Neoaortic Regurgitation Detected by Echocardiography After Arterial Switch Operation: A Systematic Review and Meta-Analysis.

Background: Neoaortic root dilatation (NeoARD) and neoaortic regurgitation (NeoAR) are common sequelae following the arterial switch operation (ASO) for transposition of the great arteries. Objectives: The authors aimed to estimate the cumulative incidence of NeoAR, assess whether larger neoaortic root dimensions were associated with NeoAR, and evaluate factors associated with the development of NeoAR during long-term follow-up. Methods: Electronic databases were systematically searched for articles that assessed NeoAR and NeoARD after ASO, published before November 2022. The primary outcome was NeoAR, classified based on severity categories (trace, mild, moderate, and severe). Cumulative incidence was estimated from Kaplan-Meier curves, neoaortic root dimensions using Z-scores, and risk factors were evaluated using random-effects meta-analysis. Results: Thirty publications, comprising a total of 6,169 patients, were included in this review. Pooled estimated cumulative incidence of ≥mild NeoAR and ≥moderate NeoAR at 30-year follow-up were 67.5% and 21.4%, respectively. At last follow-up, neoaortic Z-scores were larger at the annulus (mean difference [MD]: 1.17, 95% CI: 0.52-1.82, P < 0.001; MD: 1.38, 95% CI: 0.46-2.30, P = 0.003) and root (MD: 1.83, 95% CI: 1.16-2.49, P < 0.001; MD: 1.84, 95% CI: 1.07-2.60, P < 0.001) in patients with ≥mild and ≥moderate NeoAR, respectively, compared to those without NeoAR. Risk factors for the development of any NeoAR included prior pulmonary artery banding, presence of a ventricular septal defect, aorto-pulmonary mismatch, a bicuspid pulmonary valve, and NeoAR at discharge. Conclusions: The risks of NeoARD and NeoAR increase over time following ASO surgery. Identified risk factors for NeoAR may alert the clinician that closer follow-up is needed. (Risk factors for neoaortic valve regurgitation after arterial switch operation: a meta-analysis; CRD42022373214).

Sacubitril-valsartan vs ACE/ARB in pediatric heart failure: A retrospective cohort study.

BACKGROUND: The first angiotensin receptor/neprilysin inhibitor on the market, sacubitril-valsartan, has shown marked improvements in death and hospitalization for heart failure among adults, and is now approved for use in pediatric heart failure. While the ongoing PANORAMA-HF trial is evaluating the effectiveness of sacubitril-valsartan for pediatric patients with a failing systemic left ventricle, the enrollment criteria do not include the majority of pediatric heart failure patients. Additional studies are needed. METHODS: Using the TriNetX database, we performed a propensity score matched, retrospective cohort study to assess the incidence of a composite of all-cause mortality or heart transplant within 1 year. The 519 patients who received sacubitril-valsartan were compared to 519 matched controls who received an angiotensin converting enzyme inhibitor (ACE) or angiotensin II receptor blocker (ARB). RESULTS: There was no significant difference in the incidence of the composite outcome with sacubitril-valsartan over an ACE/ARB (13.3% vs 13.2%, p = 0.95), or among the components of mortality (5.0% vs 5.8%, p = 0.58) or heart transplantation (8.7% vs 7.5%, p = 0.50). Patients who were receiving full goal-directed medical therapy (14.4% vs 16.0%, p = 0.55) also showed no difference in the composite outcome. We observed a significantly increased incidence of hypotension (10% vs 5.2%, p = 0.006) and a trend toward reduced number of hospitalizations per year (mean (SD) 1.3 (4.4) vs 2.0 (9.1), p = 0.09). CONCLUSIONS: Sacubitril-valsartan is not associated with a decrease in the composite of all-cause mortality or heart transplantation within 1 year. Future studies should evaluate the possible reduction in hospitalizations and optimal dosing to minimize hypotension.

Impact of prolonged ischemic time on pediatric heart transplantation outcomes: Improved outcomes in the most recent era.

BACKGROUND: Impacts of ischemic time (IT) on pediatric heart transplant outcomes are multifactorial. We aimed to analyze the effect of prolonged IT on graft loss after pediatric heart transplantation. We hypothesized that graft survival with prolonged IT has improved across eras. METHODS: Patients <18 years old in the Pediatric Heart Transplant Society database were included (N=6,765) and stratified by diagnosis and era (1993-2004, 2005-2009, and 2010-2019). Severe graft failure (SGF) was defined as death, retransplant, or need for mechanical circulatory support in the first 7 days post-transplant. Descriptive statistical methods were used to compare differences between patient characteristics and IT. Kaplan-Meier survival analysis compared freedom from graft loss, rejection, and infection. Multivariable analysis was performed for graft loss and SGF (hazard and logistic regression modeling, respectively). RESULTS: Diagnoses were cardiomyopathy (N = 3,246) and congenital heart disease (CHD; N = 3,305). CHD were younger, more likely to have an IT ≥4.5 hours, and more likely to require extracorporeal membrane oxygenation or mechanical ventilation at transplant (all p < 0.001). Median IT was 3.6 hours (interquartile range 2.98-4.31; range 0-10.5). IT was associated with early graft loss (HR 1.012, 95% CI 1.005-1.019), but not when analyzed only in the most recent era. IT was associated with SGF (OR 1.016 95%CI 1.003-1.030). CONCLUSIONS: Donor IT was independently associated with an increased risk of graft loss, albeit with a small effect relative to other risk factors. Graft survival with prolonged IT has improved in the most recent era but the risk of SGF persists.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Impaired myocardial deformation persists at 2 years in offspring of mothers with diabetes mellitus.

BACKGROUND: A diabetic intrauterine environment has been proposed as a potential etiological mechanism for in utero programming of cardiac disease, and is associated with impaired fetal cardiac function. We aimed to assess cardiac function in offspring of mothers with diabetes mellitus (ODM) and determine whether fetal cardiac abnormalities persist during follow-up. METHODS: Longitudinal observational study to evaluate and compare myocardial function in 40 ODM to age-matched control offspring (CO). Myocardial deformation was measured using speckle-tracking echocardiography (STE). RESULTS: Significant differences were detected in global longitudinal strain (-20.9 ± 3.1 vs. -23.6 ± 2.2%; p = 0.001), global circumferential strain (-24.4 ± 3.9 vs. -26.9 ± 2.7%; p = 0.017), average radial strain (29.0 ± 9.8 vs. 37.1 ± 7.2%; p = 0.003), average longitudinal systolic strain rate (-1.24 ± 0.25/s vs. -1.47 ± 0.30/s; p = 0.011) and average circumferential systolic strain rate (-1.56 ± 0.37/s vs. -1.84 ± 0.37/s; p = 0.013) in comparison to CO up to 2 years of follow-up. Minimal differences were observed within ODM over the 2-year period. CONCLUSION: Impaired cardiac function in ODM persists during 2 years follow-up. Functional cardiac assessment might therefore be useful to detect these unfavorable changes, independent of screening for congenital heart disease or hypertrophic cardiomyopathy in this population. IMPACT: We demonstrate persistence of subclinical myocardial deformation abnormalities in offspring of mothers with diabetes mellitus from fetal life to early childhood years. These results extend the cellular observations in basic and translational research of developmental programming into the clinical realm. Persistence of subclinical myocardial deformation abnormalities may shed light on the known incidence of early cardiovascular disease in offspring of mother with diabetes. Cardiac myocardial strain assessment can be useful to detect these abnormalities, independent of screening for congenital heart disease or hypertrophic cardiomyopathy in this population.

Impact of race and health coverage on listing and waitlist mortality in pediatric cardiac transplantation.

BACKGROUND: Social factors like race and insurance affect transplant outcomes. However, little is known in pediatric heart transplantation. We hypothesized that race and insurance coverage impact listing and waitlist outcomes across eras. METHODS: Data from the Pediatric Heart Transplant Society multi-center registry prospectively collected between January 1, 2000-December 31, 2019 were analyzed. Patients were divided by race as Black, White and other and by insurance coverage at listing (US governmental, US private and non-US single payer systems (UK, Canada). Clinical condition at listing and waitlist outcomes were compared across races and insurance coverages. Categorical variables were compared using a chi-square test and continuous variables using the Wilcoxon rank sum test. Risk factors for waitlist mortality were examined using multiphase parametric hazard modeling. A sensitivity analysis using parametric hazard explored the interaction between race and insurance. RESULTS: At listing, compared to Whites (n = 5391) and others (n = 1167), Black patients (n = 1428) were older, more likely on US governmental insurance and had cardiomyopathy as the predominant diagnosis (p < 0.0001). Black patients were more likely to be higher status at listing, in hospital, on inotropes or a ventricular assist device (p < 0.0001). Black patients had significantly shorter time on the waitlist compared to other races (p < 0.0001) but had higher waitlist mortality (p = 0.0091), driven by the earlier era (2000-2009) (p = 0.0005), most prominently within the US private insurance cohort (p = 0.015). Outcomes were not different in other insurance cohorts or in the recent era (2010-2019). CONCLUSION: Black children are older and sicker at the time of listing, deteriorate more often and face a higher wait list mortality, despite a shorter waitlist period and favorable clinical factors, with improvement in the recent era associated with the recent US healthcare reforms. The social construct of race appears to disadvantage Black children by limiting referral, consideration or access to pediatric cardiac transplantation.

Cardiovascular Outcomes in Aortopathy: GenTAC Registry of Genetically Triggered Aortic Aneurysms and Related Conditions.

BACKGROUND: The GenTAC (Genetically Triggered Thoracic Aortic Aneurysm and Cardiovascular Conditions) Registry enrolled patients with genetic aortopathies between 2007 and 2016. OBJECTIVES: The purpose of this study was to compare age distribution and probability of elective surgery for proximal aortic aneurysm, any dissection surgery, and cardiovascular mortality among aortopathy etiologies. METHODS: The GenTAC study had a retrospective/prospective design. Participants with bicuspid aortic valve (BAV) with aneurysm (n = 879), Marfan syndrome (MFS) (n = 861), nonsyndromic heritable thoracic aortic disease (nsHTAD) (n = 378), Turner syndrome (TS) (n = 298), vascular Ehlers-Danlos syndrome (vEDS) (n = 149), and Loeys-Dietz syndrome (LDS) (n = 121) were analyzed. RESULTS: The 25% probability of elective proximal aortic aneurysm surgery was 30 years for LDS (95% CI: 18-37 years), followed by MFS (34 years; 95% CI: 32-36 years), nsHTAD (52 years; 95% CI: 48-56 years), and BAV (55 years; 95% CI: 53-58 years). Any dissection surgery 25% probability was highest in LDS (38 years; 95% CI: 33-53 years) followed by MFS (51 years; 95% CI: 46-57 years) and nsHTAD (54 years; 95% CI: 51-61 years). BAV experienced the largest relative frequency of elective surgery to any dissection surgery (254/33 = 7.7), compared with MFS (273/112 = 2.4), LDS (35/16 = 2.2), or nsHTAD (82/76 = 1.1). With MFS as the reference population, risk of any dissection surgery or cardiovascular mortality was lowest in BAV patients (HR: 0.13; 95% CI: 0.08-0.18; HR: 0.13; 95%: CI: 0.06-0.27, respectively). The greatest risk of mortality was seen in patients with vEDS. CONCLUSIONS: Marfan and LDS cohorts demonstrate age and event profiles congruent with the current understanding of syndromic aortopathies. BAV events weigh toward elective replacement with relatively few dissection surgeries. Nonsyndromic HTAD patients experience near equal probability of dissection vs prophylactic surgery, possibly because of failure of early diagnosis.

Early Improvement in Clinical Status Following Ventricular Assist Device Implantation in Children: A Marker for Survival.

While clinical status at the time of ventricular assist device (VAD) implant can negatively affect outcomes, it is unclear if early improvement after implant can have a positive effect. Therefore, the objectives of this study were to describe the clinical status of pediatric patients supported with a VAD and determine the impact of clinical status on the 1-month follow-up form on survival and ability to discharge. This was a retrospective analysis of data collected prospectively by the Pediatric Interagency Registry for Mechanical Circulatory Support Registry (Pedimacs) Registry. The Pedimacs database was queried for patients implanted between September 19, 2012, and September 30, 2019, who were alive on VAD support at 1-month postimplant on either a paracorporeal pulsatile or intracorporeal continuous device. Four factors on the 1-month follow-up were the focus of this study: mechanical ventilation, supplemental nutritional support, inotropic support, and ambulatory status. These factors were regarded as present if detected between 1-week and 1-month postimplant and were analyzed to determine their impact on survival following 1 month of VAD support and on successful discharge from hospital in patients with implantable continuous-flow devices. The eligible study cohort consisted of 414 patients with a mean age of 9.6 ± 6.2 years, weight of 40.8 ± 32.3 kg with the majority being male (56.7%) and having cardiomyopathy (68%). An isolated left ventricular assist device (LVAD) was the most common implant (85.5%). At implant, 40% were ventilated, 57% required nutritional support, 93% were on inotropes, and 58% were nonambulating. On the 1-month postimplant form, there were significant improvements in all four categories (14% ventilator support, 46% nutritional support, 53% on inotropes, and 25% nonambulating). However, there was no significant early change in the percentage of patients requiring supplemental nutrition in the paracorporeal pulsatile devices (88% vs. 82%; p = 0.2). Presence of these clinical parameters in early follow-up postimplant had a significant negative impact on survival and on the ability of patients with continuous-flow devices to be discharged. Presence of four specific clinical parameters early after VAD placement is associated with worse overall survival and an inability to discharge patients on VAD support. Ongoing work is needed for optimization of patients before implant and aggressive rehabilitation after implant to help improve long-term outcomes.

Epstein-Barr Virus Predicts Malignancy After Pediatric Heart Transplant, Induction Therapy and Tacrolimus Don't.

BACKGROUND: Patients after heart transplantation are at increased risk for malignancy secondary to immunosuppression and oncogenic viral infections. Most common among children is posttransplant lymphoproliferative disorder (PTLD), occurring in 5% to 10% of patients. We used a national database to examine the incidence and risk factors for posttransplant malignancy. METHODS: The United Network for Organ Sharing database was queried for pediatric (<18 years) heart transplant recipients from October 1987 through November 2019. Freedom from malignancy after transplant was assessed with Kaplan-Meier analysis. Cox regression was performed to generate hazard ratios (HRs) and 95% CIs for risk of malignancy development. RESULTS: Of 8581 pediatric heart transplant recipients, malignancy developed in 8.1% over median follow-up time of 6.3 years, with PTLD compromising 86.4% of the diagnosed cancers. The incidence of PTLD development was 1.3% at 1 year and 4.5% at 5 years. Older age at the time of transplant was protective against the development of malignancy (HR, 0.98; 95% CI, 0.96-0.99; P < .001), whereas a history of previous malignancy (HR, 1.9; 95% CI, 1.2-3.0; P = .007) and Ebstein-Barr virus (EBV) recipient-donor mismatch (HR, 1.7; 95% CI, 1.3-2.2; P < .001) increased the risk. Induction therapy, used in 78.9% of the cohort, did not increase malignancy risk (P = .355) nor did use of maintenance tacrolimus (P = .912). CONCLUSIONS: PTLD occurred after 7% of pediatric heart transplants, with risk increased by younger age and EBV mismatch, highlighting the importance of PTLD monitoring in EBV-seronegative recipients. Induction therapy, used in most of the pediatric heart transplants, does not seem to increase posttransplant malignancy nor does tacrolimus, the most commonly used calcineurin inhibitor.

Diaphragm Paralysis After Pediatric Cardiac Surgery: An STS Congenital Heart Surgery Database Study.

BACKGROUND: Previous single-center studies of diaphragm paralysis (DP) after pediatric cardiac surgery report incidence of 0.3% to 12.8% and associate DP with respiratory complications, prolonged ventilation and length of stay, and mortality. To better define incidence and associations between DP and various procedures and outcomes, we performed a multicenter study. METHODS: The Society of Thoracic Surgeons Congenital Heart Surgery Database was queried to identify children who experienced DP after cardiac surgery (2010-2018; 126 centers). Baseline characteristics and postoperative outcomes were compared between patients with and without DP as well as between patients who underwent plication and those who did not. Associations between center volume and center rates of DP and use of plication were also explored. RESULTS: A total of 2214 of 191,463 (1.2%) patients experienced DP. Postoperative DP portended worse outcomes, including mortality (5.6% vs 3.5%; P < .001), major morbidity (37.2% vs 10.7%; P < .001), tracheostomy (7.1% vs 0.9%; P < .001), prolonged mechanical ventilation (38.0% vs 7.8%; P < .001), and 30-day readmission (22.0% vs 10.6%; P < .001). A total of 1105 of 2214 (49.9%) patients with DP underwent plication. Patients who underwent plication were younger, were smaller, had more risk factors, and underwent more complex surgeries. Plication rates varied widely across centers. There was no correlation between center volume and center risk-adjusted rates of DP (r = .05, P = .5), nor frequency of plication (r = .08, P = .4). CONCLUSIONS: DP complicating pediatric heart surgery is rare but portends significantly worse outcomes. One-half of patients underwent plication. Center-level risk-adjusted rates of DP and plication are not associated with case volume. Significant variability in plication practices suggests a target for quality improvement.

Sports Participation and Exercise Restriction in Children with Isolated Bicuspid Aortic Valve.

Our study was to apply the 2015 American Heart Association/American College of Cardiology Athletic Participation Guidelines to a group of otherwise healthy school age children and young adults with bicuspid aortic valve (BAV) and describe the potential competitive sports restriction as they age. We performed a retrospective chart review of children and young adults aged 5 to 22 years with isolated BAV with at least two echocardiograms between 2000 and 2013. Using task force guidelines, exercise restriction was recommended for any of the following: (1) any dilation of the aortic root, (2) any dilation of the ascending aorta, (3) moderate aortic stenosis, (4) severe aortic regurgitation; (5) left ventricular dilation or (6) reduced shortening fraction. Of the 345 patients with isolated BAV, 202 were considered restricted at study entry. The final cohort included 123 children and young adults. Over the course of follow up, 36% (44 of 123) met restriction criteria. The most likely cause for restriction was aortic dilation (34%). Progression of aortic valve disease occurred in a minority of patients (3%). There were no reports of death, dissection or catheter or surgical based intervention. In conclusion, we found that strict adherence to current guidelines would result in restriction of more than 1/3 of school age children and young adults with BAV from some form of competitive athletics during school age years. Strict application of the current guidelines in this age group may lead to over-restriction of youths from competitive sports.

Diastolic Heart Failure in Patients With the Fontan Circulation: A Review.

The Fontan circulation, accomplished by direct surgical connection of the vena cavae to the pulmonary arteries, can be an effective palliation for patients with a single ventricle. However, failure of the Fontan circulation can result from mechanical obstruction, cardiac arrhythmias, increasing pulmonary vascular resistance, or deteriorating ventricular performance. Although systolic ventricular failure can usually be identified by a combination of clinical signs, symptoms, and imaging findings, diastolic ventricular dysfunction is likely an underrecognized cause of Fontan failure. Methods for detection of impaired diastolic function in a single ventricle are evolving, and established techniques appropriate in the biventricular heart lack validation in single ventricle. Association of biomarkers, cardiac magnetic resonance, and echocardiographic findings with invasively acquired pressure-volume loop data in humans may offer a way forward to accurate, noninvasive diagnosis. Today, therapy for severe diastolic ventricular dysfunction in the Fontan circulation is often disappointing and may require consideration of a ventricular assist device or even cardiac transplant. Progress toward improved outcomes of the Fontan palliation likely depends on successful innovation in primary prevention strategies and the development of more effective pharmacotherapy.

Open Thoracoabdominal Aortic Repair in Patients With Heritable Aortic Disease in the GenTAC Registry.

BACKGROUND: Although patients with various types of heritable aortopathy often require distal aortic repair, data are limited regarding the most extensive operations-open thoracoabdominal aortic aneurysm (TAAA) repairs. The objective of this multicenter registry study was to characterize TAAA repairs in a large cohort of patients with different heritable aortic diseases. METHODS: From the 3699 patients enrolled at 8 participating centers in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) Registry, we identified 155 open TAAA repairs in 142 unique patients. We examined data related to clinical characteristics, surgical techniques, and outcomes. RESULTS: The primary diagnoses included Marfan syndrome (n = 76; 54%), familial thoracic aortic aneurysm and dissections (n = 31; 22%), and Loeys-Dietz syndrome (n = 10; 7%). Most repairs were performed for aneurysms associated with aortic dissection (n = 110; 71%). The most common repairs involved the entire descending thoracic aorta with distal extension (21% Crawford extent I and 36% extent II). Adjuncts used during repair varied substantially. The operative mortality rate was 1.3%. Other complications included paraplegia (4%), acute renal failure (5%), and vocal cord paralysis (21%). Reoperation after TAAA repair was required in a subset of cases for early bleeding (n = 15; 10%) and late repair failure (n = 7; 5%). CONCLUSIONS: Open TAAA repairs are necessary in a variety of heritable aortic diseases. These patients often require extensive surgical repair, and a variety of adjunctive techniques are utilized. The risk of repair failure and the need for reoperation in a subset of patients support the need for vigilant long-term surveillance after repair.

Genetic aetiologies should be considered in paediatric cases of acute heart failure presumed to be myocarditis.

There are a variety of causes of acute heart failure in children including myocarditis, genetic/metabolic conditions, and congenital heart defects. In cases with a structurally normal heart and a negative personal and family history, myocarditis is often presumed to be the cause, but we hypothesise that genetic disorders contribute to a significant portion of these cases. We reviewed our cases of children who presented with acute heart failure and underwent genetic testing from 2008 to 2017. Eighty-seven percent of these individuals were found to have either a genetic syndrome or pathogenic or likely pathogenic variant in a cardiac-related gene. None of these individuals had a personal or family history of cardiomyopathy that was suggestive of a genetic aetiology prior to presentation. All of these individuals either passed away or were listed for cardiac transplantation indicating genetic testing may provide important information regarding prognosis in addition to providing information critical to assessment of family members.

Renal injury and recovery in pediatric patients after ventricular assist device implantation and cardiac transplant.

BACKGROUND: The use of ventricular assist devices (VADs) in children with heart failure may be of particular benefit to those with accompanying renal failure, as improved renal function is seen in some, but not all recipients. We hypothesized that persistent renal dysfunction at 7 days and/or 1 month after VAD implantation would predict chronic kidney disease (CKD) 1 year after heart transplantation (HT). METHODS: Linkage analysis of all VAD patients enrolled in both the PEDIMACS and PHTS registries between 2012 and 2016. Persistent acute kidney injury (P-AKI), defined as a serum creatinine ≥1.5× baseline, was assessed at post-implant day 7. Estimated glomerular filtration rate (eGFR) was determined at implant, 30 days thereafter, and 12 months post-HT. Pre-implant eGFR, eGFR normalization (to ≥90 mL/min/1.73 m2 ), and P-AKI were used to predict post-HT CKD (eGFR <90 mL/min/1.73 m2 ). RESULTS: The mean implant eGFR was 85.4 ± 46.5 mL/min/1.73 m2 . P-AKI was present in 19/188 (10%). Mean eGFR at 1 month post-VAD implant was 131.1 ± 62.1 mL/min/1.73 m2 , significantly increased above baseline (P < 0.001). At 1 year post-HT (n = 133), 60 (45%) had CKD. Lower pre-implant eGFR was associated with post-HT CKD (OR 0.99, CI: 0.97-0.99, P = 0.005); P-AKI was not (OR 0.96, CI: 0.3-3.0, P = 0.9). Failure to normalize renal function 30 days after implant was highly associated with CKD at 1 year post-transplant (OR 12.5, CI 2.8-55, P = 0.003). CONCLUSIONS: Renal function improves after VAD implantation. Lower pre-implant eGFR and failure to normalize renal function during the support period are risk factors for CKD development after HT.

Direct Echocardiographic Imaging of Berlin Heart Valves Can Aid in Early Detection of Berlin Heart Valve Dysfunction.

Mechanical circulatory support options remain limited for pediatric patients, especially neonates. The only U.S. Food and Drug Administration-approved pediatric device remains the Berlin Heart EXCOR, which unfortunately carries with it a 20-30% risk of neurologic complications, such as strokes. We demonstrate a new technique of direct echocardiographic color-Doppler imaging of the Berlin Heart valves to detect valve regurgitation. Increases in valve regurgitation could indicate issues with pump-valve thrombosis or increased afterload leading to valve insufficiency. Early recognition of valve thrombosis or insufficiency may reduce neurologic complications and lead to timely pump adjustments or replacement.

HeartWare Ventricular Assist Device Implantation for Pediatric Heart Failure-A Single Center Approach.

While pediatric HeartWare HVAD application has increased, determining candidacy and timing for initiation of pediatric VAD support has remained a challenge. We present our experience with a systematic approach to HVAD implantation as a bridge to pediatric heart transplantation. We performed a retrospective, single center review of pediatric patients (n = 11) who underwent HVAD implantation between September 2014 and January 2018. Primary endpoints evaluated were survival to heart transplantation, need for right ventricular assist device (RVAD) at any point, ongoing HVAD support, or death. Median patient age was 11 years (range: 3-16). Median BSA was 1.25 m2 (range: 0.56-2.1). Heart failure etiologies requiring support were dilated cardiomyopathy (n = 8), myocarditis (n = 1), congenital mitral valve disease (n = 1), and single ventricle heart failure (n = 1). Median time from cardiac ICU admission for heart failure to HVAD placement was 15 days (range 3-55), based on standardized VAD implantation criteria involving imaging assessment and noncardiac organ evaluation. The majority of patients (91%) were INTERMACS Level 2 at time of implant. Three patients (27%) had CentriMag RVAD placement at time of HVAD implantation. Two of these three patients had successful RVAD explanation within 2 weeks. Median length of HVAD support was 60 days (range 6-405 days). Among the 11 patients, survival during HVAD therapy to date is 91% (10/11) with 9 (82%) bridged to heart transplantation and one (9%) continuing to receive support. Posttransplant survival has been 100%, with median follow-up of 573 days (range 152-1126). A systematic approach to HVAD implantation can provide excellent results in pediatric heart failure management for a variety of etiologies and broad BSA range.

Scaling properties of pain intensity ratings in paediatric populations using the Faces Pain Scale-revised: Secondary analyses of published data based on the item response theory.

BACKGROUND: The Faces Pain Scale-revised (FPS-r) has been developed as an interval scale. For other pain measurement instruments, several studies found evidence for and against an interval level of measurement. OBJECTIVES: The primary aim of the current study was to evaluate the scale properties of the FPS-r using an item response theory approach. DESIGN: Secondary analysis of published data. SETTING: Three studies; Study 1 and study 2: One university hospital; Study 3: international pain registry. PARTICIPANTS: Study 1: n = 246, female: 41%, age: 11-18 years, 3 pain items; Study 2: n = 240, female: 43%, age: 11-18 years, 9 pain items; Study 3: n = 2266, female: 41%, age: 4-18 years, 3 pain items. METHODS: The rating scale model (interval scale), the graded response model (no interval scale, ordered response categories) and the partial credit model (no interval scale) were used to scale the data. RESULTS: In all three studies, the rating scale model was outperformed by the graded response model or the partial credit model in terms of model fit. Overlapping response categories were found in items associated with less pain. Response category widths were wider for categories associated with low pain intensity and smaller for categories associated with high pain intensities. Smallest response categories were 1%-67% smaller compared to the widest response category of the same item. CONCLUSION: According to these findings, the interval scale properties of the FPS-r may be questioned. Item response theory methods may help to solve the problem of missing linearity in pain intensity ratings using FPS-r.