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Shabana Z Shafy,1 Mohammed Hakim,1 Mauricio Arce Villalobos,1 Gregory D Pearson,2,3 Giorgio Veneziano,1 Joseph D Tobias1,4 1Department of Anesthesiology and Pain Medicine, Nationwide Children’s Hospital, Columbus, OH, USA; 2Department of Plastic Surgery, Nationwide Children’s Hospital, Columbus, OH, USA; 3Department of Plastic Surgery, The Ohio State University, Columbus, Ohio, USA; 4Department of Anesthesiology & Pain Medicine, The Ohio State University, Columbus, Ohio, USA Abstract: Duchenne muscular dystrophy (DMD), first described in 1834, is an X-linked dystrophinopathy, leading to early onset skeletal muscle weakness. Life expectancy is reduced to early adulthood as a result of involvement of voluntary skeletal muscles with respiratory failure, orthopedic deformities, and associated cardiomyopathy. Given its multisystem involvement, surgical intervention may be required to address the sequelae of the disease process. We present a 36-year-old adult with DMD, who required anesthetic care during surgical debridement of an ischial pressure sore. Given his significant respiratory muscle involvement, ultrasound-guided caudal epidural anesthesia was used instead of general during the surgical procedure. The technique and its applications are discussed, with particular emphasis on the feasibility and safety of using regional anesthetic techniques in patients with DMD. Keywords: Duchenne’s muscular dystrophy, caudal epidural, regional anesthesia

Background: The Norwood procedure with a modified Blalock-Taussig (MBT) shunt, the first palliative stage for single-ventricle lesions with systemic outflow obstruction, is associated with high mortality. The right ventricle-pulmonary artery (RVPA) shunt may improve coronary flow but requires a ventriculotomy. We compared the two shunts in infants with hypoplastic heart syndrome or related anomalies.Methods: Infants undergoing the Norwood procedure were randomly assigned to the MBT shunt (275 infants) or the RVPA shunt (274 infants) at 15 North American centers. The primary outcome was death or cardiac transplantation 12 months after randomization. Secondary outcomes included unintended cardiovascular interventions and right ventricular size and function at 14 months and transplantation-free survival until the last subject reached 14 months of age.Results: Transplantation-free survival 12 months after randomization was higher with the RVPA shunt than with the MBT shunt (74% vs. 64%, P=0.01). However, the RVPA shunt group had more unintended interventions (P=0.003) and complications (P=0.002). Right ventricular size and function at the age of 14 months and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups. Data collected over a mean (+/-SD) follow-up period of 32+/-11 months showed a nonsignificant difference in transplantation-free survival between the two groups (P=0.06). On nonproportional-hazards analysis, the size of the treatment effect differed before and after 12 months (P=0.02).Conclusions: In children undergoing the Norwood procedure, transplantation-free survival at 12 months was better with the RVPA shunt than with the MBT shunt. After 12 months, available data showed no significant difference in transplantation-free survival between the two groups. (ClinicalTrials.gov number, NCT00115934.) [ABSTRACT FROM AUTHOR]

Objective: Posterior cranial distraction (PCD) is a surgical technique to address craniosynostosis, especially in syndromic patients. The technique has the ability to significantly expand the cranium, while requiring minimal dural dissection, compared to cranial remodeling. Our goals were to determine the patient characteristics and surgical outcomes of PCD. The two questions that we sought to answer were: 1) What is the average published complication rate and the most common complications of PCD? and 2) How much intracranial volume expansion can one expect with PCD?, Design: A PubMed database search of articles on PCD was performed. Case reports and articles with overlapping patients were excluded. A systematic review was performed using the remaining articles., Main Outcome Measures: Patient data were extracted in order to determine the total number of patients, patients with a syndrome, types of syndromes, mean age at surgery, mean distraction distance, mean increase in intracranial volume, and complications., Results: 18 articles representing 325 patients were analyzed. A syndrome was present in 68.6% of patients. The mean age at time of surgery was 22.1 months. Mean distraction amount was 24.7 mm. Mean increase in intracranial volume was 253.2 cm 3 . The overall complication rate was 32.2%, with the most common complications being surgical-site infection, hardware-related complications and delayed wound healing., Conclusions: PCD is a powerful technique in the management of syndromic craniosynostosis, although complication rates are significantly higher than traditional remodeling techniques. Future studies should compare the effects of supratorcular and infratorcular osteotomies on intracranial volume, cosmesis and complications.

Background: Medical training is known to impose financial burden on trainees, which has been shown to contribute to burnout, even possibly compromising patient care. Financial literacy allows for management of financial situations affecting professional and personal life. The authors aimed to evaluate the financial status and knowledge among plastic surgery residents., Methods: A survey regarding finances and financial literacy of plastic surgery residents was sent to all the current accredited U.S. residency programs. The same survey was distributed internally. A descriptive analysis was performed, and multiple Fisher exact tests and a t test evaluated comparisons., Results: Eighty-six residents were included. Most trainees had a student loan (59.3%), with 22.1% having loans more than $300,000. A majority had at least one personal loan debt other than educational (51.1%). Residents with more debt were significantly less likely to pay off their balances monthly. A total of 17.4% of trainees had no plan for how to invest their retirement savings, whereas 55.8% reported not knowing how much they need to save to retire. One in five trainees did not feel prepared to manage personal finances/retirement planning after graduation, a majority had no formal personal finance education in their curriculum, and 89.5% agreed that financial literacy education would be beneficial. Our institutional data largely mirrored national data., Conclusions: Many residents are lacking in financial knowledge, despite most having significant debt. Additional financial literacy education is needed in plastic surgery training. Curricula development at an institutional or national society level are possible paths toward a coordinated response to this need., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Objective: To investigate health-related quality of life (HRQL) in children aged 2 to 7 years, who have undergone surgery for craniosynostosis., Design: Cross-sectional survey., Setting: A tertiary pediatric academic medical center., Participants: Children with craniosynostosis who underwent surgical correction, and who were 2-7 years old at the time of the study. Children from families that did not speak English were excluded., Interventions: Caregivers were asked to fill out the Pediatric Quality of Life Inventory (PedsQL) Core Parent Report and the PedsQL Cognitive Functioning Scale., Main Outcome Measures: PedsQL: Psychosocial Health Summary Score, Physical Health Summary Score, Total Core Score, Cognitive Functioning Scale Score. Scores range from 0 to 100, with higher scores reflecting greater QoLSubject factors: comorbidities, syndromic status, type of craniosynostosis, type of surgery., Results: The study included 53 subjects, of whom 13.2% had a syndrome. Core and cognitive scores did not depend on presence of a syndrome or suture involved. Subjects who underwent posterior cranial distraction achieved higher Total Core Scores than subjects who underwent open vault remodeling. Among subjects with sagittal craniosynostosis, there was a tendency for higher scores among children who underwent minimally-invasive surgery compared to those who underwent open vault remodeling., Conclusions: This study demonstrates similar HRQL among children with and without a syndrome, higher HRQL among children undergoing posterior cranial distraction than those undergoing open vault remodeling, and trends towards higher HRQL in children with sagittal craniosynostosis who underwent minimally-invasive surgery compared to those who underwent open vault remodeling., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Papillary hemangioma (PH) is a small, primarily dermal lesion occurring predominantly in the head and neck in both children and adults. Its signature characteristics are dilated thin-walled channels containing papillary clusters of mainly capillary-sized vessels and endothelial cytoplasmic eosinophilic inclusions. Given certain histopathologic similarities to congenital hemangioma which harbor mutations in GNAQ and GNA11 , we investigated whether similar mutations are present in PH. Seven PH specimens were studied. All presented in the first 4 years of life, with one being noted at birth. With the exception of one lesion, all were in the head and neck. Lesions were bluish and ranged in size from 0.5 to 2.8 cm. Four samples had GNA11 p.Q209L and 3 had GNAQ p.Q209L missense mutations. Mutations in GNA11 and GNAQ are associated with other types of somatic vascular lesions including capillary malformation, congenital hemangioma, anastomosing hemangioma, thrombotic anastomosing hemangioma, and hepatic small cell neoplasm. Shared mutations in GNA11 and GNAQ may account for some overlapping clinical and pathologic features in these entities, perhaps explicable by the timing of the mutation or influence of the germline phenotype., Competing Interests: Conflicts of Interest and Source of Funding: Supported by the Eunice Kennedy Shriver National Institute of Child Health and Human Development of the National Institutes of Health under Award Number F32HD107878. The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.5 mg twice daily). In this post hoc analysis, we evaluate whether subgroups within the population experienced a differential effect on exercise performance in response to treatment. The effect of udenafil on exercise was evaluated within subgroups defined by baseline characteristics, including peak oxygen consumption (VO 2 ), serum brain-type natriuretic peptide level, weight, race, gender, and ventricular morphology. Differences among subgroups were evaluated using ANCOVA modeling with fixed factors for treatment arm and subgroup and the interaction between treatment arm and subgroup. Within-subgroup analyses demonstrated trends toward quantitative improvements in peak VO 2 , work rate at the ventilatory anaerobic threshold (VAT), VO 2 at VAT, and ventilatory efficiency (VE/VCO 2 ) for those randomized to udenafil compared to placebo in nearly all subgroups. There was no identified differential response to udenafil based on baseline peak VO 2 , baseline BNP level, weight, race and ethnicity, gender, or ventricular morphology, although participants in the lowest tertile of baseline peak VO 2 trended toward larger improvements. The absence of a differential response across subgroups in response to treatment with udenafil suggests that the treatment benefit may not be restricted to specific sub-populations. Further work is warranted to confirm the potential benefit of udenafil and to evaluate the long-term tolerability and safety of treatment and to determine the impact of udenafil on the development of other morbidities related to the Fontan circulation.Trial Registration NCT0274115., (© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Congenital heart disease (CHD) is the most common birth defect in the United States. Neonates with CHD are often cared for by neonatologists in addition to cardiologists. However, there is a paucity of rigorous evidence and limited clinical trials regarding the management of neonates with CHD. In this review, we will describe some of the challenges of research in this field. The Pediatric Heart Network serves as an example of how a research network can effectively overcome barriers to conduct and execute well-designed multicenter studies., (Copyright © 2023 by the American Academy of Pediatrics.)

Background: Controversy remains regarding optimal management of Pierre Robin sequence (PRS). The goal of this study was to compare airway and feeding outcomes in infants with PRS who underwent surgical intervention, specifically mandibular distraction osteogenesis (MDO) or tongue-lip adhesion (TLA), or who had conservative management (CM) without surgery., Methods: All consecutive patients treated for PRS at a pediatric academic medical center, with at least one year follow-up, were included. Patients who underwent tracheostomy as an index procedure were excluded. Patients were divided into those who underwent MDO, TLA or CM. Feeding status and data from initial and follow-up polysomnograms were collected. Comparisons between groups were made using the Kruskal-Wallis test, followed by Mann-Whitney pairwise comparison with a Bonferroni correction, when appropriate., Results: 67 neonates were included. 19 underwent TLA, 29 underwent MDO and 19 underwent CM. The proportions of syndromic patients were similar between groups. Patients undergoing CM had the lowest baseline AHI (9.1), but there were no significant differences between TLA (20.1) and MDO (25.4). At follow-up, the three groups had similar mean AHI (MDO 1.3, TLA 4.2, CM 4.5). A similar proportion of patients achieved AHI 5 or less (TLA 89.5%, MDO 96.6%, CM 84.2%). At one year, there were no significant differences in weight percentiles or in risk of failure-to-thrive between groups. One patient from the TLA group required a tracheostomy., Conclusion: The three treatment modalities achieved high airway and feeding success rates. All three modalities should have a place in the armamentarium of the craniofacial surgeon.

Purpose: RAS genes (HRAS, KRAS, and NRAS) are commonly found to be mutated in cancers, and activating RAS variants are also found in disorders of somatic mosaicism (DoSM). A survey of the mutational spectrum of RAS variants in DoSM has not been performed., Methods: A total of 938 individuals with suspected DoSM underwent high-sensitivity clinical next-generation sequencing-based testing. We investigated the mutational spectrum and genotype-phenotype associations of mosaic RAS variants., Results: In this article, we present a series of individuals with DoSM with RAS variants. Classic hotspots, including Gly12, Gly13, and Gln61 constituted the majority of RAS variants observed in DoSM. Furthermore, we present 12 individuals with HRAS and KRAS in-frame duplication/insertion (dup/ins) variants in the switch II domain. Among the 18.3% individuals with RAS in-frame dup/ins variants, clinical findings were mainly associated with vascular malformations. Hotspots were associated with a broad phenotypic spectrum, including vascular tumors, vascular malformations, nevoid proliferations, segmental overgrowth, digital anomalies, and combinations of these. The median age at testing was higher and the variant allelic fraction was lower in individuals with in-frame dup/ins variants than those in individuals with mosaic RAS hotspots., Conclusion: Our work provides insight into the allelic and clinical heterogeneity of mosaic RAS variants in nonmalignant conditions., Competing Interests: Conflict of Interest The authors declare no conflicts of interest., (Copyright © 2022 American College of Medical Genetics and Genomics. Published by Elsevier Inc. All rights reserved.)

The National Heart, Lung, and Blood Institute convened a workshop in August 2021 to identify opportunities in pediatric and congenital cardiovascular research that would improve outcomes for individuals with congenital heart disease across the lifespan. A subsidiary goal was to provide feedback on and visions for the Pediatric Heart Network. This paper summarizes several key research opportunities identified in the areas of: data quality, access, and sharing; aligning cardiovascular research with patient priorities (eg, neurodevelopmental and psychological impacts); integrating research within clinical care and supporting implementation into practice; leveraging creative study designs; and proactively enriching diversity of investigators, participants, and perspectives throughout the research process., Competing Interests: Funding Support and Author Disclosures Dr Opotowsky is supported by the Heart Institute Research Core at Cincinnati Children's Hospital, and the Samuel and Molly Kaplan Fund for Adult Congenital Heart Disease. Dr Miyamoto is supported by the Children's Hospital Colorado Jack Cooper Millisor Chair in Pediatric Heart Disease. The views expressed in this manuscript are those of the authors and do not necessarily represent the views of the National Heart, Lung, and Blood Institute; the National Institutes of Health; the U.S. Department of Health and Human Services; or of other institutions. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2022 American College of Cardiology Foundation. All rights reserved.)

To improve psychosocial risk assessment and service provision for children with craniofacial conditions presenting for annual interdisciplinary team visits., Institute for Healthcare quality improvement model., U.S. pediatric academic medical center., Caregivers of children ages 0-17 years with craniofacial conditions presenting for 1692 team visits between August 2017 and July 2019., Key drivers included: (1) standardizing pre-visit triage processes; (2) administering the Psychosocial Assessment Tool-Craniofacial Version (PAT-CV); (3) utilizing PAT-CV scores in real time to add patients to psychosocial provider schedules; and (4) family education. Interventions included improving patient screening, increasing PAT-CV completion rate, altering clinic flow, providing patient and parent education about psychosocial services, and altering team member roles to fully integrate PAT-CV administration and scoring in the clinic., The primary outcome was the percentage of patients identified for psychosocial consultations via nurse triage, PAT-CV score, family or provider request who completed consultations. The secondary outcome was the percentage of patients completing needed psychosocial consultations based on elevated PAT-CV scores., Use of the PAT-CV resulted in an increase in the percentage of patients with elevated psychosocial risk who received a psychosocial consultation from 86.7% to 93.4%. The percentage of children receiving psychosocial consultation at their annual team visit due to elevated PAT-CV scores increased from 72% to 90%., Integrating a validated psychosocial risk screening instrument can improve risk identification and psychosocial consultation completion. A combination of risk screening approaches may be indicated to identify patients in need of psychosocial services.

Closed spinal dysraphism (SD) is a type of neural tube defect originating during early embryonic development whereby the neural tissue of the spinal defect remains covered by skin, often coinciding with markers of cutaneous stigmata. It is hypothesized that these events are caused by multifactorial processes, including genetic and environmental causes. We present an infant with a unique congenital midline lesion associated with a closed SD. Through comprehensive molecular profiling of the intraspinal lesion and contiguous skin lesion, an internal tandem duplication (ITD) of the kinase domain of the fibroblast growth factor receptor 1 ( FGFR1 ) gene was found. This ITD variant is somatic mosaic in nature as supported by a diminished variant allele frequency in the lesional tissue and by its absence in peripheral blood. FGFR1 ITD results in constitutive activation of the receptor tyrosine kinase to promote cell growth, differentiation, and survival through RAS/MAPK signaling. Identification of FGFR1 ITD outside of central nervous system tumors is exceedingly rare, and this report broadens the phenotypic spectrum of somatic mosaic FGFR1- related disease., (© 2022 Kautto et al.; Published by Cold Spring Harbor Laboratory Press.)

The second victim phenomenon is the distress felt by healthcare providers after a medical error. Although the phenomenon is a significant risk factor for burnout, little has been written about it in surgery, especially among residents., Methods: After institutional review board approval, a 27-question anonymous online survey was sent to plastic surgery residents throughout the United States, and to residents from all surgical specialties at our institution, for a total of 435 residents. Residents were asked to describe any adverse events they had experienced, and subsequent emotional sequelae., Results: The survey was returned by 125 residents (response rate 28.7%), of whom 53 were plastic surgery residents (42.4%) and 72 were from other surgical specialties (57.6%). In total, 110 (88%) described having been part of a medical error. An estimated 74 residents (34 from plastic surgery, 40 from other surgical specialties) provided a detailed description of the event. Sixty-four of them (86.5%) had subsequent emotional sequelae, most commonly guilt, anxiety, and insomnia. Only 24.3% of residents received emotional support. They rated other residents as the most important source of support, followed by faculty members and then family/friends., Conclusions: The second victim phenomenon seems to be common among surgical residents. The most important source of support for affected residents in our cohort was other residents. Given these findings, institutions should focus on fostering camaraderie among residents, building effective second victim response teams and training peer support specialists., (Copyright © 2022 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons.)

Background: The Long-terM OUtcomes after the Multisystem Inflammatory Syndrome In Children (MUSIC) study aims to characterize the frequency and time course of acute and long-term cardiac and non-cardiac sequelae in multisystem inflammatory syndrome in children associated with COVID-19 (MIS-C), which are currently poorly understood., Methods: This multicenter observational cohort study will enroll at least 600 patients <21 years old who meet the Centers for Disease Control and Prevention case definition of MIS-C across multiple North American centers over 2 years. The study will collect detailed hospital and follow-up data for up to 5 years, and optional genetic testing. Cardiac imaging at specific time points includes standardized echocardiographic assessment (all participants) and cardiac magnetic resonance imaging (CMR) in those with left ventricular ejection fraction (LVEF) <45% during the acute illness. The primary outcomes are the worst LVEF and the highest coronary artery z-score of the left anterior descending or right coronary artery. Other outcomes include occurrence and course of non-cardiac organ dysfunction, inflammation, and major medical events. Independent adjudication of cases will classify participants as definite, possible, or not MIS-C. Analysis of the outcomes will include descriptive statistics and regression analysis with stratification by definite or possible MIS-C. The MUSIC study will provide phenotypic data to support basic and translational research studies., Conclusion: The MUSIC study, with the largest cohort of MIS-C patients and the longest follow-up period to date, will make an important contribution to our understanding of the acute cardiac and non-cardiac manifestations of MIS-C and the long-term effects of this public health emergency., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Little is known about geographical variation in infant mortality due to congenital heart disease (CHD) and the social determinants of health that might mediate such variation. We aimed to examine US county-level estimates of infant mortality due to CHD to understand geographical patterns and factors that might influence variation in mortality., Methods: This US population-based cohort study used linked livebirth-infant death cohort files from the US National Center for Health Statistics from Jan 1, 2006, to Dec 31, 2015. All deaths attributable to congenital heart disease in infants in a given year were included. We used hierarchical Bayesian models to estimate rates of infant mortality due to congenital heart disease for all US counties. We mapped model-based estimates to explore geographical patterns. Covariates included infant sex, gestational age, maternal race and ethnicity, percentage of the county population below the poverty level, and proximity of the county to a US News & World Report 2015 top-50 ranked paediatric cardiac centre., Findings: From 2006 to 2015, 40 847 089 livebirths occurred, of which there were 13 988 infant deaths attributed to congenital heart disease, with an unadjusted infant mortality rate due to CHD of 0·34 per 1000 livebirths (95% CI 0·34-0·35). Kentucky and Mississippi had the greatest proportions of counties with a predicted rate of infant mortality due to CHD above the 95th percentile. All counties in Connecticut, Massachusetts, and Rhode Island had a predicted rate below the fifth percentile. In the model, lower mortality risk correlated with closer proximity to a top-50 ranked paediatric cardiac centre (odds ratio [OR] 0·890, 95% credible interval [CrI] 0·840-0·942), whereas higher mortality risk correlated with higher levels of poverty (OR 1·181, 95% CrI 1·125-1·239)., Interpretation: Substantial geographical variation exists in infant mortality due to CHD in the USA, highlighting the potential importance of bolstering care delivery for infants from economically deprived communities and areas remote from top-performing paediatric cardiac centres., Funding: None., Competing Interests: Declaration of interests We declare no competing interests., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Women are under-represented as leaders of cardiovascular randomized controlled trials, representing 1 in 10 lead authors of cardiovascular trials published in high-impact journals. Although the proportion of cardiovascular specialists who are women has increased in recent years, the proportion of cardiovascular clinical trialists who are women has not. This gap, underpinned by systemic sexism, has not been adequately addressed. The benefits of diverse randomized controlled trial leadership extend to patients and professionals. In this position statement, we present strategies adopted by some organizations to end gender inequality in research leadership. We offer an actionable roadmap for early-career researchers, scientists, academic institutions, professional societies, trial sponsors, and journals to follow, with the goal of harnessing the strength of women and under-represented groups as research leaders and facilitating a just culture in the cardiovascular clinical trial enterprise., Competing Interests: Funding Support and Author Disclosures Dr. Bozkurt has received personal fees from Bristol Myers Squibb, scPharmaceuticals, Baxter Healthcare Corporation, Sanofi, Relypsa, Abbott Vascular, and Liva Nova. Dr. Casadei has received personal fees from Roche Diagnostics and iRhythm. Dr. Lala has received personal fees from Zoll. Dr. Lam has received personal fees from Abbott Diagnostics, Applied Therapeutics, AstraZeneca, Bayer, Biofourmis, Boehringer Ingelheim, Boston Scientific, Corvia Medical, Cytokinetics, Darma Inc., JanaCare, Janssen Research & Development LLC, Medtronic, Menarini Group, Merck, MyoKardia, Novartis, Novo Nordisk, Radcliffe Group Ltd., Roche Diagnostics, Sanofi, Stealth BioTherapeutics, The Corpus, Vifor Pharma, and Web MD Global LLC. Dr. Mehran has received personal fees from Abbott Laboratories, Abiomed, Applied Therapeutics, AstraZeneca, Bayer, Beth Israel Deaconess, Bristol Myers Squibb, CERC, Chiesi, Concept Medical, CSL Behring, DSI, Medtronic, Novartis Pharmaceuticals, OrbusNeich, and Zoll. Dr. Pina has received personal fees from Vifor Pharma. Dr. Revkin has received personal fees from Pfizer. Dr. Volgman has received personal fees from Novartis, MSD/Bayer Global Advisory Board Member; and is a member of the Bristol Myers Squibb Foundation Diverse Clinical Investigator Career Development Program National Advisory Committee. Dr. Zannad has received steering committee and personal fees from Applied Therapeutics, Amgen, Bayer, Boehringer, Novartis, Janssen, Cellprothera, and CVRx; has received advisory board and personal fees from AstraZeneca, Vifor Fresenius, Cardior, Cereno pharmaceutical, Corvidia, Merck, Myokardia, NovoNordisk, and Owkin; has stock options at Cereno and G3Pharmaceutical; and is founder of the Global CardioVascular Clinical Trialists Forum. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Despite regulations currently in place, the incidence of lawnmower injuries in children has not decreased for several decades in the United States. In fact, studies in several countries show that the incidence of riding lawnmower injuries are actually on the rise worldwide. Those injuries tend to be devastating and limb-threatening. The purpose of this study was to evaluate a pediatric trauma center's experience with those injuries over the past 25 years., Methods: All patients who presented to a level I pediatric trauma center with injuries from lawnmowers between 1994 and 2019 were reviewed. Date of birth, gender, date of injury, mechanism of injury, type of lawnmower, and type of injury (including whether an open fracture, soft tissue defect, and/or amputation were present) were recorded., Results: A total of 142 pediatric patients were treated over the study period. The average age was 7.5 years. The three most common mechanisms of injury were being hit by a riding lawnmower moving forward, falling off a riding lawnmower, and being hit by a riding lawnmower moving backward. Of all patients, 68.3% sustained an open fracture and 38% required an amputation. Riding lawnmowers resulted in more operative procedures, longer hospital stays, and more soft tissue defects that require reconstruction than push mowers. Younger patients were at a higher risk to sustain proximal amputations (wrist/ankle or proximal) than older patients., Conclusion: Lawnmower injuries are devastating and largely avoidable. There are currently recommendations and regulations in the United States, which if followed, would prevent the vast majority of pediatric lawnmower injuries. Unfortunately, the incidence of these injuries has not decreased despite the current regulations. Broader public education is essential to decrease the incidence of serious lawnmower injuries in children. Level of Evidence: IV., (© 2021 Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons.)

Background: Minimal invasive suturectomy is one of the many surgical approaches to treat isolated single suture craniosynostosis. This approach can be technically challenging in metopic craniosynostosis given the narrow corridor and steep angle of the forehead. New instruments such as the Piezosurgery device (Mectron) have the potential to improve the ability to safely perform minimal invasive surgery in metopic craniosynostosis., Objective: To demonstrate the safety and efficacy of Piezosurgery technology in minimal invasive suturectomy for nonsyndromic metopic suture craniosynostosis and to describe our technique., Methods: A retrospective chart review was performed of all the single metopic suturectomies performed at our single institution from March 2018 to November 2019. Pre-, intra-, and postoperative data were collected to assess the safety of Piezosurgery., Results: The cohort consisted of 12 patients with an average of 95.25 d old and an average weight of 6.2 kg. A total of 91.7% were male, and 91.7% were Caucasian. There were no intraoperative or postoperative Piezosurgery device-related complications in the entire cohort., Conclusion: The use of the Piezosurgery instrument was safe in this cohort of minimal invasive metopic suturectomy. This device has greatly increased the ease of this procedure in our hands., (© Congress of Neurological Surgeons 2021.)

Introduction: We sought to compare outcomes and parental satisfaction between 2 approaches for sagittal craniosynostosis: strip craniectomy with spring-mediated skull remodeling (SMSR) and strip craniectomy with postoperative helmet (SCH)., Methods: Perioperative and outcome data for SMSR or SCH patients between September 2010 and July 2019 were retrospectively reviewed. A telephone survey was administered to parents of children who underwent both procedures., Results: A total of 62 children were treated for sagittal craniosynostosis by either SMSR (n = 45) or SCH (n = 17). The SCH group had a lower estimated blood loss (27 vs. 47.06 mL, p = 0.021) and age at surgery (13.0 vs. 19.8 weeks) than the SMSR group. Three patients underwent early springs removal due to trauma or dislodgement, all of whom converted to helmeting. Of the 62 children initially identified, 59 were determined to have an adequate follow-up time to assess long-term outcomes. The mean follow-up time was 30.1 months (n = 16) in the SCH group and 32.0 months in the SMSR group (n = 43, p = 0.39). Two patients in the SCH group and one in the SMSR group converted to open cranial vault reconstruction. Thirty parents agreed to respond to the satisfaction survey (8 SCH, 22 SMSR) based on a Likert scale of responses (0 being most dissatisfied possible, 4 most satisfied possible). Average satisfaction was 3.86/4.0 in the SCH group and 3.45/4.0 in the SMSR group. No parents in the SCH group would change to SMSR, while 3 of the 22 SMSR survey responders would have changed to SCH., Conclusions: Perioperative outcomes and average parental satisfaction were similar in both groups. Importance of helmet wear compliance and risk of spring dislodgement should be discussed with parents., (© 2021 S. Karger AG, Basel.)

The National Heart, Lung, and Blood Institute (NHLBI) has played an important role in funding the clinical science that supports many contemporary cardiology practice guidelines and in shaping the conduct of cardiovascular clinical trials. This Perspective outlines contemporary funding options as well as select important NHLBI policies, philosophy, and priorities., (Published by Elsevier Inc.)

Background: The Pediatric Heart Network Normal Echocardiogram Database Study had unanticipated challenges. We sought to describe these challenges and lessons learned to improve the design of future studies., Methods: Challenges were divided into three categories: enrolment, echocardiographic imaging, and protocol violations. Memoranda, Core Lab reports, and adjudication logs were reviewed. A centre-level questionnaire provided information regarding local processes for data collection. Descriptive statistics were used, and chi-square tests determined differences in imaging quality., Results: For the 19 participating centres, challenges with enrolment included variations in Institutional Review Board definitions of "retrospective" eligibility, overestimation of non-White participants, centre categorisation of Hispanic participants that differed from National Institutes of Health definitions, and exclusion of potential participants due to missing demographic data. Institutional Review Board amendments resolved many of these challenges. There was an unanticipated burden imposed on centres due to high numbers of echocardiograms that were reviewed but failed to meet submission criteria. Additionally, image transfer software malfunctions delayed Core Lab image review and feedback. Between the early and late study periods, the proportion of unacceptable echocardiograms submitted to the Core Lab decreased (14 versus 7%, p < 0.01). Most protocol violations were from eligibility violations and inadvertent protected health information disclosure (overall 2.5%). Adjudication committee reviews led to protocol changes., Conclusions: Numerous challenges encountered during the Normal Echocardiogram Database Study prolonged study enrolment. The retrospective design and flaws in image transfer software were key impediments to study completion and should be considered when designing future studies collecting echocardiographic images as a primary outcome.

Background: The Fontan operation creates a total cavopulmonary connection, a circulation in which the importance of pulmonary vascular resistance is magnified. Over time, this circulation leads to deterioration of cardiovascular efficiency associated with a decline in exercise performance. Rigorous clinical trials aimed at improving physiology and guiding pharmacotherapy are lacking., Methods: The FUEL trial (Fontan Udenafil Exercise Longitudinal) was a phase III clinical trial conducted at 30 centers. Participants were randomly assigned udenafil, 87.5 mg twice daily, or placebo in a 1:1 ratio. The primary outcome was the between-group difference in change in oxygen consumption at peak exercise. Secondary outcomes included between-group differences in changes in submaximal exercise at the ventilatory anaerobic threshold, the myocardial performance index, the natural log of the reactive hyperemia index, and serum brain-type natriuretic peptide., Results: Between 2017 and 2019, 30 clinical sites in North America and the Republic of Korea randomly assigned 400 participants with Fontan physiology. The mean age at randomization was 15.5±2 years; 60% of participants were male, and 81% were white. All 400 participants were included in the primary analysis with imputation of the 26-week end point for 21 participants with missing data (11 randomly assigned to udenafil and 10 to placebo). Among randomly assigned participants, peak oxygen consumption increased by 44±245 mL/min (2.8%) in the udenafil group and declined by 3.7±228 mL/min (-0.2%) in the placebo group ( P =0.071). Analysis at ventilatory anaerobic threshold demonstrated improvements in the udenafil group versus the placebo group in oxygen consumption (+33±185 [3.2%] versus -9±193 [-0.9%] mL/min, P =0.012), ventilatory equivalents of carbon dioxide (-0.8 versus -0.06, P =0.014), and work rate (+3.8 versus +0.34 W, P =0.021). There was no difference in change of myocardial performance index, the natural log of the reactive hyperemia index, or serum brain-type natriuretic peptide level., Conclusions: In the FUEL trial, treatment with udenafil (87.5 mg twice daily) was not associated with an improvement in oxygen consumption at peak exercise but was associated with improvements in multiple measures of exercise performance at the ventilatory anaerobic threshold., Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT02741115.

Background: The reliability of left ventricular (LV) systolic functional indices calculated from blinded echocardiographic measurements of LV size has not been tested in a large cohort of healthy children. The objective of this study was to estimate interobserver variability in standard measurements of LV size and systolic function in children with normal cardiac anatomy and qualitatively normal function., Methods: The Pediatric Heart Network Normal Echocardiogram Database collected normal echocardiograms from healthy children ≤18 years old distributed equally by age, gender, and race. A core lab used two-dimensional echocardiograms to measure LV dimensions from which a separate data coordinating center calculated LV volumes and systolic functional indices. To evaluate interobserver variability, two independent expert pediatric echocardiographic observers remeasured LV dimensions on a subset of studies, while blinded to calculated volumes and functional indices., Results: Of 3,215 subjects with measurable images, 552 (17%) had a calculated LV shortening fraction (SF) < 25% and/or LV ejection fraction (EF) < 50%; the subjects were significantly younger and smaller than those with normal values. When the core lab and independent observer measurements were compared, individual LV size parameter intraclass correlation coefficients were high (0.81-0.99), indicating high reproducibility. The intraclass correlation coefficients were lower for SF (0.24) and EF (0.56). Comparing reviewers, 40/56 (71%) of those with an abnormal SF and 36/104 (35%) of those with a normal SF based on core lab measurements were calculated as abnormal from at least one independent observer. In contrast, an abnormal EF was less commonly calculated from the independent observers' repeat measures; only 9/47 (19%) of those with an abnormal EF and 8/113 (7%) of those with a normal EF based on core lab measurements were calculated as abnormal by at least one independent observer., Conclusions: Although blinded measurements of LV size show good reproducibility in healthy children, subsequently calculated LV functional indices reveal significant variability despite qualitatively normal systolic function. This suggests that, in clinical practice, abnormal SF/EF values may result in repeat measures of LV size to match the subjective assessment of function. Abnormal LV functional indices were more prevalent in younger, smaller children., (Copyright © 2019 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Recent years have seen an exponential increase in the variety of healthcare data captured across numerous sources. However, mechanisms to leverage these data sources to support scientific investigation have remained limited. In 2013 the Pediatric Heart Network (PHN), funded by the National Heart, Lung, and Blood Institute, developed the Integrated CARdiac Data and Outcomes (iCARD) Collaborative with the goals of leveraging available data sources to aid in efficiently planning and conducting PHN studies; supporting integration of PHN data with other sources to foster novel research otherwise not possible; and mentoring young investigators in these areas. This review describes lessons learned through the development of iCARD, initial efforts and scientific output, challenges, and future directions. This information can aid in the use and optimisation of data integration methodologies across other research networks and organisations.

Objectives: Using the Psychosocial Assessment Tool-Craniofacial Version (PAT-CV), this study measured variation in psychosocial risk in families of children with craniofacial conditions by demographic and clinical characteristics, frequency of condition-related problems, concordance between caregivers' report of risk, and association between risk level and psychosocial service utilization., Design: Caregivers (n = 242) of 217 children with craniofacial conditions completed the PAT-CV, a psychosocial risk screener. Medical records were also abstracted., Results: The PAT-CV scores varied significantly by insurance type and syndromic versus nonsyndromic diagnosis type. Language problems were most commonly reported on the Craniofacial Problems subscale, though 13% to 56% of parents endorsed specific problems. Mothers and fathers reported similar risk levels. Families with past social work or behavioral health consults had higher PAT-CV scores than those without consultations., Conclusion: The PAT-CV efficiently screens for psychosocial risk and craniofacial-specific problems. This tool may help clinicians identify families in need of intervention.

As we commemorate the 70 th Anniversary of the National Heart, Lung, and Blood Institute (NHLBI) and celebrate important milestones that have been achieved by the Division of Cardiovascular Sciences (DCVS), it is imperative that DCVS and the Extramural Research community at-large continue to address critical public health challenges that persist within the area of Cardiovascular Diseases (CVD). The NHLBI's Strategic Vision, developed with extensive input from the extramural research community and published in 2016, included overarching goals and strategic objectives that serve to provide a general blueprint for sustaining the legacy of the Institute by leveraging opportunities in emerging scientific areas (e.g., regenerative medicine, omics technology, data science, precision medicine, and mobile health), finding new ways to address enduring challenges (e.g., social determinants of health, health inequities, prevention, and health promotion), and training the next generation of heart, lung, blood, and sleep researchers. DCVS has developed a strategic vision implementation plan to provide a cardiovascular framing for the pursuit of the Institute's overarching goals and strategic objectives garnered from the input of the broader NHLBI community. This plan highlights six scientific focus areas that demonstrate a cross-cutting and multifaceted approach to addressing cardiovascular sciences, including 1) addressing social determinants of cardiovascular health (CVH) and health inequities, 2) enhancing resilience, 3) promoting CVH and preventing CVD Across the lifespan, 4) eliminating hypertension-related CVD, 5) reducing the burden of heart failure, and 6) preventing vascular dementia. These priorities will guide our efforts in Institute-driven activities in the coming years but will not exclude development of other novel ideas or the support of investigator-initiated grant awards. The DCVS Strategic Vision implementation plan is a living document that will evolve with iterative dialogue with the NHLBI community and adapt as the dynamic scientific landscape changes to seize emerging opportunities., Competing Interests: Disclosures: None of the authors have relationships with industry or conflicts of interest to disclose. The views expressed in this article are those of the authors and do not necessarily represent the views of the National Heart, Lung, and Blood Institute; National Institutes of Health; or the United States Department of Health and Human Services.

Optimising short- and long-term outcomes for children and patients with CHD depends on continued scientific discovery and translation to clinical improvements in a coordinated effort by multiple stakeholders. Several challenges remain for clinicians, researchers, administrators, patients, and families seeking continuous scientific and clinical advancements in the field. We describe a new integrated research and improvement network - Cardiac Networks United - that seeks to build upon the experience and success achieved to-date to create a new infrastructure for research and quality improvement that will serve the needs of the paediatric and congenital heart community in the future. Existing gaps in data integration and barriers to improvement are described, along with the mission and vision, organisational structure, and early objectives of Cardiac Networks United. Finally, representatives of key stakeholder groups - heart centre executives, research leaders, learning health system experts, and parent advocates - offer their perspectives on the need for this new collaborative effort.

Background/Aims Identifying predictors of recruitment success in clinical trials, particularly prior to study launch, could contribute to higher study completion rates and improved scientific return on investment. This article evaluates the performance of clinical trials funded by the National Heart, Lung, and Blood Institute that began recruitment before and after implementation of National Heart, Lung, and Blood Institute's 2009 Accrual Policy and identifies study-related factors that predict recruitment success. Methods A retrospective analysis of National Heart, Lung, and Blood Institute's cardiovascular clinical trials with initial funding from 1996 to 2012 was performed to assess recruitment success. Success was defined as ≥100% enrollment of the proposed sample size within the duration initially proposed by investigators. Trials were assigned to categories (pre-policy vs post-policy) based on whether the first patient was enrolled before or after the 2009 Accrual Policy implementation. Potential determinants of successful recruitment were evaluated using multivariable logistic regression. Results Of 167 trials analyzed, 26.3% met the definition of success. Twenty-four trials (14.4%) were terminated early and 15 (62.5%) for insufficient recruitment. Trials failed due to <100% enrollment (22.8%), longer duration (19.8%), or both (31.1%). Trials testing behavioral interventions, those conducted within a National Heart, Lung, and Blood Institute-funded network, and those with normal controls were predictive of success. The proportion of successful clinical trials increased from 23% in the pre-policy era to 30% post-policy, although the difference was not statistically significant ( p = 0.29). Conclusion Enrollment success rates for National Heart, Lung, and Blood Institute's clinical trials are concerning. The 2009 National Heart, Lung, and Blood Institute Accrual Policy did not significantly improve trial success. Clinical trials testing behavioral interventions, those conducted within networks, and those with normal controls were predictive of recruitment success. Components of networks may provide model practices to help other trials attain success, including close attention to oversight activities such as recruitment plans, real-time enrollment monitoring, corrective action plans to address shortfalls, and close sponsor-investigator collaborations.

Objective: The purpose of this quality improvement initiative was to improve feeding and growth outcomes in infants with cleft lip and/or palate (CL/P)., Design: Institute for Healthcare Improvement quality improvement model., Setting: Large pediatric academic medical center in the Midwestern United States., Participants: One hundred forty-five infants with nonsyndromic CL/P ages 0 to 12 months., Interventions: Key drivers included (1) caregiver education and resources, (2) care coordination and flow, and (3) provider education and training. Interventions were designed around these themes and included targeting improved team communication, increased social work consultations, patient tracking, staff education, improved access to feeding equipment, and the launch of a new cleft palate feeding team., Main Outcome Measure(s): The primary outcome measure was the percentage of new patients with CL/P who met criteria for failure to thrive (FTT) per month. The secondary outcome measure was the frequency of hospitalization for infants with CL/P with a primary reason for admission of feeding difficulties or FTT., Results: The institutional FTT rate for infants with CL/P decreased from 17% to 7% ( P < .003). The frequency of hospitalization for FTT improved from once every 30 days to once every 118 days., Conclusions: Targeted interventions aimed at improving feeding efficiency and effectiveness, as well as changes in care delivery models, can reliably promote improvements in feeding and growth outcomes for infants with CL/P, even with psychosocial risk factors present.

Background: Attrition in plastic surgery is poorly characterized in the literature with previous data indicating that independent residents may have a higher rate of voluntary attrition with integrated residents having a higher rate of involuntary attrition. The aim of this study is to identify risk factors, note differences between pathways, and provide insight into resident attrition from plastic surgery residencies., Methods: An institutional review board-approved anonymous, multiple-choice and short answer, online survey regarding resident attrition was sent to all plastic surgery program directors (PDs) in the United States focusing from 2003 to 2013. Outcomes measured included demographics of the program and attritional resident, timing and reasons for attrition, and possibility of preventing attrition., Results: Thirty-three (35%) of 95 PDs responded. Average attrition rates were calculated at 2.15% for independent and 0.85% for integrated programs. Risk factors for attrition included being single, divorced, male, and having no dependents. One hundred percent of independent residents left by year 2, and 86% of integrated residents left by year 4. Lifestyle and loss of interest were most sited reasons for attrition. Most independent residents returned to their original field of training, whereas integrated residents were more likely to transfer to another integrated program. Only 17% of PDs believed attrition could have been prevented., Conclusions: Approximately 3.0% of all plastic surgery residents underwent attrition. Being single, male, divorced, or having no dependents increases the risk of attrition in plastic surgery residencies. This is the first study to demonstrate potential risks factors for plastic surgery residents undergoing attrition.

The Fontan operation creates a circulation characterized by elevated central venous pressure and low cardiac output. Over time, these characteristics result in a predictable and persistent decline in exercise performance that is associated with an increase in morbidity and mortality. A medical therapy that targets the abnormalities of the Fontan circulation might, therefore, be associated with improved outcomes. Udenafil, a phosphodiesterase type 5 inhibitor, has undergone phase I/II testing in adolescents who have had the Fontan operation and has been shown to be safe and well tolerated in the short term. However, there are no data regarding the long-term efficacy of udenafil in this population. The Fontan Udenafil Exercise Longitudinal (FUEL) Trial is a randomized, double-blind, placebo-controlled phase III clinical trial being conducted by the Pediatric Heart Network in collaboration with Mezzion Pharma Co, Ltd. This trial is designed to test the hypothesis that treatment with udenafil will lead to an improvement in exercise capacity in adolescents who have undergone the Fontan operation. A safety extension trial, the FUEL Open-Label Extension Trial (FUEL OLE), offers the opportunity for all FUEL subjects to obtain open-label udenafil for an additional 12 months following completion of FUEL, and evaluates the long-term safety and tolerability of this medication. This manuscript describes the rationale and study design for FUEL and FUEL OLE. Together, these trials provide an opportunity to better understand the role of medical management in the care of those who have undergone the Fontan operation., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To evaluate the effect of the Affordable Care Act (ACA) on the payer distribution and reimbursement rate for hand surgery at our institution., Methods: We reviewed records of 4,257 patients who underwent hand surgery at our institution between January 2008 and June 2016; 2,601 patients underwent surgery before the implementation of the ACA, and 1,656 patients after. Type of procedure, insurance status, amount of money billed, and amount collected were recorded., Results: After the implementation of the ACA, we performed fewer metacarpal fracture repairs, distal radius fracture repairs, and abscess incision and drainage procedures. We performed more endoscopic carpal tunnel releases. The proportion of uninsured patients decreased significantly (15% to 6.4%), and the proportion of patients on Medicare (15.4% to 20.3%) and Medicaid (9.5% to 17.8%) increased significantly. The overall reimbursement rate did not change significantly (32.3% to 30.3%) between the 2 time periods., Conclusions: After the implementation of the ACA, we observed a significant reduction in the number of uninsured patients and an increase in Medicaid and Medicare patients. However, this led to no significant change in reimbursement rates., Type of Study/level of Evidence: Economic and design analysis II., (Copyright © 2018 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)

The Pediatric Heart Network randomized trial of atenolol versus losartan in the Marfan syndrome showed no treatment differences in the rates of aortic-root growth or clinical outcomes. In this report we present treatment effects on aortic stiffness and determine whether baseline aortic stiffness predicts aortic-root growth and clinical outcomes. Echocardiograms at 0, 6, 12, 24, and 36 months from 608 subjects (6 months to 25 years) who met original Ghent criteria and had a maximum aortic-root z-score (ARz) >3 were centrally reviewed. Stiffness index (SI) and elastic modulus (EM) were calculated for aortic root and ascending aorta. Data were analyzed using multivariable mixed effects modeling and Cox regression. Heart rate-corrected aortic-root SI over 3 years decreased with atenolol but did not change with losartan (-0.298 ± 0.139 vs 0.141 ± 0.139/year, p = 0.01). In the entire cohort, above-median aortic-root SI (>9.1) and EM (>618 mm Hg) predicted a smaller annual decrease in ARz (p ≤0.001). Upper-quartile aortic-root EM (>914 mm Hg) predicted the composite outcome of aortic-root surgery, dissection, or death (hazard ratio 2.17, 95% confidence interval 1.02 to 4.63, p = 0.04). Crude 3-year event rates were 10.4% versus 3.2% for higher versus lower EM groups. In conclusion, atenolol was associated with a decrease in aortic-root SI, whereas losartan was not. Higher baseline aortic-root SI and EM were associated with a smaller decrease in ARz and increased risk for clinical outcomes. These data suggest that noninvasive aortic stiffness measures may identify patients at higher risk of progressive aortic enlargement and adverse clinical outcomes, potentially allowing for closer monitoring and more aggressive therapy., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: To evaluate the psychometric properties of the Psychosocial Assessment Tool-Craniofacial Version (PAT-CV), a screening instrument for psychosocial risk in families of children with craniofacial conditions, and to examine risk classification of patients in a craniofacial population., Design: Prospective, cross-sectional, single-center study., Setting: Interdisciplinary cleft lip and palate/craniofacial center at a US children's hospital., Participants: Parents/caregivers (n = 242) of 217 children ages 1 month to 17 years being treated for a congenital syndromic or nonsyndromic craniofacial condition completed the PAT-CV and validating measures from July 2015 to July 2016. The PAT-CV was completed by 121 caregivers a second time to assess test-retest reliability., Main Outcome Measures: PAT-CV, Child Behavior Checklist, Adult Self-Report, Pediatric Quality of Life Inventory, Craniofacial Experiences Questionnaire, and Family Environment Scale., Results: Construct validity of the PAT-CV was supported by significant correlations ( P < .001) between PAT-CV total and subscale scores and the validated measures. PAT-CV total scores categorized 59.9% of families within the universal risk group, 32.3% within the targeted risk group, and 7.8% within the clinical range. Good criterion validity was indicated by significantly higher scores ( P < .0001) obtained on the validated measures for those in the targeted and clinical risk groups. Internal consistency (Cronbach's α = 0.86) and test-retest reliability for the PAT-CV total score ( r = 0.77, P < .0001) were acceptable., Conclusions: The PAT-CV appears to be a reliable and valid screening instrument for psychosocial risk. Accurate identification of risk and implementation of appropriate interventions may contribute to improvements in medical and psychosocial outcomes.

Uhl's anomaly, first reported in 1952, is an extremely rare congenital cardiac defect characterized by partial or complete loss of the right ventricular myocardium and unknown etiology. Fewer than 100 cases have been described. The response to medical management is poor and there is no known ideal surgical approach or timing for treatment. We report the case of a previously active adolescent male presenting with cardiac arrest, who underwent successful bidirectional cavopulmonary anastomosis ("Glenn" anastomosis) with right atrial reduction and right ventricular free wall plication.

In an era of ongoing need for research to enable evidence-based care for the expanding population with congenital heart disease (CHD), economic fluctuations have impacted research funding. We characterize trends in NIH-funded CHD research from 2005 to 2015. We searched the NIH RePORTER database from 2005 to 2015 using the terms "congenital heart" and "cardiac morphogenesis". Projects were characterized by year, institute, mechanism, costs, type and topic, and funding trends were analyzed. From 2005 to 2015, NIH funded 633 CHD research projects with total costs of $991 million. The National Heart, Lung, and Blood Institute funded 83% of CHD projects (528, $857 million). The R01 mechanism was used for 45% of projects (288, $421 million). Projects were 70% basic/early translational research, 27% clinical research, and 3% both. Cardiac developmental biology was the most common topic (52%), followed by technology/therapy development (15%), and diagnosis/management (12%). The total number of CHD projects ranged from 153 to 221 per year (30-58 new projects/year), and costs per year ranged from $58 to $116 million. The number of projects and total costs increased until 2012, but decreased again thereafter. CHD research did not experience as much erosion as overall NIH purchasing power; in constant dollars, CHD research funding levels in 2015 were $12 million higher than those in 2005. The NIH supported a diverse portfolio of CHD projects from 2005 to 2015. Support of CHD research projects trended upward until 2012, but declined thereafter due to fiscal austerity measures.