OBJECTIVE: This article highlights the most recent advances in a review of the current literature in the field of pediatric heart failure and transplantation.
RESULTS: 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).
CONCLUSIONS: 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.

Early identification of kidney dysfunction in patients with advanced heart failure is crucial for timely interventions. In addition to elevations in serum creatinine, kidney dysfunction encompasses inadequate maintenance of sodium and volume homeostasis, retention of uremic solutes, and disrupted endocrine functions. Hemodynamic derangements and maladaptive neurohormonal upregulations contribute to fluctuations in kidney indices and electrolytes that may recover with guideline-directed medical therapy. Quantifying the extent of underlying irreversible intrinsic kidney disease is crucial in predicting whether optimization of congestion and guideline-directed medical therapy can stabilize kidney function. This scientific statement focuses on clinical management of patients experiencing kidney dysfunction through the trajectory of advanced heart failure, with specific focus on (1) the conceptual framework for appropriate evaluation of kidney dysfunction within the context of clinical trajectories in advanced heart failure, including in the consideration of advanced heart failure therapies; (2) preoperative, perioperative, and postoperative approaches to evaluation and management of kidney disease for advanced surgical therapies (durable left ventricular assist device/heart transplantation) and kidney replacement therapies; and (3) the key concepts in palliative care and decision-making processes unique to individuals with concomitant advanced heart failure and kidney disease.

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To investigate the biomechanical effects of direct ventricular assistance and explore the optimal loading mode, this study established a left ventricular model of heart failure patients based on the finite element method. It proposed a loading mode that maintains peak pressure compression, and compared it with the traditional sinusoidal loading mode from both hemodynamic and biomechanical perspectives. The results showed that both modes significantly improved hemodynamic parameters, with ejection fraction increased from a baseline of 29.33% to 37.32% and 37.77%, respectively, while peak pressure, stroke volume, and stroke work parameters also increased. Additionally, both modes showed improvements in stress concentration and excessive fiber strain. Moreover, considering the phase error of the assist device\'s working cycle, the proposed assist mode in this study was less affected. Therefore, this research may provide theoretical support for the design and optimization of direct ventricular assist devices.
为了研究直接心室辅助的生物力学影响以及探究最优的加载模式，本文基于有限元方法建立了心衰患者的左心室模型，并提出了一种维持压迫力峰值的加载模式，从血流动力学和生物力学两个方面与传统的正弦加载模式进行了对比。结果表明，两种模式都能显著提升血流动力学参数，射血分数分别从基线29.33%增加到37.32%与37.77%，峰值压力、每搏量和每搏功等参数都有所增加；且两种模式的应力集中、过度纤维应变等现象均有所改善。然而，当考虑到辅助装置工作周期的相位误差时，本文所提出的辅助模式受到的影响更小，故本文研究或可为直接心室辅助装置的设计和优化提供理论支持。.

Heart failure (HF) is a progressive disease. It is estimated that more than 250,000 patients suffer from advanced HF with reduced ejection fraction refractory to medical therapy. With limited donor pool for heart transplant, continue flow left ventricle assist device (LVAD) is a lifesaving treatment option for patients with advanced HF. This review will provide an update on indications, contraindications, and associated adverse events for LVAD support with a summary of the current outcomes data.

Cardiogenic shock is a multisystem pathology that carries a high mortality rate, and initial pharmacotherapies include the use of vasopressors and inotropes. These agents can increase myocardial oxygen consumption and decrease tissue perfusion that can oftentimes result in a state of refractory cardiogenic shock for which temporary mechanical circulatory support can be considered. Numerous support devices are available, each with its own hemodynamic blueprint. Defining a patient\'s hemodynamic profile and understanding the phenotype of cardiogenic shock is important in device selection. Careful patient selection incorporating a multidisciplinary team approach should be utilized.

Patients on left ventricular assist devices (LVAD) are prone to excessive hemostasis disturbances due to permanent contact of artificial pump surfaces with blood components. We aimed to investigate if fibrin clot permeability is altered in patients on long-term continuous-flow LVAD therapy and if the clot permeability is associated with clinical characteristics and adverse events. We investigated 85 end-stage heart failure patients (90.6% men, age 48.6-63.8 years) scheduled for continuous flow long-term LVAD support according to current clinical indications. The patients were assessed periodically: prior to LVAD implantation (T1), 3-6 months (T2) after LVAD implantation, 6-12 months after (T3) and then every 6 months. We tested the first three blood samples (T1-T3) and the last available blood sample (T4), but no longer than 5 years after LVAD implantation. We assessed hemostasis parameters (Activated Partial Thromboplastin Time (APTT) Prothrombin Time, Activated Partial Thromboplastin Time, Fibrinogen, D-dimer, Antithrombin, Thrombin Time, Factor VIII, and von Willebrand Factor, aspirin-induced platelet inhibition, adenosine-diphosphate test) changes during the study period. Fibrin Clot Permeability was evaluated using a pressure system and Permeability Coefficient (Ks) was calculated. We observed a decrease in fibrin clot permeability (Ks) between T1, T2, T3 and T4 time periods; P < 0.01 for each comparison. Fibrin clot permeability was negatively correlated with fibrinogen concentration: r = - 0.51, P < 0.001, factor VIII activity r = - 0.42, P < 0.001. There was no association of Ks with age, Left Ventricular Ejection Fraction (LVEF) and medications P > 0.001, however cumulative measurements in patients on aspirin showed shortening of Ks in this group P = 0.0123. Major adverse cardiac and cerebrovascular events (MACCE) occurred in 36.5% patients, bleeding events in 25.9%, Net Adverse Clinical Events (NACE) in 62.4%; 31.7% patients died, and 17.6% underwent transplantation. The transplantation was considered as the endpoint. Discrepancies in Ks were observed between patients with MACCE, bleeding, and NACE, and patients without adverse events. Ks showed a constant trend towards normalization (P < 0.01) only in patients without adverse events. Patients with advanced heart failure have disturbed clot structure. A trend towards normalization of the Ks values is associated with fewer thromboembolic and bleeding complications in this group of patients.