Endomyocardial Biopsy in Pediatric Myocarditis and Dilated Cardiomyopathy: A Tool in Search for a Role

Endomyocardial biopsy (EMB) is a well-known diagnostic tool for the investigation and treatment of myocardial diseases and remains the gold standard for the diagnosis of myocarditis. Due to its invasiveness, with a complication rate ranging from 1 to 15%, its role in the diagnostic work-up of pediatric heart failure is not well established. The aim of this review is to define the role of EMB as diagnostic technique in the work up of children presenting with severe left ventricular dysfunction with the support of our center experience.

Recent and Upcoming Drug Therapies for Pediatric Heart Failure

Pediatric heart failure (HF) is an important clinical condition with high morbidity, mortality, and costs. Due to the heterogeneity in clinical presentation and etiologies, the development of therapeutic strategies is more challenging in children than adults. Most guidelines recommending drug therapy for pediatric HF are extrapolated from studies in adults. Unfortunately, even using all available treatment, progression to cardiac transplantation is common. The development of prospective clinical trials in the pediatric population has significant obstacles, including small sample sizes, slow recruitment rates, challenging endpoints, and high costs. However, progress is being made as evidenced by the recent introduction of ivabradine and of sacubitril/valsartan. In the last 5 years, new drugs have also been developed for HF with reduced ejection fraction (HFrEF) in adults. The use of well-designed prospective clinical trials will be fundamental in the evaluation of safety and efficacy of these new drugs on the pediatric population. The aim of this article is to review the clinical presentation and management of acute and chronic pediatric heart failure, focusing on systolic dysfunction in patients with biventricular circulation and a systemic left ventricle. We discuss the drugs recently approved for children and those emerging, or in use for adults with HFrEF.

Serum Copeptin Level as a Predictor Marker of Pediatric Heart Failure Outcomes

Background: This study aimed to assess the role of copeptin as a diagnostic marker of heart failure (HF) and outcomes. Method: We randomly recruited 76 cardiac patients aged 1 month to 15 years and 65 control healthy children matched in age and sex. Based on plasma copeptin level, the study population were sub-grouped into quartiles (Q). Results: The mean age of cases and control was 40.52 ±34.35 months and 42.43 ±30.42 months respectively. Median copeptin level was higher among patients 16.80 (16.4) compared to control 8.00 (3.0), P<0.01. Copeptin level was not statistically significantly different in-between patients with different etiologies of HF, P =0.515. Total leukocytic count, platelets, serum sodium, inotropic score, and troponin were significantly correlated with copeptin quartile. Three-fourth of dead children were within the Q4, and 12.5 % were within the first one, P=0.214. Around 76.5% of patients who had multiorgan dysfunction were within the Q4 while 5.9% belonged to Q1, P=0.022. Of those who developed sepsis, 82.6% and 4.3% were located within Q4 and Q1, P<0.01. All patients who required mechanical ventilation were within Q4, P= 0.005. Conclusion: Plasma level of copeptin is elevated in pediatric HF regardless its etiology and can be used as a predictor of poor outcomes.

Current and Future Drug and Device Therapies for Pediatric Heart Failure Patients: Potential Lessons from Adult Trials

This review discusses the potential drug and device therapies for pediatric heart failure (HF) due to reduced systolic function. It is important to realize that most drugs that are used in pediatric HF are extrapolated from adult cardiology practices or consensus guidelines based on expert opinion rather than on evidence from controlled clinical trials. It is difficult to conclude whether the drugs that are well established in adult HF trials are also beneficial for children because of tremendous heterogeneity in the mechanism of HF in children and variations in the pharmacokinetics and pharmacodynamics of drugs from birth to adolescence. The lessons learned from adult trials can guide pediatric cardiologists to design clinical trials of the newer drugs that are in the pipeline to study their efficacy and safety in children with HF. This paper’s focus is that the reader should specifically think through the pathophysiological mechanism of HF and the mode of action of drugs for the selection of appropriate pharmacotherapy. We review the drug and device trials in adults with HF to highlight the knowledge gap that exists in the pediatric HF population.

Pediatric heart failure

Pediatric heart failure is a significant challenge in clinical practice, a global burden worldwide. Compared to adult population, heart failure in children has a variety of etiologies depending on the patients age. However, pediatric heart failure guidelines are still extrapolated from adult population. In infants and children, the pharmacological treatment is based on angiotensin-converting enzyme inhibitors, beta-blockers, aldosterone receptors antagonists, and other diuretics in the presence of congestion, with a lower use of device therapy. In end-stage heart failure, as a bridge to transplantation or recovery, ventricular assist devices or different palliative surgical interventions are used nowadays. This review focuses on the most important causes, pathophysiological mechanisms, clinical manifestation and management of pediatric heart faliure based on recent guidelines. Furthermore, it emphasize the newer pharmacological or non-pharmacological therapies that have been recently approved in pediatric population.