Management Of Pediatric Heart Failure Research Articles

Advances in understanding and managing pediatric heart failure and transplant.

This article highlights the most recent advances in a review of the current literature in the field of pediatric heart failure and transplantation. Diagnostically, the identification of new genetic factors has contributed to a deeper understanding of cardiomyopathy in children. Novel medications like sacubitril/valsartan and Sodium-Glucose cotransporter-2 (SGLT2) inhibitors, which are now standard in the adult population are being studied in pediatric population and offer new promise of pediatric heart failure treatment. Ventricular assist devices are more commonly used in cardiomyopathy patients and single ventricle patients as a bridge to transplant. Recent pediatric heart transplant society (PHTS) data demonstrated that waitlist survival improved significantly over the past decades (i) and new treatments such as daratumumab and eculizumab have been used in high-risk populations and demonstrate promising results. TEAMMATE trial is the first multicenter randomized clinical trial (RCT) in pediatric heart transplant (HT) to evaluate the safety and efficacy of everolimus (EVL) and low-dose tacrolimus (TAC) compared to standard-dose TAC and mycophenolate mofetil (MMF). It will provide valuable information about the safety and efficacy of EVL, TAC, and MMF (ii).Donor cell-free DNA has been used more in pediatric transplant recipients and has significantly decreased invasive EMB (iii). This past 5 years have witness dramatic progress in the field of pediatric heart failure and transplantation including more use of mechanical support in heart failure patients with various underlying etiology, especially use of mechanical support in single ventricle patients and the use of sacubitril/valsartan and SGLT2 inhibitors in the pediatric population. The problem of the highly sensitized transplant recipient remains, although novel therapeutics have been added to our toolbox of options to maintain healthy allograft function. Ongoing research aims to further enhance our understanding and management of pediatric heart failure, emphasizing the need for continued innovation in this complex field.

Acute Effects of Sacubitril/Valsartan with Initial Initiation in Pediatric Patients in the Cardiac Intensive Care Unit.

There are very few objectively studied and proven medical interventions for the management of pediatric heart failure. Due to improvement in morbidity and mortality in the adult heart failure population, sacubitril/valsartan has started to be used in pediatric patients. The aim of this study was to characterize the acute cardiovascular effects of sacubitril/valsartan in the first 48h after initiation. Single center retrospective study of pediatric patients in the cardiac intensive care unit who were initiated on sacubitril/valsartan for the first time over a three-year period. Clinical data was collected immediately prior to and within 48h following initiation. A total of 16 patients with a mean age of 9.6years were started on sacubitril/valsartan with a mean daily dose of 1.6mg/kg/day in the first 48h. Significant decreases were noted in N-terminal brain natriuretic peptide and vasoactive-inotrope score. No significant changes were noted in other clinical variables. The initiation of sacubitril/valsartan in a small cohort of pediatric patients with heart failure in the cardiac intensive care unit is associated with a significant decrease in N-terminal brain natriuretic peptide with a concurrent decrease in vasoactive-inotrope score and without significant change venous oxygen extraction ratio or other hemodynamic variables.

Diagnosis and Management of Pediatric Heart Failure.

Heart failure is a highly morbid syndrome, recognized as a major cause of adult mortality. Heart failure in pediatric patients, whether in the setting of congenital or acquired heart disease, is similarly associated with high mortality and resource utilization. Understanding the clinical presentation, diagnosis, and initial stabilization of pediatric heart failure is paramount for any acute care clinician as it may mimic common childhood ailments like viral respiratory or gastrointestinal illnesses. Pediatric heart failure occurs in patients with palliated or unpalliated congenital heart disease, familial or acquired cardiomyopathy, acquired valve disease, and myocarditis. This review will focus on heart failure in pediatric patients with structurally normal hearts and will summarize what is known about patterns of presentation, etiologies, diagnostic evaluation, and the acute and chronic management of this highly morbid syndrome.

Medical management of pediatric heart failure.

Pediatric heart failure is a complex, heterogenous syndrome that occurs relatively rarely in children, but carries a high burden of morbidity and mortality. This article reflects on the current state of medical therapy for both acute and chronic pediatric heart failure, based on expert consensus guidelines, and the extrapolation of data from trials performed in adults. For the management of acute heart failure specifically, we rely on an initial assessment of the perfusion and volume status of a patient, to guide medical therapy. This paradigm was adapted from adult studies that demonstrated increased morbidity and mortality in heart failure patients whose hemodynamics or examination findings were consistent with a PCWP >18 mmHg and a CI ≤2.2 L/min/m2. The cornerstone of treatment in the acute setting therefore relies on achieving a euvolemic state with adequate cardiac output. In the chronic setting, patients are typically maintained on a regimen of an angiotensin converting enzyme inhibitor, a beta-blocker, and spironolactone. For those with refractory heart failure, intravenous milrinone therapy has become a mainstay of bridging children to cardiac transplantation. The pediatric-specific data driving these clinical practices are limited and often times, conflicting. The future of pediatric heart failure depends on collaboration, quality improvement, and a commitment to pediatric-specific indications for new medical and device therapies.

Establishing Baseline Metrics of Heart Failure Medication Use in Children: A Collaborative Effort from the ACTION Network.

Heart failure metrics specific to the pediatric population are required to successfully implement quality improvement initiatives in children with heart failure. Medication use at the time of discharge following admission for decompensated heart failure has been identified as a potential quality metric in this population. This study aimed to report medication use at discharge in the current era for children admitted with acute decompensated heart failure. All patients < 21years of age with an index admission (1/1/2011-12/31/2019) for acute heart failure and a coexisting diagnosis of cardiomyopathy were identified from the Pediatric Health Information System. Medication use patterns were described and compared across age groups and centers. A total of 2288 patients were identified for inclusion. An angiotensin-converting enzyme inhibitor or an angiotensin receptor blocker (ACEi/ARB) was prescribed in 1479 (64.6%), beta blocker in 1132 (49.5%), and mineralocorticoid receptor antagonist (MRA) in 864 (37.8%) patients at discharge. The use of ACEi/ARB at discharge has decreased over time (64.6% vs. 69.6%, p = 0.001) and the use of beta blockers has increased (49.5% vs. 36.8%, p < 0.001) compared to a historical cohort (2001-2010). There is considerable variability in medication use across centers with an overall increase in beta blocker and decrease in ACEi/ARB use over time. Collaborative efforts are needed to standardize care and define quality metrics to identify best practices in the management of pediatric heart failure.

Approach to a Child with Congestive Heart Failure.

Pediatric heart failure (PHF) is an important cause of mortality and morbidity. Whereas ischemic heart disease is the most important cause of heart failure in adults, congenital heart diseases (CHD) and cardiomyopathies are important etiologies of PHF. Management of PHF also differs from that of adults. Here authors have reviewed the literature on PHF with respect to etiology, symptoms, investigations and treatment strategies.

Pediatric heart failure

The etiology of heart failure (HF) in children varies according to age, and congenital heart diseases are the most common cause of HF in pediatric population. HF must be differentiated from shock state. New-onset HF may be difficult to diagnose due to similarities in symptoms with respiratory and gastrointestinal diseases. Acute HF may be triggered by disturbance in preload, afterload, contractility, heart rate (HR), and diastolic functions, which contribute to cardiac output. The congestive state is treated using loop diuretics, and digitalis has little role in the management of pediatric HF.

Angiotensin Receptor and Neprylisin Inhibitor: A new drug in pediatric cardiologist's armamentarium.

Heart failure due to congenital heart disease and cardiomyopathies is a significant burden in the pediatric population. Pharmacological strategies for the management of pediatric heart failure are largely based on the extrapolation of adult data and Delphi process based on expert opinion. There are differences in the etiology, clinical course, and outcome of pediatric heart failure as compared to adult, thus the results of adult heart failure trials cannot be simply extrapolated to pediatric patients. There have been a lot of newer drugs for adults with heart failure, but there is a void for pediatric population with heart failure due to many reasons. Early results of multi-centric randomized control PANORAMA HF Trial and subsequent Food and Drug Administration approval for Angiotensin Receptor and Neprylisin Inhibitor (Sacubitril / Valsartan) for pediatric patients have tried to fill in this void and paved the way for a newer class of drugs for heart failure with proven benefits in pediatric patients.

The Management of Pediatric Heart Failure: Current State and Future Managements

The Management of Pediatric Heart Failure: Current State and Future Managements

Initial presentation and management of pediatric heart failure.

Heart failure is a rare but morbid diagnosis in the pediatric patient presenting to the emergency department (ED). Familiarity of the ED physician with the presentation, work-up, and management of pediatric heart failure is essential as accurate diagnosis is reliant on a high degree of suspicion. Studies evaluating pediatric heart failure are limited by its rarity and the heterogeneity of underlying conditions. However, recent reports have provided new data on the epidemiology, presentation, and outcomes of children with heart failure. The recent studies reviewed here highlight the significant diagnostic and management challenges that pediatric heart failure presents given the variety and lack of specificity of its presenting signs, symptoms, and diagnostic work-up. This review provides the ED physician with a framework for understanding of pediatric heart failure to allow for efficient diagnosis and management of these patients. The primary focus of this review is heart failure in structurally normal hearts.

Hemodynamic Monitoring in the Acute Management of Pediatric Heart Failure.

One of the basic tenets of cardiac critical care is to ensure adequate tissue oxygenation. As with other critical illness such as trauma and acute myocardial infarction studies have demonstrated that making the right diagnosis at the right time improves outcomes. The same is true for the management of patients at risk for or in a state of shock. In order to optimize outcomes an accurate and timely assessment of cardiac function, cardiac output and tissue oxygenation must be made. This review discusses the limitations of the standard assessment of cardiovascular function, and adjunctive monitoring modalities that may be used to enhance the accuracy and timely implementation of therapeutic strategies to improve tissue oxygenation.

Drug Treatment of Heart Failure in Children: Focus on Recent Recommendations from the ISHLT Guidelines for the Management of Pediatric Heart Failure.

The International Society of Heart and Lung Transplantation (ISHLT) recently updated consensus pediatric heart failure guidelines from those published in 2004 with an aim to provide a practical evidence-based resource whilst recognizing the influence of adult heart failure practice. The new guidelines were formed from published evidence for heart failure management and used parallels with adult literature where pediatric evidence was lacking. This is a summary of the pharmacological therapies discussed in the new 2014 guidelines, emphasizing changes from the previous recommendations with regards to treatment of chronic heart failure with reduced ejection fraction, chronic heart failure with preserved ejection fraction, and acute decompensated heart failure. Each recommendation is classified according to strength and level of evidence. We also discuss future perspectives in the pharmacological treatment of heart failure. The 2014 ISHLT guidelines have evolved considerably from those published in 2004 with extensive information surrounding the underlying pathophysiology, investigations and recommended treatment. The new guidelines contain a modest amount of new pediatric data on pharmacological therapies and extrapolate adult data when appropriate. It is likely that most new recommendations for pediatric heart failure will continue to be based on therapies of proven benefit in adult heart failure studies.

Pediatric Heart Failure in the Developing World.

The exact prevalence of heart failure among children of developing countries is not known, as the data is limited. The relative frequency of different causes of pediatric heart failure varies widely across different countries and even among different parts of large countries like India. Children of developing countries face a double burden of etiologies. Conditions such us congenital heart disease, myocarditis and cardiomyopathies are common causes of pediatric of heart failure. In addition, diseases like rheumatic heart disease, nutritional deficiencies, and other tropical diseases also result in heart failure among children of the developing countries. However, most of the developing countries have low resources and hence management of pediatric heart failure becomes challenging. Advanced therapies for heart failure are rarely used in children of developing countries and cardiac transplant remains a distant dream.

Mechanical assist devices in neonates and infants.

Clinical advances in the field of mechanical support have led to additional treatment strategies in adult and, subsequently, pediatric heart failure management. The limited number of device options available to the pediatric population has led to a reallocation of resources in order to broaden the pediatric device armamentarium. Although there are very few device options in pediatrics, there are short-term support options that include the RotaFlow and PediMag. The longer-term support option for small children is limited to only the Berlin heart EXCOR device, which had been approved by the FDA since December 2011. There is a dire need for further device options for the pediatric patient and many devices funded by the PumpKIN program are under development to further the care of this population.

Abstract 18356: Autologous Intracoronary Stem Cell Therapy is Safe and Feasible for Children With Dilated Cardiomyopathy: A Randomised Controlled Cross-Over Pilot Study

Meta-analysis of adult stem cell therapy studies post myocardial infarction has shown moderate &amp; sustained improvement of function. Positive results have also been seen in adult dilated cardiomyopathy (DCM) &amp; chronic ischaemic heart failure. The place of stem cells in pediatric heart failure is unknown. This randomised, blind, crossover placebo controlled trial sought to evaluate the potential use of stem cells in the management of pediatric heart failure &amp; is the first of its kind within the pediatric population. Methods &amp; results: Ten children (mean age 7.2y, range 2.2-14.1y, 6 male) with DCM were recruited from Heart Failure clinic. Patient profiles on study entry: NYHA/Ross class 3-4, ejection fraction (EF) on MRI mean 40% (range 30-48%). On enrolment patients were randomised to receive stem cell or placebo intracoronary injection &amp; MRI, with cross-over of intervention at 6 months &amp; final MRI assessment at 1 year. Bone marrow processing was performed using standard techniques. The mononuclear cells, obtained within 1.5 hours of aspiration, were divided to provide 1ml for coronary injection &amp; 1ml for phenotyping. Intracoronary injections &amp; MRI were performed under the same anaesthetic. Mononuclear cell count mean was 51.7 x 106/ml (range 11.8-115.1 x 106/ml). Nine patients completed the protocol, the tenth patient had Duchenne Muscular Dystrophy (DMD) &amp; did not undergo general anaesthesia, this data was excluded from the group analysis and is presented separately. There were no withdrawals, morbidity or mortality. Primary end point, freedom from death or transplant, was achieved in all patients. Secondary end points, indexed end diastolic volume (EDV) 6 months post cell infusion for n=9, was reduced in 7 &amp; unchanged in 2 (P=0.01 for comparison of means), end systolic volume (ESV) decreased in 6 following cell injection. (P=0.05 for comparison of means). EDV increased in 5 patients following placebo. EF did not change significantly. EDV reduced in all patients with an injected cell count &gt;34.2 x 106. Conclusion: This pilot study demonstrates that intracoronary injection of autologous stem cells is safe &amp; feasible in children. Encouraging data on EDV were generated, larger scale studies will be required to measure clinical effect.

Current Pharmacologic Management of Pediatric Heart Failure in Congenital Heart Disease

Pharmacologic therapy represents the mainstay of treatment for heart failure in children. However, medical therapy for this population is not widely standardized. This is mainly due to the heterogeneity of potential etiologies, the specific challenge of patients with univentricular physiology and the lack of evidence-based prospective randomized clinical trials in pediatric patients. In fact, most current strategies are based largely on extrapolated data from adult studies. Although the classic drugs for heart failure, i.e. diuretics, angiotensin-converting enzyme inhibitors, ß -blockers and cardiac glycosides, still play a major role in the treatment of pediatric heart failure, newer alternative therapies such as levosimendan and nesiritide are increasingly utilized with promising early results. A systematic literature search of PubMed and MEDLINE databases using relevant terms was performed. All clinical trials and relevant manuscripts about the current pharmacologic treatment of heart failure in the pediatric population were reviewed. New drugs such as levosimendan and nesiritide and the treatment of single-ventricle patients were also included.

Is there a role for carvedilol in the management of pediatric heart failure? A meta analysis and e-mail survey of expert opinion

Is there a role for carvedilol in the management of pediatric heart failure? A meta analysis and e-mail survey of expert opinion

Contemporary management of pediatric heart failure

The spectrum of causes of pediatric heart failure is broad and differs significantly from that seen in most adult patients. Left-to-right shunts and outflow obstruction lesions are responsible for a large number of pediatric cases of heart failure. Most of these are now treated successfully with surgery or catheter intervention. Medical therapy is the mainstay of care for myocardial disorders with diuretics, angiotensin-converting enzyme inhibitors, β-blockade and cardiac glycosides. There are few prospective trials of these agents in a pediatric population, but extrapolated data support their use in children. In addition to medical therapy, interventions such as automatic implantable cardioverter defibrillators and resynchronization therapy have become increasingly common in pediatric heart disease, as well as in adult patients with congenital heart disease.

Does nesiritide offer something new in the management of pediatric heart failure?*

Does nesiritide offer something new in the management of pediatric heart failure?*

Cardiomyocyte injury to transplant: pediatric management.

Cardiomyocyte injury in pediatric patients has a vast number of causes, which are often distinct from the causes of adult heart failure. However, the management of pediatric heart failure and heart transplantation has generally been inferred from adult studies. New therapies show great promise for the neurohormonal regulation of heart failure and the ability to control immunosuppression after heart transplantation. Large, randomized, multicenter, controlled clinical trials are needed to determine the efficacy of these therapies in this population. This article reviews the current recommendations and evidence-based medicine, where available, for the medical management of myopathic dysfunction and transplantation in pediatric patients.