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Acute aortic syndrome can be a fatal pathology if not diagnosed and managed early. Although acute aortic syndrome is more often a diagnosis of adulthood, it may occasionally afflict the pediatric patients. We herein present a case of a 5-year-old female that was discovered to have multiple acute and congenital aortic abnormalities after presenting to the emergency department with infectious symptoms and lower extremity pain. Acute aortic syndrome may not be a top differential consideration in children with acute chest pain; however, it is important to consider because delayed diagnosis and management can have fatal implications., (© 2021 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Competing Interests: Disclosures of Conflicts of Interest: J.W.R. disclosed no relevant relationships. S.S.W. Activities related to the present article: disclosed no relevant relationships. Activities not related to the present article: author receives book royalties from Elsevier. Other relationships: disclosed no relevant relationships. M.A.G. disclosed no relevant relationships. A.A.R. disclosed no relevant relationships.

The aim of this case study was to describe the distinct approaches used by physical education (PE) teachers to accommodate students with disabilities in New York elementary school PE classes. The participants included 1 adapted PE specialist, 5 PE teachers, and 5 elementary school students with various impairments. Through thematic analysis, observations and interviews revealed 3 main approaches: (a) normalized instruction-traditional curriculum with no differentiation in the program; (b) differentiated instruction-adaptations tailored specifically to the needs of each student with disability; and (c) universally designed instruction based on the principles of Universal Design for Learning (UDL) and accessibility to all students. Differentiated instruction, entailing modifications in the program and pedagogical accommodations, was the most prevalent approach at the research site, but lessons based on UDL principles were also observed. In association, the 2 approaches (differentiated instruction and UDL) represented significant resources to accommodate students with disabilities in PE.

Aims: This study explored how paediatric healthcare professionals experienced and coped with end-of-life conflicts and identified how to improve coping strategies., Methods: A questionnaire was distributed to all 2300 professionals at a paediatric university hospital, covering the frequency of end-of-life conflicts, participants, contributing factors, resolution strategies, outcomes and the usefulness of specific institutional coping strategies., Results: Of the 946 professionals (41%) who responded, 466 had witnessed or participated in paediatric end-of-life discussions: 73% said these had led to conflict, more frequently between professionals (58%) than between professionals and parents (33%). Frequent factors included professionals' rotations, unprepared parents, emotional load, unrealistic parental expectations, differences in values and beliefs, parents' fear of hastening death, precipitated situations and uncertain prognosis. Discussions with patients and parents and between professionals were the most frequently used coping strategies. Conflicts were frequently resolved by the time of death. Professionals mainly supported designating one principal physician and nurse for each patient, two-step interdisciplinary meetings - between professionals then with parents - postdeath ethics meetings, bereavement follow-up protocols and early consultations with paediatric palliative care and clinical ethics services., Conclusion: End-of-life conflicts were frequent and predominantly occurred between healthcare professionals. Specific interventions could target most of the contributing factors., (©2017 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization. Single-center, prospective, observational study of pediatric and young adult RCM patients undergoing assessment of diastolic parameters by simultaneous transthoracic echocardiogram (TTE) and invasive catheterization. Twenty-one studies in 15 subjects [median (IQR) = 13.8 years (7.0-19.2), 60% female] were acquired with median left ventricular end-diastolic pressure (LVEDP) 21 (IQR 18-25) mmHg. TTE parameters of diastolic function, including pulmonary vein A wave duration (r s  = 0.79) and indexed left atrial volume (r s  = 0.49), demonstrated significant positive correlation, while mitral valve A (r s  = -0.44), lateral e' (r s  = -0.61) and lateral a' (r s  = -0.61) velocities showed significant negative correlation with LVEDP. Lateral a' velocity (≤0.042 m/s) and pulmonary vein A wave duration (≥156 m/s) both had sensitivity and specificity ≥80% for LVEDP ≥ 20 mmHg. In pediatric and young adult patients with RCM, lateral a' velocity and pulmonary vein A wave duration predicted elevated LVEDP with high sensitivity and specificity; however, due to technical limitations the latter was reliably measured in 12/21 patients. These noninvasive parameters may have utility in identifying patients that require further assessment with invasive testing. These findings require validation in a multicenter prospective cohort prior to widespread clinical implementation.

Having a disability and being a teacher can be a critical site for examining practices associated with ability, competence, and pedagogy. While there is a growing literature base that examines the experiences of students with disabilities in physical education, there is virtually no research that examines the experiences of physical education teachers with disabilities. Using the capability approach, this article explores the experiences of a physical education teaching intern with a physical disability, significant school members, and the students he interacted with through interviews and documents. The results yielded 3 primary themes. The first, "the fluid nature of the disability discourse," demonstrated the complexity of disability and explored the contrast between static tendencies that stereotype disability and the disability experience. The second theme, "doing things my way," reflected the intern's need to distinguish himself as a teacher by defining contexts for experiencing competence. The third and final theme, "agent of change," explored how the intern's experiences as a teacher with a disability informed his educational narrative.

Advances in management of non-cardiac issues in Duchenne muscular dystrophy (DMD) have improved such that DMD-associated cardiac disease has become the leading cause of death for such patients. Cardiac dysfunction measured by standard transthoracic echocardiographic methods, e.g., fractional shortening (FS) and ejection fraction (EF), is rarely present during the first decade of life. The current study used transthoracic echocardiogram (TTE) to assess strain (ε), an indicator of regional ventricular function, in young DMD patients. A retrospective review of the TTE database was performed. TTE results from DMD patients <8 years (n = 63) performed during 2009 to 2010 were compared with TTE results from an unaffected control group (n = 61). Feature tracking analysis software was used to measure total circumferential strain (ε cc) as well as segmental ε cc based on the American Society of Echocardiography 16-segment model. Although there were no differences in FS, the absolute value for left-ventricular (LV) ε cc at the mid-chamber level was decreased in DMD (-21.7 % ± 3.8 % vs. -19.8 % ± 4.2 %, p < 0.01; unaffected vs. DMD). Segmental ε(cc) was similarly affected in the anteroseptal segment (-23.0 % ± 6.1 % vs. -18.9 % ± 7.0 %, p = 0.001; controls vs. DMD), the inferior segment (-20.7 % ± 5.16 % vs. -17.7 % ± 6.1 %, p = 0.003; controls vs. DMD), and the inferolateral segment (-18.3 % ± 6.2 % vs. -15.9 % ± 6.7 %, p = 0.04; controls vs. DMD). In the present study we demonstrate both total and segmental LV ε cc (anteroseptal, inferior, and inferolateral segments) abnormalities at the mid-chamber level in a large group of young DMD patients with normal FS. These novel findings substantiate that the disease process is present and results in abnormal myocardial function before standard measures detect global dysfunction.

Malonyl coenzyme A (CoA) decarboxylase (MCD) deficiency is a rare autosomal recessive organic acidemia characterized by varying degrees of organ involvement and severity. MCD regulates fatty acid biosynthesis and converts malonyl-CoA to acetyl-CoA. Cardiomyopathy is 1 of the leading causes of morbidity and mortality in this disorder. It is unknown if diet alone prevents cardiomyopathy development based in published literature. We report a 10-month-old infant girl identified by newborn screening and confirmed MCD deficiency with a novel homozygous MLYCD mutation. She had normal echocardiogram measurements before transition to high medium-chain triglycerides and low long-chain triglycerides diet. Left ventricular noncompaction development was not prevented by dietary interventions. Further restriction of long-chain triglycerides and medium-chain triglycerides supplementation in combination with angiotensin-converting enzyme inhibitors helped to improve echocardiogram findings. Patient remained asymptomatic, with normal development and growth. Our case emphasizes the need for ongoing cardiac disease screening in patients with MCD deficiency and the benefits and limitations of current dietary interventions.

Background: Pediatric restrictive cardiomyopathy carries a poor prognosis secondary to a high risk of sudden death previously attributed to ventricular tachyarrhythmias. The extent of conduction abnormalities in this population and their relationship to life-threatening events has not been previously reported., Methods and Results: A retrospective study of pediatric patients with restrictive cardiomyopathy diagnosed between April 1994 and May 2011 was performed. Demographic, cardiac, and ECG characteristics and the mechanisms of serious arrhythmic events (death or episode of acute hemodynamic compromise thought to be secondary to arrhythmia) were evaluated. Sixteen patients (1-17 years of age) were reviewed, with 5 sudden cardiac events noted, including 4 deaths. Two deaths were caused by development of acute heart block; another patient with syncope had intermittent heart block and survived as the result of pacing features of an implanted defibrillator system. The median PR interval (222 versus 144 ms; P<0.01) and the QRS duration (111 versus 74; P=0.01) were significantly longer in those who had an acute cardiac event. Older age at presentation was associated with sudden cardiac events (P<0.01). No other functional or echocardiographic variables were associated with a sudden cardiac event., Conclusions: Pediatric patients with restrictive cardiomyopathy are at risk for acute high-grade heart block, and, in this cohort, bradycardic events represented a significant portion of all arrhythmic events. Aggressive ECG monitoring strategies looking for conduction system disease should be ongoing in all patients with restrictive cardiomyopathy. Implantation of a defibrillator/pacemaker should be considered as prophylactic management.

Qualitative research methods were used to explore the factors that informed general and adapted physical education teachers' coteaching practices within an inclusive high school physical education program. Two physical education teachers and one adapted physical education teacher were observed over a 16-week period. Interviews, field notes, and documents were collected and a constant comparative approach was used in the analysis that adopted a social model framework. Primary themes included community as the cornerstone for student learning, core values of trust and respect, and creating a natural support structure. Coteaching practices existed because of the shared values of teaching, learning, and the belief that all students should be included. Recommendations include shifting orientations within professional preparation programs to account for the social model of disability.

Background: We hypothesized that recombinant B-type natriuretic peptide (BNP) (nesiritide) could improve urine output and neurohormonal markers of heart failure without worsening renal function in pediatric patients., Methods and Results: We analyzed our experience involving 140 nesiritide infusions in 63 consecutive children. Serum levels of BNP and electrolytes were measured before and after therapy. Dosing was begun at 0.01 mcg.kg.min without a bolus and titrated to a maximum of 0.03 mcg.kg.min, in 0.005-mcg.kg.min increments. Blood pressure, heart rate, and heart rhythm were monitored. In a substudy, 20 patients with decompensated cardiomyopathy-related heart failure received 72 hours of nesiritide with prospective assessment of aldosterone, norepinephrine, plasma renin, and endothelin-1 levels before and after therapy. The heart rate decreased significantly (P = .001). Urine output increased significantly on Days 1 and 3 (P < or = .001 and .004, respectively). The mean serum creatinine level decreased from 1.135 to 1.007 mg/dL (P < or = .001). In the substudy, aldosterone levels decreased from 37.5 +/- 57.1 to 20.5 +/- 41.9 ng/dL (P = .005). Plasma renin, norepinephrine, and endothelin-1 levels decreased nonsignificantly. Two infusions were discontinued because of hypotension., Conclusions: Nesiritide safely treated decompensated heart failure in children. Increased urine output reflected improving renal function. Improved neurohormonal markers were seen after 72 hours of therapy, and complications were uncommon.

Objectives: It is known that levels of brain natriuretic peptide predict outcomes of treatment for adults with decompensated heart failure. We hypothesized that it could predict outcomes in children with this condition., Methods: We divided retrospectively 82 patients with serial measurements of brain natriuretic peptide into 3 groups: those who survived and did not need readmission within less than 60 days; those who survived but needed readmission within less than 60 days; and those who died in hospital or within less than 60 days. Initial and final levels of the peptide correlated with adverse outcomes., Results: The percent change in level of the peptide was minus 78 percent, minus 38 percent, and 138 percent in the readmission-free group, the readmitted, and nonsurviving groups, respectively. Final levels were significantly lower in the readmission-free group than in the readmitted and nonsurviving groups (p equals 0.013 and p is less than 0.00001, respectively) and in the readmitted group than in the nonsurvivors (p equals 0.013). On univariate analysis, the final level, the change in level, and the percentage change in level significantly predicted outcomes (p equals 0.0002, 0.0072 and 0.0005, respectively). On multivariate analysis, only the final level of the peptide significantly predicted outcomes (p equals 0.01)., Conclusions: A final level of brain natriuretic peptide of greater than or equal to 760 picograms per millilitre strongly predicted an adverse outcome. Patients with higher final levels may be at higher risk of death and readmission, suggesting that this variable effectively predicts the response to treatment and prognosis in children with heart failure.

Background: Obesity and cachexia are risk factors for adverse outcomes in adult transplant patients. However, little is known about the effects of body mass index (BMI) on outcomes in pediatric heart transplant patients., Methods: Patients > 2 years of age undergoing heart transplantation from 1985 to 2004 at our institution were included in this study. BMI was assessed at the time of transplant and at 1 year post-transplant. Long-term outcomes were assessed by weight group., Results: The cohort included 105 patients with a mean age at transplant of 9.6 +/- 5.3 years. The mean BMI percentile at the time of transplant was 39 +/- 34, with 22 (21%) patients underweight (< 5th percentile) and 8 (8%) patients overweight (> or = 95th percentile). Among patients surviving to 1 year (n = 92), the mean BMI percentile increased to 57 +/- 33 (p < 0.05). Overall graft survival was decreased in patients underweight at transplant, mean 6.7 years (95% confidence interval [CI] 3.6 to 9.9), vs normal weight patients, mean 10.6 years (95% CI 8.8 to 12.4) (p < 0.05). Patients overweight at transplant did not have decreased graft survival. Neither low nor high BMI at 1 year post-transplant was associated with adverse outcomes. On multivariate analysis, being underweight at transplant was an independent predictor of decreased graft survival (p = 0.03)., Conclusions: Weight gain was nearly universal post-transplant with only 4% of patients underweight at 1 year. In the small number of patients overweight at transplant, graft survival was similar to normal-weight patients. Conversely, being underweight at transplant was an independent predictor of decreased graft survival.

This study sought to determine the potential of recombinant B-type natriuretic peptide (nesiritide) for the treatment of pediatric decompensated heart failure. Nesiritide is a widely used and effective treatment for decompensated heart failure (HF) in adults, but its safety and efficacy in pediatric patients is unclear. Outcomes of 55 separate nesiritide infusions of varying durations in 32 patients (13 males and 19 females; mean age, 8.01 years; range, 0.01-20.4) were evaluated prospectively. All patients received nesiritide in the intensive care unit. The starting dose (0.01 microg/kg/min) was titrated to a maximum of 0.03 microg/kg/min. All patients were monitored for clinical signs and symptoms, hemodynamics, urine output, electrolytes, oxygen requirements, and oral intake. Functional status was assessed by patients and/or their parents. All patients successfully underwent initiation and titration of nesiritide infusion. No hypotension or arrhythmias were noted during 478 cumulative days of therapy. Nesiritide was given safely with vasoactive medications. Mean urine output improved from 2.35 +/- 1.71 cc/kg/hr on the day before nesiritide initiation (baseline) to 3.10 +/- 1.94 cc/kg/hr on day 4 of treatment (p < 0.01). Serum creatinine decreased from 1.04 to 0.92 mg/dl (p = 0.096), mean central venous pressure from 13 to 7 mmHg (p = 0.018), and mean weight from 30.4 to 29.7 kg (p < 0.001) with therapy. Thirst, as subjectively assessed by patients old enough to respond, decreased with infusion in 31 of 42 cases (74%). Mean New York Heart Association functional class improved significantly (p < 0.001). Nesiritide infusion, alone or in combination, is a safe treatment for decompensated HF in pediatric patients. It is associated with decreased thirst and improved urine output and functional status, and it may be efficacious in the treatment of pediatric HF.

Angiotensin-converting enzyme (ACE) inhibitors have become an important part of the pharmacologic armamentarium in the battle against treatment of ventricular dysfunction. There have been a number of large controlled, randomized trials in adults with both asymptomatic and symptomatic ventricular dysfunction, which confirm the safety and efficacy of this category of drugs for the treatment of this potentially lethal condition. ACE inhibitors may be used to treat infants, children and adolescents with asymptomatic and symptomatic ventricular dysfunction as well. The data supporting their use in children is less complete than that concerning the treatment of adults. We review here the various causes of ventricular dysfunction and congestive heart failure (CHF) in infants, children, and adolescents; the data available regarding treatment of these conditions with ACE inhibitors, and the safety and efficacy of these drugs for the various conditions. The pharmacokinetics and proposed mechanisms of action of ACE inhibitors in children are reviewed, as are speculated long-term results of ACE inhibitor use in cohorts of growing children. Recommendations are made for future studies.

The Pediatric Cardiomyopathy Registry (PCMR) was established to describe the epidemiologic features and clinical course of selected cardiomyopathies in patients aged 18 years or younger and to promote the development of etiology-specific treatments. Sixty-one private and institutional pediatric cardiomyopathy practices in the United States and Canada were recruited to participate in the PCMR. The registry consists of a prospective, population-based cohort of patients in 2 regions (New England and the Central Southwestern United States) and a retrospective cohort of patients diagnosed between 1991 and 1996. Annual follow-up data are collected on all patients. As of June 1999, the PCMR consisted of 337 prospectively identified and 990 retrospectively identified patients. The PCMR has demonstrated the feasibility of establishing a large database of sociodemographic and clinical information on children with pediatric cardiomyopathy. Through this cooperative effort, the PCMR will obtain precise estimates of the incidence of pediatric cardiomyopathy and a better understanding of the natural history of this disease.

The embryological development of the heart is an awesome and complex process. The heart is formed from groups of cells that coalesce into sheets of tissue. These sheets fuse to form a tube that grows into the atria and ventricles. The twists and turns of the developing heart tissue and the simultaneous septation of atria, ventricles, and truncus, along with a shifting of the atrioventricular canal and conus then position the portions of the heart in alignment to allow normal blood flow. Errors at any stage of embryological formation can result in specific congenital defects. Understanding the development of the heart in utero can provide the pediatric nurse a basis for understanding the physiological effects of embryological failures that result in congenital heart defects in the infant and child.

Anthracyclines, potent cytotoxic agents used to treat a broad spectrum of malignancies, are limited in their use by an attendant risk of cardiotoxicity. Malignancies affect all age ranges, and anthracyclines are used in all age ranges, thereby exposing a broad population of patients to the development of heart disease. For some treated patients, anthracyclines affect cardiac muscle, resulting in cardiomyopathy. The type and degree of cardiomyopathy, as well as when during or after treatment the condition occurs, are dependent on what risk factors are present. Age is a major risk factor. Children and adults may develop restrictive and dilated cardiomyopathy. The length of subsequent survival and amount of subsequent somatic growth may influence late anthracycline-associated cardiac outcome. Early cardiotoxicity, occurring during or within 1 year of completion of treatment, is the largest risk factor for the development of late cardiotoxicity, which occurs beyond a year of completion of treatment. Risk factors, which appear to be specific for early cardiotoxicity in children, include black race, trisomy 21, and the use of amsacrine therapy after anthracycline therapy. More cardiotoxic effects are seen in survivors of childhood cancer, the longer from completion of treatment a patient is followed. Cumulative as well as peak anthracycline doses affect adults and children alike, and cardiotoxicity occurs early and late. In adults, left ventricular contractility is affected by anthracyclines. Children may manifest impairment of left ventricular contractility and increased afterload due to thinning of left ventricular walls. Patients with an early presentation of depressed left ventricular contractility are likely to show progression of cardiac disease with time. In addition, female gender appears to affect early and late cardiotoxicity in both adults and children, although this risk factor has been described predominantly in the survivors of childhood cancer. Thus, although anthracycline chemotherapy has improved overall survivorship of patients with cancer, there is a significant risk of cardiotoxicity associated with this class of drugs.