Earlier reviews have reported unacceptably high incidence of pediatric heart transplant (PHT) waiting list mortality. An increase in ventricular assist devices (VAD) suggests a potential positive ...effect. This study evaluated PHT waiting list mortality in the era of pediatric VADs.
United Network of Organ Sharing (UNOS) database from 1999 to 2012 showed 5,532 pediatric candidates (aged ≤ 18 years) actively listed for PHT: 2,191 were listed in 1999 to 2004 (Era 1) and 3,341 were listed in 2005 to 2012 (Era 2).
Waiting list mortality was lower in Era 2 (8%) vs Era 1 (16%; p < 0.001). VAD therapy was used more frequently in Era 2 (16%) than in Era 1 (6%; p < 0.001) and was associated with better waiting list survival (p < 0.001). There were more UNOS Status 1A patients in Era 2 (80%) vs Era 1 (68%; p < 0.001). Independent predictors of waiting list mortality included weight < 10 kg (odds ratio OR, 2.7 95% confidence interval CI, 1.1-6.9), congenital heart disease diagnosis (OR, 2.4; 95% CI, 1.9-3.0), blood type O (OR, 2.2; 95% CI, 1.8-2.8), extracorporeal membrane oxygenation (OR, 1.5; 95% CI, 1.1-2.2), mechanical ventilation (OR, 1.8; 95% CI, 1.4-2.3), and renal dysfunction (OR 1.6; 95% CI, 1.2-2.0). Independent predictors of survival on the waiting list included VAD therapy (OR 4.2; 95% CI, 2.4-7.6), cardiomyopathy diagnosis (OR 3.3; 95% CI, 2.4-4.6), blood type A (OR, 2.2; 95% CI, 1.8-2.8), UNOS list Status 1B (OR, 1.9; 95% CI, 1.2-3.0), listed in Era 2 (OR 1.8; 95% CI, 1.4-2.2), and white race (OR 1.3; 95% CI, 1.1-1.6).
Despite an increase in the number of children listed as Status 1A, there was more than a 50% reduction in waiting list mortality in the new era. Irrespective of other factors, patients supported with a VAD were 4 times more likely to survive to transplant.
Pediatric patients with left ventricular noncompaction (LVNC) and severe ventricular dysfunction are at risk for sudden death. The aims of this study were to (1) evaluate outcomes, (2) describe ...arrhythmic burden on Holter monitoring, and (3) analyze the utility of Holter monitoring and its impact on care in pediatric patients with LVNC and preserved or mild ventricular dysfunction. This was a retrospective study including patients <21 years of age with LVNC and ejection fractions ≥45%. Demographic and outcome data were analyzed. Individual and cumulative Holter data were evaluated for all patients. Arrhythmias, conduction system disease, and symptoms were analyzed for each Holter recording. The incidence of significant findings and the impact on care were determined for each study. Outcome and Holter data were compared between patients on the basis of the ejection fraction (≥55% normal or ≥45% to <55% mild). This study included 72 patients, 65 with normal function and 7 with mild dysfunction (mean age 13 years). There was a single death in the cohort, which was sudden in nature. Simple ventricular ectopy was common on Holter monitoring and more common in patients with mild dysfunction (86% vs 27%, p = 0.005). Significant Holter findings (4% vs 6%) and changes to patient care (2% vs 4%) improved with cumulative Holter monitoring. In conclusion, in contrast to patients with severe dysfunction, pediatric patients with LVNC and normal or mild dysfunction have significantly better outcomes. However, worsening LV systolic function was correlated with increasing ventricular ectopy. The role of Holter monitoring is unknown, but it may have utility in patient care if used as part of ongoing screening.
Abstract Background Acute fulminant myocarditis is a life-threatening disease in children. A limited number of reports suggest that mechanical circulatory support (MCS) may be used to successfully ...bridge children with acute fulminant myocarditis to recovery or transplantation. We evaluated the effectiveness of MCS in children with myocarditis and identified risk factors associated with adverse outcomes. Methods and Results Between 2001 and 2009, 16 children were treated for myocarditis at our institution; each child received MCS provided by extracorporeal membrane oxygenation, ventricular assist device(s), or both. Of these patients, 75% (12/16) survived: 7 recovered ventricular function, and 5 underwent successful orthotopic heart transplantation. In patients who were bridged to recovery, mean left ventricular ejection fraction significantly improved from initiation to termination of MCS (20 ± 9.3% to 62 ± 5%; P = .0004). Viral pathogens were detected in 11 patients by polymerase chain reaction, and viral presence was associated with death or need for transplantation ( P = .011). Upon histologic analysis, absence of viral infection and lack of myocardial inflammation were associated with recovery ( P values .011 and .044, respectively). Conclusions In children with acute fulminant and persistent myocarditis, MCS is a life-saving treatment strategy, particularly in the absence of viral infection.
Electrocardiograms continue to be part of screening programs for athletes and familial hypertrophic cardiomyopathy (HC). Whether electrocardiographic (ECG) findings of left ventricular (LV) ...hypertrophy can distinguish between healthy populations and those with HC remains unclear. We sought to (1) analyze the relation between ECG voltage and LV mass in patients with HC and (2) evaluate ECG characteristics of patients with phenotypical HC. Retrospective cohort of patients with HC aged 13 to 18 years. Relation between ECG voltages (RV6 , SV1 , and RV6 + SV1 ) and echocardiogram measurements of LV mass was investigated using smoothing splines to display relations and compared with those in a prospectively obtained population of adolescents. Frequency of abnormal LV voltages and nonvoltage ECG changes (Q waves, T-wave changes, and ST changes) were analyzed for association with HC. Fifty-three patients with HC (72% men) were age and gender matched to 104 control patients. Smoothing splines demonstrated that parabolic rather than linear relations existed between LV mass and SV1 , RV6 , and RV6 + SV1 in patients with HC and not the control cohort. LV hypertrophy by ECG voltage criteria was present in 34% of patients with HC and associated with poor sensitivity (29%). In patients with HC, 56% demonstrated nonvoltage ECG abnormalities and were associated with improved sensitivity (68%) and high specificity (94%). In conclusion, there is a parabolic relation between LV voltages and LV mass in adolescents with HC that may lead to “pseudonormalization.” Voltage abnormalities were associated with poor sensitivity, whereas nonvoltage criteria were associated with improved sensitivity with high specificity.
Abstract
Cardiac dysfunction can develop in large pediatric burns during the acute and recovery phase. When occurring in this population, the cardiac abnormality appears as left ventricular ...dysfunction or dilated cardiomyopathy. Recent studies have demonstrated perioperative and long-term cardiac dysfunction resulting in longer hospital stays for patients over 40% total body surface area. The objective of this study was to assess if early use of echocardiograms in large burns would allow for early recognition of patients at risk for cardiac dysfunction. Pediatric burn patients ages 0 to 18 years who sustained a burn injury of 30% TBSA or more or developed cardiac dysfunction during hospital course were evaluated. Echocardiograms were obtained upon admission with monthly repeats until three normal studies were attained or the patient was discharged and when symptomatic. Of the 130 acute burn patients admitted during 7/2017 to 10/2018, 10 patients met criteria for enrollment in this study. The average age was 5 years (0.8–10 years), 70% were males and 90% sustained flame injuries.Total TBSA average was 45% (24–70%) with average full-thickness burns of 33% (0–67%). Twenty echocardiogram studies were obtained. One patient with 25% TBSA burn, demonstrated severe left ventricular dysfunction with an ejection fraction (EF) of 25% from post-arrest myocardial stunning. Repeat echocardiogram studies demonstrated full recovery with normal EF. The remaining patients, despite large TBSA injuries, did not exhibit any abnormalities on echocardiogram examinations. No cardiac interventions were required. Use of echocardiograms is best performed on symptomatic burn patient populations.
Abstract
Introduction
Long term cardiac dysfunction and reversible cardiomyopathy can develop in large burns. In addition, cardiac arrhythmias can occur with any burn size. The incidence of cardiac ...arrhythmias and dysfunction in pediatric burns is unclear. Therefore, the objective of this prospective study was to evaluate the incidence of arrhythmias and cardiac dysfunction in pediatric burns and assess if early recognition improves clinical outcomes.
Methods
Pediatric burn patients ages 0 - 18 with TBSA injuries greater than 30% or those with cardiac dysfunction/ arrhythmia were enrolled after obtaining IRB approval. Exclusion criteria included pre-existing cardiac disease, desquamative skin disorders and electrical injury. For patients with burns greater than 30%, echocardiograms (Echo) were obtained on admission and then monthly. Echo exams were discontinued after three negative exams or upon discharge. All patients with arrhythmias had electrocardiograms (EKGs) and electrolyte panels obtained.
Results
Of the 130 acute burn patients admitted during 2017–2018, 10 patients were enrolled. The average age was 5 years (0.8 to 10 yrs), 70% were males and 90% sustained flame injuries. Average TBSA was 45% (24–70%) with average full thickness burns of 33% (0–67%). 20 Echos and 21 EKGs were obtained.
Only three patients experienced cardiac symptoms, reflecting an incidence rate of 2%. One patient had sustained bradycardia with EKG reading anterior wall myocardial infarction: however troponins, ECHO and clinical evaluation were not consistent with this reading. A severe medication side effect was found to be the inciting cause. Another patient demonstrated bradycardia with sleep, requiring no intervention. The final symptomatic patient, with a 25% TBSA burn, experienced four bradycardic arrests, caused by PEA arrest at the referring hospital after aspiration and receipt of multiple pain medications. Responding to epinephrine, the bradycardia resolved. Admission Echo demonstrated an ejection fraction of 25% with a normal EKG. Repeat ECHOs demonstrated full cardiac recovery.
The remaining patients did not exhibit any abnormalities on ECHO examinations aside from trivial tricuspid regurgitation. In addition, electrolytes panels did not demonstrate any findings contributing to cardiac arrhythmias.
Given these negative findings and the cost involved with ECHO examination, it was elected to discontinue the prospective study.
Conclusions
Although cardiac dysfunction and arrhythmias can impact patient care, the incidence of cardiac disease in pediatric burns is low. Elective Echos in large burns demonstrated no abnormal findings. Use of ECHOs is best performed with symptomatic disease.
Applicability of Research to Practice
Incidence of cardiac disease is low in pediatric burns. Use of echocardiograms are best used in patients with symptomatic disease.
Entrustable professional activities (EPAs) have become a popular framework for medical trainee assessment and a supplemental component for milestone and competency assessment. EPAs were developed to ...facilitate assessment of competencies and furthermore to facilitate translation into clinical practice. In this review, we explore the rationale for the introduction of EPAs, examine whether they fulfill the promise expected of them, and contemplate further developments in their application with specific reference to training in pediatric cardiology.
Mesalamine-containing products are often a first-line treatment for ulcerative colitis. Severe adverse reactions to these products, including cardiovascular toxicity, are rarely seen in pediatric ...patients. We present a case of a 16-year-old boy with ulcerative colitis treated with Asacol, a mesalamine-containing product, who developed sudden onset chest pain after four weeks on therapy. Serial electrocardiograms showed nonspecific ST segment changes, an echocardiogram showed mildly decreased left ventricular systolic function with mild to moderate left ventricular dilation and coronary ectasia, and his troponins were elevated. Following Asacol discontinuation, his chest pain resolved, troponins were trending towards normal, left ventricular systolic function normalized, and coronary ectasia improved within 24 hours suggesting an Asacol-associated severe drug reaction. Mesalamine-induced cardiovascular toxicity, although rare, may represent a life-threatening disorder. Therefore, every patient presenting with acute chest pain should receive a workup to rule out this rare drug-induced disorder.
The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE ...requiring more intensive therapy are associated with increased post-HTx mortality.
This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought.
Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors.
PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.
Hospitalised children have become more medically complex and increasingly require specialised teams and units properly equipped to care for them. Within paediatric cardiology, this trend, which is ...well demonstrated by the expansion of cardiology-specific ICUs, has more recently led to the development of acute care cardiology units to deliver team-based and condition-focused inpatient care. These care teams are now led by paediatric cardiologists with particular investment in the acute care cardiology environment. Herein, we describe the foundation and development of an Acute Care Cardiology Advanced Training Fellowship to meet the clinical, scholarly, and leadership training needs of this emerging care environment.
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