Identifying Specific Elements Necessary for a Pediatric Cardiac Rehabilitation Program: An e-Delphi Study

Encouraging children and adolescents with congenital heart disease (CHD) to participate in regular exercise is an essential component in helping them rise toward their full potential physically, socially, and emotionally. Identifying appropriate tools and resources that promote healthy and active lifestyles specifically for this population are currently lacking and must be developed. Cardiac rehabilitation programs are an effective modality with beneficial clinical outcomes for adult patients with coronary heart disease, however these programs are scarce for the CHD pediatric population and not well defined. PURPOSE: The purpose of this study was to identify relevant content for the development and structure of a pediatric cardiac rehabilitation curriculum for young patients with CHD using a consensus approach. METHODS: A three-round e-Delphi study among CHD and pediatric exercise physiology (EP) experts was conducted. In the first round, experts provided opinions in a closed- and open-ended electronic questionnaire to identify specific elements necessary for inclusion in a Pediatric Cardiac Rehab Program. In the second round, experts were asked to re-rate the same items after feedback and summary data was provided from round one. In the third round, the same experts were asked to re-rate items that did not reach consensus from round two. RESULTS: 47 experts were contacted via e-mail to participate on the Delphi panel. 37 consented, 35 completed round one, 29 completed round two and 28 completed the final round. After the third round, consensus was reached in 55 of 60 (92%) questionnaire items. Experts identified specific elements across four domains: exercise training, education, outcome metrics and self-confidence to be included in a Pediatric Cardiac Rehab Curriculum. CONCLUSION: This study established consensus toward the ideal program structure, exercise training principles, educational content, patient outcome measures and self-confidence promotion. By identifying the key components within each domain, there is potential to benchmark recommended standards and practice guidelines for the development of a Pediatric Cardiac Rehab curriculum to be used healthcare team members for optimizing the health and wellness of pediatric CHD patients.

Ventricular force-frequency relationships during biventricular or multisite pacing in congenital heart disease.

Traditional indices to evaluate biventricular (BiV) pacing are load dependent, fail to assess dynamic changes, and may not be appropriate in patients with congenital heart disease (CHD). We therefore measured the force-frequency relationship (FFR) using tissue Doppler-derived isovolumic acceleration (IVA) to assess the dynamic adaption of the myocardium and its variability with different ventricular pacing strategies. This was a prospective pilot study of pediatric and young adult CHD patients with biventricular or multisite pacing systems. Color-coded myocardial velocities were recorded at the base of the systemic ventricular free wall. IVA was calculated at resting heart rate and with incremental pacing. FFR curves were obtained by plotting IVA against heart rate for different ventricular pacing strategies. Ten patients were included (mean: 22±7years). The FFR identified a best and worst ventricular pacing strategy for each patient, based on the AUC at baseline, submaximal, and peak heart rates (P<.001). However, there was no single best ventricular pacing strategy that was optimal for all patients. Additionally, the best ventricular pacing strategy often differed within the same patient at different heart rates. This novel assessment demonstrates a wide variability in optimal ventricular pacing strategy. These inherent differences may play a role in the unpredictable clinical response to BiV pacing in CHD, and emphasizes an individualized approach. Furthermore, the optimal ventricular pacing varies with heart rate within individuals, suggesting that rate-responsive ventricular pacing modulation may be required to optimize ventricular performance.

Post-arrest therapeutic hypothermia in pediatric patients with congenital heart disease

BackgroundWhile therapeutic hypothermia (TH) is an effective neuroprotective therapy for neonatal hypoxic-ischemic encephalopathy, TH has not been demonstrated to improve outcome in other pediatric populations. Patients with acquired or congenital heart disease (CHD) are at high risk of both cardiac arrest and neurodevelopmental impairments, and therapies are needed to improve neurologic outcome. The primary goal of our study was to compare safety/efficacy outcomes in post-arrest CHD patients treated with TH versus controls not treated with TH. MethodsPatients with CHD treated during the first 18 months after initiation of a post-arrest TH protocol (temperature goal: 33.5 °C) were compared to historical and contemporary post-arrest controls not treated with TH. Post-arrest data, including temperature, safety measures (e.g. arrhythmia, bleeding), neurodiagnostic data (EEG, neuroimaging), and survival were compared. ResultsThirty arrest episodes treated with TH and 51 control arrest episodes were included. The groups did not differ in age, duration of arrest, post-arrest lactate, or use of ECMO-CPR. The TH group’s post-arrest temperature was significantly lower than control’s (33.6 ± 0.2 °C vs 34.7 ± 0.5 °C, p < 0.001). There was no difference between the groups in safety/efficacy measures, including arrhythmia, infections, chest-tube output, or neuroimaging abnormalities, nor in hospital survival (TH 61.5% vs control 59.1%, p = NS). Significantly more controls had seizures than TH patients (26.1% vs. 4.0%, p = 0.04). Almost all seizures were subclinical and occurred more than 24 h post-arrest. ConclusionOur data show that pediatric CHD patients who suffer cardiac arrest can be treated effectively and safely with TH, which may decrease the incidence of seizures.