2026 AHA科学声明：儿童急性失代偿性心力衰竭的评估和管理

Causes

Evaluation of the child with ADHF requires identifying the cause of the disease state, identifying risk factors and tailoring management of ADHF to the individual patient. Among pediatric patients, HF can be due to congenital heart disease or cardiomyopathies.

Pediatric cardiomyopathies are diseases of the heart muscle affecting ≈1 in 100 000 children.$^{1}$ Dilated cardiomyopathy is the most common subtype (50%), followed by hypertrophic cardiomyopathy (35%-50%) and restrictive cardiomyopathy (<5%).$^{2}$ The diversity of causes, underlying pathophysiology, and different phenotypic expressions influence disease presentation and response to treatment. Approximately 40% of those who present with symptomatic dilated cardiomyopathy undergo a heart transplantation or die within the first 2 years of diagnosis.$^{3}$ Unlike adult patients with structurally normal hearts who develop HF, pediatric HF cases are more diverse and include patients with congenital heart disease who are at greatly increased risk of developing HF.$^{4}$ The prevalence of HF in congenital heart disease is increasing and is estimated to be between 6.2% and 39% from 2 single-site studies.$^{5,6}$ Congenital heart disease is the leading indication for pediatric HF-related emergency department visits and hospitalizations.$^{7}$ Moreover, pediatric HF readmissions for those with congenital heart disease have not decreased in a decade.$^{8}$ Patients with congenital heart disease often face chronic volume overload (from lesions such as ventricular septal defects, atrioventricular septal defects, and patent ductus arteriosus) or pressure overload (from lesions such as aortic stenosis, pulmonary stenosis, or coarctation). When left unrepaired, these lesions often present with signs and symptoms of acute HF. Obstructive lesions such as critical ductal-dependent lesions can present with acute HF symptoms in infancy and warrant prompt attention and management to prevent critical cardiogenic shock (CS) and death.$^{9}$

Patients with single-ventricle congenital heart disease require special consideration because they often have a combination of defects that can lead to HF, including multiple cardiotomies, neurohormonal overactivity, chronic cyanosis, residual obstructive or regurgitant lesions, and coronary insufficiency.$^{4,10}$ Patients with single-ventricle congenital heart disease can develop ADHF at any palliative stage but appear to be at highest risk after the Norwood operation.$^{11}$ Although it is more common in children with single-ventricle congenital heart disease to have chronic HF secondary to intrinsic myo-

cardial dysfunction, atrioventricular valve regurgitation and persistent residual anatomic lesions can also play a role. If these lesions remain unaddressed, they can lead to ADHF during periods of increased metabolic demand such as those precipitated by infection or sepsis or by progressive worsening of the hemodynamic milieu.

ADHF can be the mode of presentation from acute rejection after cardiac transplantation. Among pediatric patients with heart transplantation experiencing acute rejection, ≈44% have associated hemodynamic compro- mise requiring inotropic support or mechanical circula- tory support (MCS).$^{12}$ Therapies to treat their underlying rejection (antibody or cellular) can potentially reverse their cardiac dysfunction,$^{13,14}$ although it can significantly lower graft survival in the long term.$^{12}$

# Diagnostic Challenges

There are numerous challenges when evaluating ADHF in children. There is no standardized nomenclature on anatomic and functional staging of pediatric HF.$^{15}$ Most recently, an American Heart Association scientific statement addressed standardized evaluation and management of chronic HF in pediatric and adolescent patients with congenital heart disease.$^{4}$ Children with congenital heart disease and worsening HF present with heterogeneous signs and symptoms, including but not limited to growth failure, pubertal failure, food intolerance, nausea, vomiting, respiratory distress, decreasing exercise tolerance, worsening i cyanosis, and recurrent arrhythmia.$^{4,15}$ In the context of ADHF in children, early diagnosis and treatment of HF is key to preventing CS. CS is defined as a low cardiac output state resulting in tissue hypoxia and life-threatening end-organ hypoperfusion.$^{16}$ Adult patients with ADHF and CS are now routinely staged with the validated Society for Cardiovascular Angiography and Interventions shock staging system.$^{17}$ A recent pediatric study found that ≈1 in 4 pediatric patients with ADHF experiences CS.$^{18}$ Using the Society for Cardiovascular Angiography and Interventions stages (Figure 1), those investigators found a graded increase in mortality with each increasing Society for Cardiovascular Angiography and Intervention stage, from 7% in stage A to 36% in stage E. Unlike in adults, assessment of jugular venous pulse, serial measurement of lactate, continuous monitoring of blood pressure, invasive hemodynamic data, and urine output were not routinely performed. This limits our ability to estimate the true prevalence of CS in the overall pediatric ADHF cohort.$^{18}$

# INITIAL MANAGEMENT IN THE EMERGENCY DEPARTMENT

Pediatric patients with ADHF present with unique challenges in the emergency department (ED), requiring a

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|CS Stage|Definition| |---|---| |Stage A "At risk"|No hypotension, no vasoactive agents, no other criteria for CS| |Stage B "Beginning"|Hypotension OR vasoactive agents without other criteria for CS| |Stage C "Classic"|Hypotension AND vasoactive agents OR other criteria for CS| |Stage D "Deteriorating"|MCS or > 2 vasoactive agents| |Stage E "Extremis"|Circulatory collapse with CPR|

Figure 1. Modified Society for Cardiovascular Angiography and Interventions staging for CS.$^{18}$ CPR indicates cardiopulmonary resuscitation; CS, cardiogenic shock; and MCS, mechanical circulatory support. Adapted with permission from Naidu et al.$^{17}$ Copyright © 2021 The Authors. Published by Elsevier Inc on behalf of the Society for Cardiovascular Angiography and Interventions Foundation. User License: Creative Commons Attribution-NonCommercial-NoDerivs (CC BY-NC-ND 4.0).

systematic and prompt workup to ensure timely diagnosis and treatment. The initial evaluation should include a thorough history and review of systems, focusing on symptoms such as dyspnea, feeding difficulties, vomiting, abdominal discomfort, fatigue, and exercise intolerance, which are common features of HF in children. Vital signs should be monitored closely, with special attention paid to blood pressure and heart rate because tachycardia and hypotension may signal impending hemodynamic collapse and cardiac arrest. ADHF can mimic sepsis, a common presentation in pediatric emergency care. Frontline professionals should carefully evaluate before initiating sepsis protocols or administering intravenous fluids because some children with hypotension may be experiencing ADHF.

A key component of the assessment is the determination of whether the patient is presenting with principal features of low cardiac output or those of congestion, both of which have distinct management implications. Clinical signs such as altered mental status, reduced urine output, poor pulses, and cool extremities suggest low cardiac output. End-organ hypoperfusion and tissue hypoxia are classic features of CS, a more severe form of ADHF. Elevated lactate levels and systemic hypotension are common manifestations of CS, although hypotension may be a late finding in children. In contrast, jugular vein distention, pulmonary crackles, hepatomegaly, gallop rhythm, and peripheral edema may indicate fluid overload and congestion.$^{19}$ Laboratory tests, including complete blood count, comprehensive metabolic panel, lactate, and NT-proBNP (N-terminal pro-B-type natriuretic peptide), provide important insights into cardiac and end-organ function.$^{20}$ Both early echocardiography and point-of-care cardiac ultrasound are essential for evaluating cardiac structure and function, helping to identify conditions such as cardiomyopathy or congenital heart disease. In addition, chest radiography

can help identify cardiomegaly or pulmonary congestion, and an ECG can determine any arrythmias. Early identification and management of ADHF in pediatric patients can significantly improve outcomes, emphasizing the importance of a comprehensive and efficient approach in the ED setting.

Intravenous loop diuretics are the cornerstone of decongestive treatment in the 'Emergency setting. Diuretics decrease preload and provide relief of con-gestive symptoms in patients with volume overload. In the emergency setting, intravenous diuretics are pre-ferred because of the greater bioavailability and onset of action compared with oral diuretics. The American Heart Association/American College of Cardiology/ Heart Failure Society of America guidelines recommend that patients with evidence of fluid overload be treated promptly with intravenous loop diuretics.$^{21}$ The main goal of diuretic therapy is to restore an euvolemic state and resolve signs and symptoms of congestion. If the diuretic response is inadequate, higher doses of the loop diuretic or the addition of a second diuretic may be necessary. In patients with chronic diuretic use, DOSE trial (Diuretic Optimization Strategies Evaluation) results suggest that an optimal starting point for intravenous diuretic dosing is 1 to 2.5 times the patient's total oral dose divided in half and administered every 12 hours.$^{22}$ Most patients who receive intravenous diuretics will require oral diuret-ics at hospital discharge.$^{21}$

promptly with intravenous loop diuretics.$^{21}$ The main goal of diuretic therapy is to restore an euvolemic state and resolve signs and symptoms of congestion. If the diuretic response is inadequate, higher doses of the loop diuretic or the addition of a second diuretic may be necessary. In patients with chronic diuretic use, DOSE trial (Diuretic Optimization Strategies Evaluation) results suggest that an optimal starting point for intravenous diuretic dosing is 1 to 2.5 times the patient's total oral dose divided in half and administered every 12 hours.$^{22}$ Most patients who receive intravenous diuretics will require oral diuretics at hospital discharge.$^{21}$

Assessing oxygen delivery is critical in ADHF to detect tissue hypoxia, guide treatment decision-making, and predict prognosis. Oxygen delivery depends on cardiac output and blood oxygen content, which is influenced by hemoglobin concentration, oxygen saturation, and Pao$_{2}$$. Direct monitoring methods include arterial blood gas analysis and lactate levels. Noninvasive tools such as pulse oximetry and near-infrared spectroscopy can help estimate hypoxemia, although oxygen saturation alone

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does not reflect oxygen delivery and may be affected by poor peripheral perfusion.

Patients presenting with respiratory distress due to pulmonary edema may benefit from noninvasive respiratory support in the form of high-flow oxygen delivery and continuous positive airway pressure or bilevel positive airway pressure.$^{23}$ Positive airway pressure can improve oxygenation by recruiting collapsed alveoli and reducing respiratory muscle effort, offering symptomatic relief. Early use of continuous positive airway pressure or bilevel positive airway pressure may reduce the need for intubation and mechanical ventilation. Hemodynamically, it reduces right ventricular preload and left ventricular (LV) afterload, enhancing cardiac performance by increasing stroke volume and unloading the myocardium. Caution should be exercised when providing positive pressure in patients with isolated right HF because this may lead to an acute rise in right ventricular afterload and precipitate decompensation.

The American Heart Association/American College of Cardiology/Heart Failure Society of America guidelines for adult HF management recommend that inotropes be used to maintain systemic perfusion and preserve end-organ performance in patients with CS. Among patients with advanced HF despite optimized oral therapies, continuous inotropic support is reasonable as a bridging therapy to MCS or heart transplantation; however, routine use of inotropes in ADHF may pose challenges. Inotropes can be proarrhythmic, increase myocardial oxygen consumption, and lead to increased mortality in adults. There is a lack of evidence demonstrating the superior-ity of one inotroper over another, but in pediatric ADHF, it is considered standard of care to use an intravenous continuous infusion of epinephrine for resuscitation and rescue of the circulation. Epinephrine increases cardiac output, heart rate, and systemic vascular resistance but also leads to increased risk of tachyarrhythmias and may increase myocardial oxygen demand. For chronic bridging to transplantation or MCS, a continuous infusion of milirnone is commonly administered in children.$^{24}$ It is generally recommended that the lowest effective dose be used to minimize the risk of adverse effects and that the need for ongoing inotropic support be regularly re-evaluated (see the Isotropes section in Intensive Care Management).

INTENSIVE CARE MANAGEMENT

Cardiogenic Shock

CS is highly prevalent (up to 26%) and portends high mortality and morbidity.$^{18}$ In the largest single-center observational study applying a modified version of the Society for Cardiovascular Angiography and Interventions CS guidelines and definitions in patients <21 years of age presenting with CS, Puri et al$^{18}$ showed

that these patients present with worse systolic function, higher BNP (B-type natriuretic peptide) concentration, and higher frequency of hepatic and renal injury. Nearly 90% used mechanical ventilation, and ≈50% required MCS. The undulant nature of children's CS stages could provide false reassurance as to the course being predictable. Timely treatment and stabilization are key.

The optimal vasoactive therapy in children presenting with ADHF is unknown. In adults, timing of inotropes is important. Peacock et al$^{25}$ evaluated >35 000 hospital admission episodes in adults presenting with ADHF from ADHERE (Acute Compensated Heart Failure National Registry) and showed that administration of vasodilators or inotropes <6 hours after admission was associated with better survival and shorter hospital length of stay. Because inotropes initiation is more common in the ICU, this could delay early administration if the transition from the ED to the ICU is delayed. In children evaluated for ADHF in the ED, Macicek et al$^{26}$ showed that the median time to initiation of vasoactive agents was significantly shorter for patients whose infusions were started in the ED compared with in the ICU. This offers an opportunity for safe administration and prevents delays while waiting for ICU transfer.


Inotropes

Inotropes enhance cardiac contractility, thereby increasing cardiac output to support mean arterial pressure and maintain adequate systemic perfusion. The preferred route of administration for inotropes is through a central line to mitigate the risk of extravasation. In neonates, administration through the umbilical arterial catheter is discouraged. The use of inotropes in patients without hypotension, impaired perfusion, low cardiac output, and reduced end-organ perfusion may be harmful.$^{27-29}$ Selection of an appropriate inotrope is based primarily on the patient's clinical presentation, perfusion, and blood pressure parameters.

Milrinone, a phosphodiesterase inhibitor, increases myocardial contractility by preventing breakdown of cAMP. This results in potent arterial and venous dilation, leading to increased inotropy, improved lusitropy, and decreased systemic and peripheral vascular resistance. The typical starting dose is 0.25 µg·kg⁻$^{1}$·min⁻$^{1}$ with titration up to a dose of 0.75 µg·kg⁻$^{1}$·min⁻$^{1}$.$^{27,30}$ The relatively long half-life of milrinone (1–4 hours) distinguishes it from other inotropes. In patients with acute kidney injury, reduced doses and slow titration are often required to prevent drug accumulation attributable to impaired renal clearance. Furthermore, milrinone retains its efficiency when coadministered with β-blockers because the mechanism of action is independent of β-adrenergic receptors, offering a potential advantage over agents such as dobutamine and dopamine.

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Dobutamine, a synthetic catecholamine with dosing ranging from 2 to 20 µg·kg$^{-1}$·min$^{-1}$, primarily stimulates β-1 adrenergic receptors in the myocardium, increasing both inotropy and chronotropy. At doses <5 µg·kg$^{-1}$·min$^{-1}$, it activates peripheral β-2 receptors, causing vasodilation, whereas at higher doses it induces vasoconstriction. Dobutamine is often administered for short durations because of its rapid onset of action and short half-life of 2 to 3 minutes.$^{31,32}$ Despite its robust β-1 activity compared with epinephrine and dopamine, obutamine is generally not preferred because of an increased risk of dysrhythmias and potential tachyphylaxis.

Epinephrine acts on β-1 receptors to increase inotropy and on β-2 receptors at doses <0.05-0.1 µg·kg⁻$^{1}$·min⁻$^{1}$ to reduce systemic vascular resistance. At doses >0.1 µg·kg⁻$^{1}$·min⁻$^{1}$, epinephrine stimulates peripheral α-adrenergic receptors, increasing systemic vascular resistance. Epinephrine has stronger α-1 receptor activity than dopamine and dobutamine and is considered a more potent inotrope than dopamine.$^{28}$

Dopamine at doses <3 µg·kg⁻$^{1}$·min⁻$^{1}$ has been described as enhancing renal perfusion due to activation of D₁ receptors.33 In the ROSE AHF trial (Renal Optimization Strategies Evaluation in Acute Heart Failure), 360 adults randomized to low-dose dopamine, low-dose neisritide, or placebo were assessed for decongestion enhancement and renal function preservation.34 This showed that neither low-dose dopamine (2 µg·kg⁻$^{1}$·min⁻$^{1}$) nor low-dose neisritide (0.005 µg·kg⁻$^{1}$·min⁻$^{1}$ without bolus) enhanced decongestion or improved renal function when added to diuretic therapy. Randomized studies in children assessing low-dose dopamine effectiveness are lacking. At doses between 3 and 10 µg·kg⁻$^{1}$·min⁻$^{1}$, dopamine primarily stimu-

lates β-1 receptors in the myocardium, increasing both inotropy and chronotropy. Doses exceeding 10 µg·kg⁻$^{1}$·min⁻$^{1}$ activate peripheral α receptors, resulting in increased systemic vascular resistance.

Hemodynamic Profiles and Monitoring

Clinical assessment of congestion and perfusion has been used for >2 decades by adult cardiologists to assess patients with ADHF (Figure 2).$^{35}$ The effect of these hemodynamic profiles on morbidity and mortality among pediatric patients with ADHF has not been prospectively evaluated. Given its limited utility in pediatric patients, it is difficult to draw any conclusions about profiles in children with ADHF, but it is likely that the hemodynamic profiles of older teenage children are more like profiles of adults presenting with ADHF. Despite these challenges, once the diagnosis has been established, monitoring of hemodynamic parameters can guide treatment. An expert-based consensus by the Cardiovascular Dynamics Section of the European Society of Paediatric and Neonatal Intensive Care provided weak agreement on the use of thermodilution catheters in children with refractory CS and recommended against the use of pulmonary coronary arterial catheters to measure cardiac output in children.$^{36}$ There was strong agreement on the use of standard invasive measurements, including blood pressure, central venous pressure, Syo, saturation, serial lactate, and clinical examination of perfusion and com- comitant near-infrared spectroscopy tissue saturations in children with hemodynamic instability. Use of pulmonary artery pressure monitoring is limited in pediatric HF, with no benefit demonstrated in this cohort to date.$^{37}$ Among patients with congenital heart disease, a small series of


Figure 2. Clinical assessment of congestion and perfusion by clinician to determine the hemodynamic profile of a patient presenting with acute decompensated heart failure.

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adult Fontan patients with pulmonary artery pressure monitoring demonstrated no benefit in reducing HF hospitalizations.$^{38}$

Respiratory Management

Pulmonary congestion from high LV end-diastolic pressure will increase respiratory drive in response to hypoxia, hypercapnia, and respiratory and metabolic acidosis, redistributing cardiac output toward respiratory muscles, worsening an already increased oxygen consumption and low oxygen delivery state. In the randomized comparison of standard oxygen therapy, continuous positive airway pressure (continuous positive airway pressure 5–15 cm water), and noninvasive positive with inspiratory pressure (8 to 20 cm water, expiratory pressure 4 to 10 cm water) that enrolled patients as young as 16 years of age with cardiogenic pulmonary edema, noninvasive positive with inspiratory pressure induced a more rapid improvement in blood gas analysis and respiratory distress but failed to have an effect on short-term mortality (7 days)$^{39}$; however, this study had limitations given the low mortality and intubation rates. The low intubation rate is particularly important because elective or emergent endotracheal intubation carries a high risk of cardiac arrest due to a decrease in coronary perfusion attributed to sedation effects in blood pressure, vagal tone changes, and decreased cardiac output from decreasing heart rate. When administering sedation, practitioners need to consider preservation of hemodynamics and potential augmentation of cardiac output by titrating inropes and avail the highest airway management expertise. Extracorporeal membrane oxygenation (ECMO) team activation for standby preparedness in case of cardiovascular collapse or elective placement has become routine in comprehensive pediatric heart centers.

Delaying intubation has negative effects in adults with HF. Van Diepen et al$^{40}$ reported that each 1-hour delay in mechanical ventilation initiation was associated with increased 30-day mortality in adult patients with refractory CS from myocardial ischemia who required mechanical ventilation. Such data, however, are lacking in children.

Enteral and Parenteral Nutrition

The cause of malnutrition in pediatric HF is multifactorial.$^{41}$ In addition, children with HF often present with tachypnea, tachycardia, fatigue, nausea, and vomiting and as a result may not be able to meet their estimated nutrition requirements through oral intake alone.$^{42}$ Estimating caloric, protein, and fluid needs for patients with HF can be especially challenging given that their body weight may fluctuate greatly with fluid status. A recent joint definition released by the American Society of Parenteral and Enteral Nutrition and the Michigan Pediatric Malnutrition Diagnostic Tool provides a categorical schema classify-

ing malnutrition severity according to a child's Z score for height/length, weight, and body mass index. Categorization of American Society of Parenteral and Enteral Nutrition malnutrition severity based on weight at a time when the patient was believed to be euvolemic, defined as the lowest weight within a 7-day period, can help provide a more accurate estimated requirement calculation without overfeeding.$^{43}$ The prevalence of wasting in pediatric HF has been reported to be as high as 86%, highlighting the importance of nutrition assessment through all stages of treatment to provide appropriate and adequate intake of energy, protein, and micronutrients. Once the patient is hemodynamically stable and preferably within the first 48 hours of admission, delivery of enteral nutrition should be prioritized.$^{41}$

Enteral nutrition support can be provided through gastric feeding or small-bowel feeding and is preferred because of gut integrity maintenance and the lower likelihood of metabolic complications such as refeeding syndrome. Gastric feeding offers metabolic and lifestyle benefits. Small-bowel feedings are indicated for patients with gastric complications, gastroparesis, or high aspiration risk. Patients should be monitored closely for tolerance and complications related to enteral feeding.

Parenteral nutrition should be considered only when an enteral route is not medically feasible. Candidates should be evaluated for risk versus benefit because parenteral nutrition can lead to a higher risk of infection from bacterial translocation. In a multicenter randomized controlled trial of 1440 critically ill children, of whom >35% were cardiac patients, withholding the start of parenteral nutrition for 7 days in the pediatric ICU was clinically superior to providing early parenteral nutrition and was associated with a shorter duration of mechanical ventilatory support, lower need for renal replacement therapy, and shorter duration of hospital stay.$^{44}$

VADs AND ECMO

MCS encompasses ECMO and VAD support. Support with ECMO is indicated when concomitant cardiac failure and pulmonary failure are present. Typically, VAD support can be provided in temporary (<15 days) or durable (>15 days) fashion.

When ADHF is refractory to medical management, MCS is the best next step. Device selection is influenced by the presence of congenital heart disease or other anatomic constraints, institutional resources, and the cause of ADHF.

Peripheral Cannulation Strategies for MCS

Globally, ECMO is the most widely used MCS modality for ADHF in children.$^{45}$ Peripheral cannulation for vernoarterial (VA)-ECMO is frequently used in noncardiac surgical patients because of its rapid deployment and

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suitability across a wide range of patient sizes, offering both cardiac and pulmonary support. Limitations include vessel size constraints, especially in femoral cannulation, which is typically feasible in older children or adolescents and may require distal perfusion catheters through direct cannulation or through a Gore-Tex (W.L. Gore & Associates, Inc, Newark, DE) tube anastomosed to a major entering artery. In smaller children and infants, cervical cannulation may be needed through carotid artery cut-down and jugular vein access. The various configurations of VA-ECMO are often operator and center dependent.$^{46}$ Regardless of cannula location, peripheral ECMO increases afterload such that a failing myocardium, specifically the LV, may hinder ventricular recovery, which is discussed later.

Aside from ECMO, peripheral forms of MCS may also include the use of temporary MCS devices such as the use of ECMO cannulas as mentioned in the femoral configurations but without the need for an oxygenator (eg, RotaFlow, Getinge Group, Göteborg, Sweden; CentriMag, Abbott Medical, Pleasanton, CA). This can be done when pulmonary support is not felt to be needed and the primary mode of decompensation is either transient such as in sepsis or myocarditis or used as a bridge to durable support. Similarly, percutaneous MCS such as with a percutaneous microaxial pump (PMP; eg, Impella, Abbott, Danvers, MA$^{47}$) or intra-aortic balloon pump can be used to provide support when there is preserved pulmonary function and primarily ventricular dysfunction. Intra-aortic balloon pump is infrequently used in the pediatric population; however, there is growing experience with PMP support in children, although a size constraint remains with even the smallest version of such pumps. Adverse events with PMPs include vascular injury from the insertion site, hemolysis, and the potential for thromboembolic events. One additional advantage of the PMP technology is the option for biventricular support.

Central Cannulation

Cardiopulmonary support for postoperative cardiac surgery patients is the most common indication for ECMO through a central cannulation. De novo central cannulation of ECMO for ADHF is uncommon and may be highly influenced by center variation and institutional practices. Potential advantages to central cannulation strategies include the ability to generate higher flow rates with larger cannulas, especially if there are challenges in achieving desired flows with peripheral cannulation. In addition, introducing a left-sided heart vent is relatively straightforward with central cannulation; however, this does introduce the additional morbidities of a median sternotomy and an "open" chest, which increases the risk of infection and bleeding.$^{48}$

VADs that use central cannulation can be divided into temporary and durable MCS options. Temporary MCS

devices such as paracorporeal continuous-flow pumps can be used with or without an oxygenator and can be connected with cannulas used for cardiopulmonary bypass or ECMO. For long-term support devices, either paracorporeal or intracorporeal, this requires durable cannulas that are surgically sewn to the heart and vessels. In children with a normal 2-ventricle anatomy, when a paracorporeal pulsatile and continuous-flow VAD is implanted, the inflow cannula will be from the LV apex or left atrium with an outflow cannula to the aorta. Intra-corporeal continuous-flow devices use the own inflow connector of the pump that is sewn to the LV apex and with an outflow graft to the aorta. Both described strategies are examples of central cannulation with direct left-sided heart decompression using durable VADs with the intent to provide augmented cardiac output and reduced LV filling pressures for optimal support. The complications inherent to these types of durable VADs are well described in the literature and include infection, bleeding, and cerebrovascular accidents.$^{49}$

Ventricular Decompression

LV distention is a frequent complication of VA-ECMO due to afterload mismatch that occurs when preload is unable to compensate for an increase in afterload. The right ventricle is effectively decompressed in VA-ECMO from venous draining into the ECMO circuit; however, there may be variable LV decompression, which can lead to LV distention, increased LV end-diastolic pressure, and left atrial hypertension.

Although LV distention is a recognized complication, there is a divergence of opinion about the timing and strategies for LV decompression. Pediatric patients compared with adult patients may have worse LV compliance, which puts them at greater risk for LV distention in the setting of increased afterload in VA-ECMO. Therefore, pediatric patients may benefit more from early prophyactic LV decompression because the myocardium is more susceptible to maladaptive remodeling.$^{50,51}$ Evidence of LV distention may include decreased or lack of pulsatility on arterial waveform, pulmonary edema on chest x-ray, pulmonary hemorrhage, echocardiographic imaging demonstrating a lack of aortic valve opening, significant aortic valve regurgitation, LV dilation, decreased contractility, or signs of stasis. Early decompressive strategies may include medical interventions that decrease afterload, ranging from adjusting ECMO flows to the using medications that alter systemic vascular resistance. If signs of LV distention persist despite medical interventions, early decompressive strategies such as balloon atrial septostomy, percutaneous left atrial or LV vent placement, surgical LV vent placement, or the addition of a PMP often should be considered. Although infrequently used in pediatrics, an intra-aortic balloon pump can also be

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used for LV decompression. To date, no studies have demonstrated increased efficacy or superiority of any one specific strategy.

Transition From Temporary VAD to Durable VAD

Some patients who present with new-onset ADHF may initially be placed on temporary MCS such that the patient can be “rescued” from extremis and undergo recovery of end-organ function. This time that is afforded with temporary MCS will also allow further investigation into the cause of ADHF, especially in new or atypical presentations. Despite initial improvement in hemodynamics, however, patients may then become dependent on MCS, and if there are no contraindications of ongoing support, a decision point is often reached to convert to durable MCS. These decisions may be influenced by the size of the patient, presence of congenital or extracardiac abnormalities, underlying syndromes or conditions, adequate recovery of end-organ function, potential transplantation eligibility, and the patient or family’s wishes for quality of life. Such decisions will benefit from a multidisciplinary team approach in determining suitability in the conversion from temporary MCS to durable support.

Myocardial Recovery

Unloading the LV during VAD support commonly results in a reduction of LV size. It is rare, however, for reverse remodeling to lead to sustained improvement in LV function sufficient to remove the device. Such myocardial recovery occurs in only ≈5% of adult patients, although contemporary studies report more favorable results.$^{52,53}$ Among children, successful exploration rates are wide ranging and are influenced by age, diagnosis, and type of device. The use of guideline-directed medical therapy during and after VAD support is considered essential for myocardial recovery in adults. Although complete myocardial recovery is ideal, adults with partial recovery (ejection fraction >40%) have similar BNP concentrations, LV size, and degree of mitral valve regurgitation while supported compared with those with complete recovery.$^{52}$ This suggests that myocardial recovery is a continuum and that more patients may benefit from VAD exploration than previously thought. Data on long-term outcomes after VAD exploration in children are limited, but one of the largest studies reported a high event-free survival rate (84%) and sustained high functional capacity Class 1 (83%) at 10 years.$^{54}$

TRANSITIONS OF CARE AND BEST PRACTICES

Children hospitalized with ADHF who do not undergo cardiac transplantation are usually transitioned from intravenous to oral therapies before discharge. In adults,

initiation of guideline-directed medical therapy is recommended during hospitalization after clinical stabilization.$^{21}$ Among children, however, these medications are often not prescribed. Approximately 65% of children with ADHF are prescribed an angiotensin-converting enzyme inhibitor or angiotensin receptor blocker (ARB) at hospital discharge, and <20% are prescribed the standard combination of angiotensin-converting enzyme inhibitor or angiotensin receptor blocker, β-blocker, and aldosterone antagonist.$^{56}$ There is wide practice variability across centers, and prescription rates have not changed significantly over the past decade. This represents an opportunity for quality improvement in pediatric ADHF. Safety and efficacy data for sodium-glucose cotransporter-2 inhibitors are currently lacking in children with HF.

Multidisciplinary teams are necessary to optimize outcomes in pediatric patients with ADHF, including pediatric palliative care services for children with terminal or life-threatening conditions.$^{57}$

There are ample adult data to inform management strategies that could improve outcomes among children presenting with ADHF. Key points to consider derived from HF expert panels include the following: (1) defining and standardizing the workup of children with suspected ADHF; (2) understanding the path toward improvement continues through the process of decongestion, cardiac output improvement, transition to oral therapies, and connects with the initial postdischarge clinic visit; (3) appreciating the need to continuously assess the clinical trajectory after admission to ascertain whether the patient is improving toward the target or stalling after initial response; (4) recognizing that failure to improve, including failure to tolerate guideline-directed medical therapy for HF, is typically associated with a much worse prognosis; (5) flagging the transition day to oral diuretic therapies as a trigger to verify steps for homegoing readiness, including completion of family, other caregiver, and patient education and medical milestones to be achieved before discharge; (6) using the discharge day as a time to review and communicate outpatient care instructions with referring health care professionals rather than abruptly changing or initiating new therapies; and (7) documenting critical events of the hospitalization and plans of care in a format that is easily accessible to the outpatient team and is available when the patient or the family calls with worsening symptoms.$^{58,59}$

For pediatric patients hospitalized with their first ADHF exacerbation, readmission rates are high, with estimates of 22% for 30-day HF-related readmissions.$^{8}$ Those at highest risk of readmission were identified as infants. Close multidisciplinary follow-up has been shown in adult HF trials to reduce all-cause readmissions and all-cause mortality,$^{60}$ although the literature is limited in pediatrics. Identifying which patients are at highest risk of readmission and having close follow-up management in the outpatient setting are crucial to the prevention of readmission.

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Behavioral Health

A critical component of multidisciplinary care is awareness of the profound mental health burden among children and adolescents with congenital and acquired heart disease, including those with ADHF. In a recent American Heart Association scientific statement,$^{61}$ children with HF were identified as at heightened risk of psychological symptoms because of the complex nature of their condition, frequent hospitalizations and medical interventions, and limitations of daily activities. Anxiety and depression are common, along with symptoms of acute and posttraumatic stress, especially among those who have experienced prolonged hospital stays.$^{62}$ This mental health burden affects not only the child but also their caregivers and other family members, who may experience significant and enduring emotional distress and, in some cases, mental health conditions. Grief, loss, and uncertainty surrounding the medical trajectory and its effect on daily life can exacerbate psychological distress for both patients and their families.

To address these difficulties, a range of prevention and intervention strategies is recommended. Regular mental health screening for patients and caregivers allows early identification of psychological symptoms

and referral to appropriate services. Integrating mental health professionals, psychologists, social workers, child life specialists, and psychiatrists into the multidisciplinary cardiac care team facilitates timely access to specialized, evidence-based mental health treatments.$^{63}$ Psychotherapy can alleviate psychological symptoms in patients of all ages. Family therapy can foster open communication and coping, and peer support groups offer a community of understanding and shared experiences to promote psychological resilience and emotional health.

RESEARCH NEEDS AND FUTURE DIRECTIONS

As the population of children with congenital and acquired heart diseases increases, pediatric cardiologists, intensivists, anesthesiologists, surgeons, and all members of the cardiac care team will need to become increasingly facile in managing those presenting with ADHF. A systematic approach to evaluating and managing them across their hospital course is essential. Figure 3 proposes a structural approach that can be used to treat children with ADHF, ensuring continuity of care from ED to hospital discharge.

 Figure 3. Systematic evaluation and management of a patient presenting with acute decompensated heart failure in the emergency department, in the intensive care unit, and on the inpatient floor.

Clinicians should be facile in escalating care based on the rapidly evolving patient condition and use appropriate hemodynamic support to rescue and reverse the ongoing pathophysiology. Clinicians should also use this opportunity to start oral heart failure (HF) therapies and upittrate before discharge. Ultimately, a comprehensive discharge plan discussing the different aspects of HF care should be outlined and explained to the family to ensure a smooth transition to the outpatient HF clinic. BNP indicates B-natiruetic peptide; CHD, congenital heart disease; CS, cardiogenic shock; CVP, central venous pressure; CXR, chest radiograph; ECMO, extracorporeal membrane oxygenation; ICU, intensive care unit; MVO2, mixed venous oxygen saturation; and INRS, near-infrared spectroscopy.

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CLUNICAL STATEMENTS AND EUDINELINES

Knowledge gaps that would benefit from organized, multi-institutional trials and that should be considered are (1) identifying patient profiles at highest risk for ADHF admission; (2) evaluating the optimal vasoactive agent for patients with varying hemodynamic profiles of CS; (3) creating a pediatric grading system for identifying, evaluating, and managing pediatric patients with varying stages of CS; (4) determining the timing and method of ventricular decompression; (5) developing a risk stratification tool that helps guide therapy for those presenting with ADHF; (6) determining the utility of pulmonary artery pressure-guided treatment to reduce HF readmissions; (7) developing machine learning risk prediction models that leverage electronic health data and bedside hemodynamic monitoring to identify those at highest risk for decompensation and need for advanced cardiac therapies; and (8) identifying optimal strategies for preventing or minimizing pediatric and caregiver traumatic stress and improving quality of life.

As this document highlights, children presenting with ADHF are at highest risk of death, need for advanced cardiac therapies, and hospital readmission. Hence, there is an urgent need to establish a multi-institutional collaborative involving key clinical, research, and patient-family stakeholders to ensure a systematic approach that addresses critical knowledge gaps and improves outcomes for children with ADHF.

ARTICLE INFORMATION

The American Heart Association makes every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.

This statement was approved by the American Heart Association Science Advisory and Coordinating Committee on December 5, 2025, and the American Heart Association Executive Committee on March 2, 2026. A copy of the document is available at https://professional.heart.org/statements by using either "Search for Guidelines & Statements" or the "Browse by Topic" area. To purchase additional reprints, call 215-356-2721 or email Meredith.Edelman@wolterskuwer.com

The American Heart Association requests that this document be cited as follows: Cabrera AG, Price JF, Hong BJ, Jeewa A, Tabulov C, Wong SS, Reardon L, Kasparian NA, Amdani S; on behalf of the Young Hearts Pediatric Heart Failure and Transplantation Committee of the Council on Lifelong Congenital Heart Disease and Heart Health in the Young; the Council on Clinical Cardology; the Council on Cardiovascular and Stroke Nursing; and the Council on Cardiovascular Radiology and Intervention. Evaluation and management of the child with acute decompensated heart failure: a scientific statement from the American Heart Association. Circulation. 2026;153:·········...doi: 10.1161/CIR.000000000000001428

The expert peer review of AHA-commissioned documents (eg, scientific statements, clinical practice guidelines, systematic reviews) is conducted by the AHA Office of Science Operations. For more on AHA statements and guidelines development, visit https://professional.heart.org/statements. Select the "Guide-lines & Statements" drop-down menu, then click "Publication Development."

Permissions: Multiple copies, modification, alteration, enhancement, and distribution of this document are not permitted without the express permission of the American Heart Association. Instructions for obtaining permission are located at https://www.heartorg/pemissions. A link to the "Copyright Permissions Request Form" appears in the second paragraph (https://www.heartorg/en/about-us/statements-and-policies/copyright-request-form). Heart Association.

Disclosures

Writing Group Disclosures

|Writing group member|Employment|Research grant|Other research support|Speakers' bureau/ honoraria|Expert witness|Ownership interest|Consultant/ advisory board|Other| |---|---|---|---|---|---|---|---|---| |Antonio G. Cabrera|Nationwide Children's Hospital|None|None|None|None|None|None|None| |Shahnawaz Amdani|Cleveland Clinic Children's Hospital|University of Michigan: The Impact of Fontan Circulatory Failure, Frailty, and Resilience on Heart Failure and Transplant Outcomes (site PI, receives no salary support)*; Mezzion: Fontan Udenafil Exercise Longitudinal Assessment Trial (the FUEL-2 Study; site PI, receives no salary support)*|None|None|None|None|None|None| |Borah J. Hong|Seattle Children's Hospital|None|None|None|None|None|None|None| |Aamir Jeewa|The Hospital for Sick Children Toronto (Canada)|Berlin Heart (Clinical Events Committee, co-chair)*|None|None|None|None|None|None| |Nadine A. Kasparian|Cincinnati Children's Hospital Medical Center Heart Institute Heart and Mind Wellbeing Center|NIH (NHLBI UM1)†; The Brett Boyer Foundation (Discovery Award)†; additional ventures (Single Ventricle Research Fund)†; American Heart Association (Visiting Professorship Award)†; Children's Heart Alliance of Cincinnati (research award)†; NIH (pending NHLBI)†|None|None|None|None|None|Cincinnati Children's Hospital Medical Center (director, Heart and Mind Well-being Center)††|

(Continued)

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Writing Group Disclosures (Continued)

|Writing group member|Employment|Research grant|Other research support|Speakers' bureau/ honoraria|Expert witness|Ownership interest|Consultant/ advisory board|Other| |---|---|---|---|---|---|---|---|---| |Jack F. Price|Texas Children's Hospital|None|None|None|None|None|None|None| |Leigh C. Reardon|Ahmanson/UCLA Adult Congenital Heart Disease Center|None|None|Actelion/ Janssen*|None|None|None|None| |Christine E. Tabulov|University of South Florida|None|None|None|None|None|None|None| |Sally S. Wong|American Heart Association|None|None|None|None|None|None|None|

This table represents the relationships of writing group members that may be perceived as actual or reasonably perceived conflicts of interest as reported on the Disclosure Questionnaire, which all members of the writing group are required to complete and submit. A relationship is considered to be "significant" if (a) the person receives $5000 or more during any 12-month period, or 5% or more of the person's gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $5000 or more of the fair market value of the entity. A relationship is considered to be "modest" if it is less than "significant" under the preceding definition.

*Modest.

†Significant.

Reviewer Disclosures

|Reviewer|Employment|Research grant|Other research support|Speakers' Bureau/ honoraria|Expert witness|Ownership interest|Consultant/ advisory board|Other| |---|---|---|---|---|---|---|---|---| |Rebecca Ameduri|Mayo Clinic|None|None|None|None|None|None|None| |Elizabeth Blume|Boston Children's Hospital|None|None|None|None|None|None|None| |Ashwin K. Lal|University of Utah|None|None|None|None|None|None|None| |Kimberly Yee Lin University of Pennsylvania|Children's Hospital of Philadelphia/ University of Pennsylvania|None|None|None|None|None|None|Cytokinetics*| |Kurt R. Schumacher|University of Michigan/Michigan Medicine|None|None|None|None|None|None|None|

This table represents the relationships of reviewers that may be perceived as actual or reasonably perceived conflicts of interest as reported on the Disclosure Questionnaire, which all reviewers are required to complete and submit. A relationship is considered to be "significant" if (a) the person receives $5000 or more during any 12-month period, or 5% or more of the person's gross income; or (b) the person owns 5% or more of the voting stock or share of the entity, or owns $5000 or more of the fair market value of the entity. A relationship is considered to be "modest" if it's less than "significant" under the preceding definition.

*Modest.

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