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INTRODUCTION
The purpose of this review is to summarize the current state of the art management of heart failure (HF) in children; however, given the broad spectrum, it will focus on systolic HF. In order to review the management, it is important that we briefly review the definition, causes, stages and physiology of HF in children. Detailed discussion about the diagnosis, work-up and investigations is beyond this scope of this review but is extensively reviewed in the literature.1)2)
What is heart failure?
HF has recently been defined as a “complex clinical syndrome with symptoms and signs that result from any structural or functional impairment of ventricular filling or ejection of blood.”3) In children it leads to characteristic signs and symptoms such as poor growth, feeding difficulties, respiratory distress, exercise intolerance and fatigue, and is associated with circulatory, neurohormonal, and molecular abnormalities. In the more severe cases, children can present with signs of low cardiac output and circulatory collapse. Treatment usually involves medications, nutritional support, activity modifications, and potentially surgical intervention, pacemaker, respiratory or mechanical support, or even heart transplantation. Managing HF requires a multi-disciplinary approach often involving paediatric cardiologists, intensivists, cardiovascular surgeons, nurses, dietitians, physiotherapists, social workers, and other healthcare professionals.
Causes and stages of heart failure in children
Etiologies of HF in children can be congenital or acquired but are multiple and diverse. Congenital heart defects presenting with HF can include left to right shunts (e.g. ventricular septal defects, aortopulmonary windows), valve abnormalities with either regurgitation or stenosis, and other structural abnormalities.4) Post-operative reasons are diverse and include abnormal physiology related to residual lesions, ischemic complications (e.g. coronary ischemia), sequelae of cardiopulmonary bypass, and arrhythmias to name a few.4) Cardiomyopathies, both primary and secondary, or other genetic conditions affecting the heart muscle are the other major category with dilated cardiomyopathies being most common, followed by hypertrophic and restrictive cardiomyopathies as summarized in other published guidelines.1) Less common but with unique management options include arrhythmias or dyssynchrony-induced HF.
Several grading/staging classifications exist including the American College of Cardiology/American Heart Association (AHA) staging ranging from patients at risk but without symptoms or signs of HF (Stage A), early disease/pre-heart failure (Stage B), HF symptoms (Stage C), and advanced HF (Stage D).3)5)6) The widely applied New York Heart Association (NYHA) staging is used for symptomatic patients (Stages C and D).3)6) Recently, Amdani et al.4) proposed a grading scheme for chronic HF in children and adolescents with congenital heart disease taking into consideration HF class, growth, circulating biomarkers (brain natriuretic peptide [BNP] or N-terminal prohormone of brain natriuretic peptide [NT-proBNP]), oxygen consumption on exercise test, invasive measurements and hospitalizations. Newer management strategies target early disease prior to overt HF.7)
More recently, adult HF classifications have been developed according to left ventricular ejection fraction (LVEF) with descriptors based on ventricular function.3) The terminology for heart failure with reduced ejection fraction (HFrEF) and heart failure with preserved ejection fraction (HFpEF) are increasingly being used in the pediatric population.4)
Basic heart failure physiology
Regardless of the cause, HF presentation reflects cardiac dysfunction and hemodynamic decompensation manifesting as signs of increased preload, afterload and decreased cardiac output which then leads to neurohormonal activation and a vicious cycle.
There are multiple maladaptive pathways activated in the failing heart.8)9) Neurohormonal activation is initially compensatory. Reduced cardiac output leads to reduced renal perfusion and reduced baroreceptor stimulation. This leads to activation of the renin-angiotensin-aldosterone system (RAAS) and the sympathetic nervous system leading to vasoconstriction, sodium and water retention, increased heart rate, increased contractility and vasoconstriction. This and other cellular and molecular pathways (decreased mitochondrial oxidation, increased glycolysis, abnormal fatty acid oxidation) unfortunately lead to unfavorable myocardial remodeling including myocyte hypertrophy, myocyte apoptosis, myocardial fibrosis, and subsequently chamber dilation and myocardial dysfunction which are bigger problems in the long-term.9)10) Eventually, compensatory mechanisms fail, and cardiac output decreases.
Medications that modify these maladaptive pathways and promote “reverse remodelling” of the myocardium are key to the management of HF and form the basis for “guideline directed medical therapy” (GDMT) as discussed in detail below.8)
Heart failure presentations
Presentation in acute HF manifests as characteristic signs and symptoms including poor growth, feeding difficulties, exercise intolerance, fatigue, abdominal symptoms including nausea and vomiting, tachycardia, and tachypnea. Chronic HF may be present with or without HF symptoms and is associated with structural remodeling of the heart. Acute decompensated heart failure (ADHF) is evident in the setting of an abrupt presentation, such as cardiogenic shock, either at initial presentation or as worsening of pre-existing underlying HF. One could consider the spectrum of HF to include children at risk for developing cardiomyopathy (e.g. gene-positive but phenotype negative), and asymptomatic children with cardiomyopathy7); but this review will focus on children with symptomatic HF.
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CHRONIC HEART FAILURE MANAGEMENT
Overarching principles
There is limited evidence for almost all medical therapies used in the management of HF in children.6)11) The vast majority of therapies are extrapolated from adult experience and directed towards the improvement of heart function due to systolic dysfunction.2)12)13) Diastolic HF, such as that seen in HFpEF, have even more limited evidence for medical therapies in children. The goal of therapy should be to relieve symptoms, promote reverse remodelling of the myocardium from the maladaptive changes, and to improve quality of life and survival.
An overview of the approach to management is summarized in Table 1 and Figure 1 by stage of HF including lifestyle, activity, pharmacotherapy, cardiac implantable electronic devices, and supportive interventions addressing factors such as growth, nutrition, anemia, and respiratory issues. There is even less data available to support the optimal management of chronic HF in children with repaired congenital heart disease; pharmacologic and non-pharmacologic interventions are extrapolated from adult data and implemented based on expert opinion, experience and consensus.1)4)11) Children with abnormal heart function should be managed by a multidisciplinary team with experience in HF management given the complexities and the survival benefit from guideline directed wholistic HF management, in addition to the need to identify and refer early those that will need to progress to advanced HF therapies such a ventricular assist device or heart transplantation.3)4)
 | Figure 1Approach to the management of the pediatric patient with heart failure with reduced ejection fraction (HFrEF).ACEI = angiotensin-converting enzyme inhibitor; ARB = angiotensin receptor blocker antagonist; ARNI = angiotension receptor-neprilysin inhibitor; CRT = cardiac resynchronization therapy; ECMO = extracorporeal membrane oxygenation; GDMT = guideline directed medical therapy; HF = heart failure; ICD = implantable cardiac defibrillator; MRA = mineralocorticoid receptor antagonist; LVEF = left ventricular ejection fraction; SGLT2i = sodium-glucose cotransporter-2 inhibitor; TIC = tachycardia-induced cardiomyopathy; VAD = ventricular assist device.
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Table 1
Overview of management of HF by stage
Adapted from the Hospital for Sick Children, Pediatric Dilated Cardiomyopathy Management Protocol 2024 (used with permission).
ACEI = angiotensin-converting enzyme inhibitor; AED = automated external defibrillator; ARB = angiotensin receptor blocker antagonist; ARNI = angiotension receptor-neprilysin inhibitor; CPAP = continuous positive airway pressure; CRT = cardiac resynchronization therapy; DCM = dilated cardiomyopathy; EF = ejection fraction; eGFR = estimated glomerular filtration rate; GDMT = guideline directed medical therapy; HF = heart failure; HFpEF = heart failure with preserved ejection fraction; HFrEF = heart failure with reduced ejection fraction; ICD = implantable cardiac defibrillator; LVNC = left ventricular noncompaction; MRA = mineralocorticoid receptor antagonist; NYHA = New York Heart Association; QOL = quality of life; SGLT2i = sodium-glucose cotransporter-2 inhibitor; ULN = upper limit of normal; VAD = ventricular assist device; VT = ventricular tachycardia.
Pharmacologic therapies
The goal of standard HF therapies is to establish combination therapy with oral medications, based on adult experience showing reduced hospitalizations and mortality, which is referred to as GDMT.3) GDMT is strongly recommended by multiple international organizations with published guidelines2)3)6)8)13)14)15)16)17)18)19) and includes angiotensin-converting enzyme inhibitors (ACEIs) or angiotension receptor-neprilysin inhibitors (ARNIs), beta-blockers, mineralocorticoid receptor antagonists (MRAs), and most recently, sodium-glucose cotransporter-2 inhibitors (SGLT2is). Introduction of GDMT should occur as soon as possible after the initial HF presentation with rapid sequential medication introduction and uptitration to target or tolerated doses within the first weeks to months.20)21) An overview of the approach to pharmacologic management is summarized in Table 1 and Figure 1 by stage of HF.
Diuretics
Diuretics play a role in the setting of symptomatic congestive HF and should be implemented as soon as possible following presentation to relieve symptoms of fluid overload.3) Though there are no pediatric trials, data in adults support improved symptoms, quality of life and improved exercise tolerance.22)23) First line is most commonly a loop diuretic (i.e. furosemide). Additive therapy may be required typically with a thiazide diuretic (hydrochlorothiazide or metolazone). Electrolytes and renal function should be monitored. Caution should be used in the setting of restrictive physiology due to dependence on preload (e.g. restrictive or hypertrophic cardiomyopathy). As patients transition into stable chronic HF, diuretics should be weaned and discontinued as tolerated.
Angiotensin converting enzyme inhibitors
ACEIs (e.g. captopril, enalapril, ramipril, perindopril) counteract the negative effects of the upregulation of the RAAS including reducing afterload and decreasing wall stress in the failing heart. Tolerance should be tested with a smaller test dose and the medication uptitrated to the highest tolerated dose within the dosing guidelines (Table 2). Patients should be monitored for hypotension and renal dysfunction. ACEIs should be avoided in the neonatal period. Currently, ACEIs are often the first line therapy for patients presenting with a new diagnosis of HFrEF,3) but this is slowly being overtaken by the angiotensin-receptor-neprilysin inhibitor (see below).24) Of note, in patients with congenital heart disease the one randomized placebo-controlled trial of enalapril (in infants with single ventricle) did not show benefit, however, ACEIs continue to be used in this patient population for HF management.25)
Table 2
Heart function dosing and uptitration guidelines
Adapted from the Hospital for Sick Children, Pediatric Dilated Cardiomyopathy Management Protocol 2024 (used with permission).
ACEI = angiotensin-converting enzyme inhibitor; ARNI = angiotension receptor-neprilysin inhibitor; bid = twice a day; GA = gestational age; q12h = every 12 hours; q6h = every 6 hours; q8h = every 8 hours; qAM = once a day in the morning; qd = once a day; qid = four times a day; qod = every other day; SGLT2i = sodium-glucose cotransporter-2 inhibitor.
Angiotensin receptor blocker antagonists/angiotensin receptor-neprilysin inhibitor
Angiotensin receptor blocker antagonists (ARBs; e.g. valsartan, losartan, irbesartan) have similar effects to ACEIs and historically have been used in patients intolerant of ACEIs.12) Currently one is available as a combination drug with a neprilysin inhibitor (sacubitril-valsartan) which promotes natriuresis, and is standard of care in adults,24) Though there is an ongoing pediatric trial,26)27) sacubitril-valsartan has and Drug Administration for use in children greater than one year of age based on a reduction in NT-proBNP over 12 weeks and is quickly becoming the drug of choice for GDMT in children.28) Uptitration as tolerated is also required for an ARNI (Table 2).
Beta-blockers
Beta-blockers have been shown to have the highest mortality benefit in adult with HF3) and continue to be recommended for all children with HFrEF as part of GDMT. Carvedilol is the recommended first choice but alternatives (e.g. metoprolol, propranolol, bisoprolol) can be considered especially if there are concurrent rhythm, hypertension, tolerance or other concerns (Table 2). Patients should be monitored for hypotension, bradycardia and hypoglycemia (in infants) with consideration given to dose reduction if necessary. Beta blockers should be used with caution in children with a single ventricle systemic right ventricle (RV) as the pediatric carvedilol did trend towards a worse outcome than placebo in this subgroup.4)29)
Mineralocorticoid receptor antagonists
MRAs (e.g. spironolactone, eplerenone) promote reverse remodeling of the myocardium due to their antifibrotic effects and are recommended in HFrEF (with adequate renal function and no hyperkalemia) due to adult studies showing improvement in mortality and HF hospitalizations (Table 2).3)30) For patients with congenital heart disease, most specifically single ventricle with RV morphology, fibrosis is not a significant contributor to RV failure so the use of MRAs may not be effective based on underlying physiology.31)32)
Sodium-glucose cotransporter-2 inhibitors
SGLT2is are a new class of medication that are quickly becoming standard of care for adults with symptomatic HF.3)33)34) There is currently limited but increasing experience in children and it is reasonable to consider adding an SGLT2i to established GDMT in the setting of ongoing symptomatic HF (Table 2).35)36)37) Uptitration is based on testing for glucosuria. Patients should be monitored for urinary tract infections, hypotension, volume depletion and hypoglycemia. There is evolving clinical experience for use in adults and children with congenital heart disease including a systemic RV.35)37)38)39)
Other adjuvant heart failure medications
Several other medications with limited experience and/or evidence in paediatrics may have an adjuvant role in patients with symptomatic HFrEF despite GDMT or intolerant of GDMT. These medications are not first line but could be considered in addition to GDMT. Digoxin is a cardiac glycoside that can be beneficial, especially with regards to HF symptoms but there is a paucity of data in pediatric HF other than in the single ventricle population where observational studies have shown a lower interstage mortality in stage I palliated patients on digoxin.40)41) Ivabradine has a mechanism of action that slows the heart rate and can be used in the setting of ongoing tachycardia and in patients intolerant of beta blockers due to hypotension. It has been shown to reduce mortality and HF hospitalizations in adults.13)42) In children with dilated cardiomyopathy, improvement in functional class, NT-proBNP, and LVEF has been demonstrated with the use of ivabradine.43) There is minimal experience on the use of ivabradine in HF in children with congenital heart disease. Vericiguat is a novel soluble guanylate cyclase inhibitor which promotes vascular smooth muscle cell relaxation through the release of endogenous nitric oxide and potential beneficial effects on endothelial function, decreasing fibrosis and reverse remodelling.44) There is an ongoing pediatric trial (ClinicalTrials.gov NCT06428383#) looking at the role of vericiguat in children with reduced LVEF (including congenital heart disease).
Nutrition
Malnutrition is associated with adverse clinical outcomes and mortality in pediatric dilated cardiomyopathy patients as reported from the Pediatric Cardiomyopathy Registry in over 900 patients.45) Optimizing nutrition is necessary for the management of HF but children in HF often have decreased appetite, feeding intolerance, poor absorption of nutrients, and poor growth (due to both inadequate intake and increased energy expenditure). The ability to optimize nutrition and establish a good growth trajectory is often important in judging response to GDMT and in making decisions about consideration of advanced HF therapies including heart transplantation. Strategies to optimize caloric intake to meet energy requirements, ideally with the support of a dietitian/nutritionist, include higher calorie feeds, supplemental enteral feeds especially in infants and young children (initially nasogastric tube with consideration of a gastrostomy tube), and very rarely parental nutrition (see below).4)
Fluid restriction
Overall fluid restriction may not be required in stable patients with chronic HF who are well controlled on GDMT. However, attention should be given to minimizing water intake and focusing on fluids with nutritional content instead (e.g. infant formula, nutritional supplements, milk). In the setting of congestion and/or HF symptoms, fluid restriction, most specifically water, is often necessary in the early phase.
Sodium restriction
Avoiding high sodium intake should be considered in symptomatic HFrEF but supportive literature is lacking.3) In general sodium supplementation is not recommended for the hyponatremia associated with HF. Instead, consideration should be given to fluid restriction, especially water, and diuretic choice.
Anemia
Anemia is common in patients with HF and has been correlated with adverse outcomes in adult and pediatric HF patients.46)47) A basic evaluation for causes of anemia should be carried out and any identified issues addressed. Most common is iron deficiency which has been reported in almost two thirds of a single center pediatric HF cohort and associated with worse clinical outcomes.47) Enteral iron supplementation may not be adequate, especially in the setting of feeding intolerance, and consideration should be given to intravenous iron supplementation. Blood transfusion should be considered in patients with a hemoglobin less than 100 g/L with acute symptomatic HF.
Anticoagulation
Children with reduced systolic function are at an increased risk of intracardiac thrombi and thromboembolism. There are no widely accepted consensus guidelines for prophylactic anticoagulation but in general consideration should be given in patients with sustained severe left ventricular dysfunction (ejection fraction <25%). Options include aspirin, low molecular weight heparin, a vitamin K antagonist (e.g. warfarin), or a direct oral anticoagulant (DOAC; e.g. rivaroxaban, apixaban). Aspirin is often used as first line with vitamin K antagonist or low-molecular-weight heparin or DOAC based on the presence of intra-cardiac thrombus, thromboembolic event or any history of stroke, and/or resistent or paroxysmal atrial fibrillation or atrial flutter. Age should be taken into consideration with infants less than 12 months of age most commonly receiving low molecular weight heparin, and children over 12 months of age more commonly receiving warfarin or increasingly a DOAC (though pediatric data is limited).
Exercise and rehabilitation
Regular at least moderate physical activity as tolerated should be encouraged to improve functional health status and quality of life.3)48)49) In patients with significant exercise limitations, recommendations should be made with expertise from experienced physiotherapists and/or exercise physiologists. Case-by-case discussions with patients and families regarding activity, exercise and participation in sports should be done in a shared decision-making model taking into consideration the holistic benefits in comparison to the risks of the underlying heart disease.
Respiratory considerations/ventilation
Sleep disordered breathing including obstructive sleep apnea is common in adults with HF but not children.12)50) However, when present, it does increase left ventricular afterload and contributes towards desaturation, both potentially exacerbating HF. Non-invasive ventilation is not commonly utilized in the setting of chronic HF but should be used in the setting of significant sleep apnea or sleep-disordered breathing. Continuous positive airway pressure (CPAP) ventilation may reduce left ventricular afterload and contribute to increasing cardiac output while simultaneously helping reduce the work of breathing and shortness of breath due to decreased lung compliance from pulmonary edema.
Electrophysiology considerations
It is imperative with any patient with a HF presentation, to evaluate for any arrhythmia or related factors that could be contributing including tachyarrhythmias, bradyarrhythmias, heart block, bundle branch block or pre-excitation.
Dilated cardiomyopathy with pre-excitation is associated with mechanical dyssynchrony that worsens adverse ventricular remodeling. Ablation of the accessory pathway has been associated with partial to complete recovery of ventricular function in dilated cardiomyopathy patients.51)52)53) Ablation may also be indicated in children with tachycardia-induced cardiomyopathy refractory to medical management.51)54)
Cardiac resynchronization therapy (CRT) is and established treatment for electromechanical dyssynchrony and HF in adult HF with notable improvement in heart function and survival.12)55)56) CRT devices have three leads (1 atrial and 2 ventricular) so that multi-site pacing can be done to optimize mechanical contraction and improve function. CRT devices can be both pacemakers and defibrillators. CRT use has been reported in children both with structurally normal hearts and congenital heart disease with good effect57) and is included in pediatric HF guidelines.6) CRT should be considered in symptomatic children who are optimized on medical HF management but with ongoing systemic left ventricle (LV) and/or RV dysfunction (EF <45%), wide QRS duration (native/paced greater than the upper limit of normal for age), and mechanical dyssychrony as assess by echocardiography.57)
Surveillance and recovery
Predictors of functional recovery are lacking in the pediatric population with existing literature reporting a 22% recovery from the Pediatric Cardiomyopathy Registry.59) Following the establishment of GDMT at target and/or tolerated doses, typically patients are followed by echocardiography after a minimum of 3 months. However, the optimal timing to reassess for reverse remodelling and functional recovery remains to be determined as there is adult experience in both ischemic and non-ischemic cardiomyopathy showing progressive recovery out to one year following initiation of GDMT. Circulating biomarkers such as BNP or NT-proBNP can also help guide response to therapy though data in children is limited, especially in certain age groups (e.g. infants) and in the setting of co-morbidities (e.g. renal dysfunction, pulmonary hypertension).60)
Medication withdrawal following recovery
Consideration of withdrawal of GDMT in patients with improvement in function is not well studied and likely is impacted by the underlying etiology. Easiest to consider is the patient with documented myocarditis and full recovery of function over time. More challenging, however, is the patient with a known genetic cardiomyopathy, presenting the dilemma of whether to treat indefinitely or consider medication withdrawal. There is little to no reported experience in the literature in children though the TRED-HF trial in adults reported one third to one half had recurrent ventricular dysfunction and cautioned against medication withdrawal.61)
If a decision is made to move forward with withdrawal of medications, HF should have resolved with normalization of systolic function and chamber dimensions that is sustained for at least 12–24 months (this may vary based on duration of dysfunction and underlying cause of cardiomyopathy). For older children and adolescents, ACEI may be stopped first followed by beta-blockers in 6 months if follow-up echocardiogram shows preserved function. Beta-blocker dose should be halved and then stopped in 2 weeks to avoid rebound tachycardia. Younger patients may be permitted to grow out of their doses with annual follow-up. Echocardiogram should be repeated in 6 months to ensure no relapse followed by ongoing long-term routine follow-up surveillance.
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ACUTE HEART FAILURE
There are many reasons why a patient may present in acute HF.62) Post-operative dysfunction usually is transient, and the focus is on short-term support. Acute cardiogenic shock with emergent or semi-urgent resuscitation followed by recovery or chronic care can be seen with many etiologies including myocarditis, a new presentation of cardiomyopathy, or sepsis to name a few. Then there are the patients with an exacerbation of chronic HF like a chronic dilated cardiomyopathy who is now failing medical management leading to a chronic HF patient who becomes dependent on systemic medications, technology or mechanical support (Figure 1).
Acute decompensated heart failure
Children presenting in ADHF have high morbidity and mortality regardless of whether they have a structurally normal heart or congenital heart disease.62)63) Patients can be classified into one of four hemodynamic categories based on presence or absence of congestion (dry/wet) and/or perfusion (warm/cold). The approach to management will be guided by clinical presentation. The therapeutic aim is to convert patients to a “warm and dry” state (well perfused and no congestion). Patients that present in a “warm and wet” state (well perfused but congested) may only need diuretics for their initial management. However, patients with a “cold and wet” presentation will likely need vasodilators to reduce afterload and improve cardiac output in addition to diuretics. In addition, in children, inotropic agents are often used to improve perfusion, especially in the “cold and dry” presentation.63)64)
Fluid management and diuretics
Adequate and prompt fluid removal to achieve symptomatic improvement is key. Careful monitoring for hypotension, electrolyte abnormalities, and worsening renal function is necessary. Diuretics may be the only therapy needed to improve symptoms in the patient with adequate perfusion. Conversely, early lack of response to diuretics has been associated with worse outcomes in children including use of mechanical circulatory support (MCS) and death,65) potentially identifying patients that might benefit from earlier institution of other therapies.
Inotropes
Inotropes improve contractility, increase cardiac output, lower end-diastolic pressure, and some reduce afterload; all of which improve end organ function in patients with low cardiac output.6) However, they do this at the expense of increasing myocardial oxygen demand. Due to the long-term association with increased mortality in adult studies, inotropes should only be used in the setting of ADHF or acute symptomatic HF unresponsive to or intolerant of the initiation of oral HF medications/GDMT.12)66)67) That being said, there is a lower threshold for use in the smallest infants or those with complex congenital heart disease given the risk of complications from mechanical support, especially ventricular assist devices (VADs).63)
In general, inotropes are indicated in the setting of ADHF with end organ dysfunction due to low cardiac output and are considered to be detrimental and contraindicated if this is not present. Inotropes should not be started solely for congestive symptoms (wet) in the absence of clinical evidence of low cardiac output (cool); more notably they should not be started on the basis of echocardiographic appearances alone. The choice of inotropic support will depend partly on clinical presentation from both the congestion and perfusion perspectives. Commonly used inotropes include milrinone, epinephrine and dobutamine which are frequently used but poorly studied.64) Different physicians and different intensive care units often prefer different inotrope combinations.63)
Milrinone, a phosphodiesterase-3 inhibitor, improves myocardial contractility and has both systemic and pulmonary vasodilator effects. It has been shown to improve low cardiac output syndrome post-cardiopulmonary bypass in children.68)69) It is preferred in children due to familiarity and better tolerance. Longer term use of milrinone in children has been shown to be safe and efficacious as a bridge to oral GDMT or transplantation70)71) in contrast to the increased mortality observed in the adult population.66) Milrinone can be used for home inotropic support in children unable to tolerate transition to enteral GDMT as a bridge to heart transplantation.71)72)
Epinephrine and dobutamine act on beta-1 and beta-2 adrenergic receptor agonists with some alpha-1 activity increasing stroke volume, heart rate, contractility and cardiac output. Dopamine acts on coronary, renal and mesenteric receptors leading to vasodilation and natriuresis but is rarely used in contemporary HF management.64)
Levosimendan increases cardiac contractility by calcium sensitization of troponin C and has vasodilatory properties leading to vasodilation. There is a paucity of data on the use of levosimendan in pediatric HF with limited evidence that it affects outcomes.73) It is not currently licensed for use in North America due to concern about lack of evidence supporting efficacy but is used in multiple countries around the world.
Inotropes clinically appear to improve perfusion and provide symptomatic relief.63)64) However, the goal should be to transition to chronic HF GDMT. If not possible, they then can be used as a bridge to both MCS and heart transplantation. Inotrope dependence and/or increasing inotrope needs with evidence of end-organ dysfunction should prompt consideration of MCS.74)
Vasodilators
Vasodilators such as nitroglycerin and nitroprusside, whose mechanism of action is via their metabolite nitric oxide, are commonly used in adult patients with HF. Despite an extensive experience in adults, there is limited reported experience for acute HF management in pediatrics, mostly in the setting of valvular regurgitation, and no pediatric data to support the use of one particular agent over another.64)75) Nitroprusside has the most history of use with a short half-life, and can be titrated to effect so is often the preferred choice in pediatric settings.
Vasopressors
Use of vasopressors such as norepinephrine or vasopressin should be avoid if at all possible unless there is symptomatic and/or severe hypotension with end-organ dysfunction despite standard inotrope therapy. Occasionally, however, they may be preferred over inotropes in the context of significant ventricular outflow tract obstruction (which can be worsened by inotropes) or myocardial ischemia (due to increase in myocardial oxygen demands with inotropes).
Mechanical ventilation
As noted above, non-invasive positive pressure ventilation such as CPAP or bilevel positive airway pressure may be considered given its effect of potentially reducing afterload on the LV. It may help with symptom relief of pulmonary edema/increased work of breathing and from apneic episodes. Caution should be exercised in the setting of significant right ventricular dysfunction.
In ADHF, invasive ventilation may need to be considered in patients especially with significant respiratory compromise. It should be performed in a controlled setting by experienced intensive care unit due to the high risk of cardiac arrest at the time of intubation and sedation.
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ADVANCED HEART FAILURE THERAPIES
Mechanical circulatory support
The indications and decision-making for MCS, including VADs, are complex and include patient-specific risk factors (size, anatomy, prior surgery, etc.) in addition to center experience and types of available devices. Guidelines do exist for the care of children in need of MCS.74) In general, end-stage HF on maximal therapies (inotropes, respiratory support, etc.) and with imminent end-organ dysfunction should be considered for MCS support. Patients should be cared for in a center with a multidisciplinary team experienced in the use of MCS for children in end-stage HF.
Transplant considerations
In the setting of worsening acute or acute on chronic HF, consideration should be given to heart transplantation prior to decompensation and the development of end-organ dysfunction which may impact transplant candidacy and/or perioperative survival.6) Timing of assessment and potential listing must also take into account the anticipated waiting time and ability to bridge to transplantation. For patients with ADHF with end-organ dysfunction, transplant candidacy may need to be deferred until recovery of end-organ function, stability on advanced support (e.g. chronic inotropic therapy or VAD), and nutritional and physical rehabilitation.
Palliative care
Both the acute and chronic courses and fluctuating trajectories in children with HF impose significant symptomatic, medical, emotional, developmental and psychological burdens on the child and family. Often the underlying disease and resultant HF are life limiting and should be discussed throughout the HF trajectory. Life is filled with uncertainty and cumulative acquired morbidities, all of this affecting quality of life. Palliative care importantly plays a role in improving quality of life and the lived experience for both the child and the family. This should include establishing goals of care and reassessing them regularly in addition to addressing symptoms, psychological and decision-making support, advanced care planning, and end of life care. The AHA reviewed all of the important considerations in their scientific statement on palliative care for children with heart disease.76)
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MULTIDISCIPLINARY APPROACH TO HEART FAILURE MANAGEMENT
The care of children with any stage of HF requires teamwork that is coordinated and both protocolized and individualized. Multidisciplinary teams of experts must work together to assess, diagnose, treat, and support the patient and the family across the spectrum of pediatric HF presentations and clinical courses (Figure 1). This can include pediatric cardiologists who specialize in HF, other pediatric cardiology subspecialists (electrophysiology, imaging, interventional catheterization, etc.), cardiovascular surgeons, cardiac intensivists, advanced nurse practitioners (HF, VAD and transplant), nurses, dietitians, physiotherapists, occupational therapists, social workers, neuropsychologists, pharmacists, child life specialist, amongst others. Input from different medical subspecialities can also be important including geneticists and genetic counsellors, neurologists, neuromuscular specialists, infectious diseases, nephrologists, and many others. The care trajectory for patients with HF is often life long and consideration must be given to a systematic and planned transition process from pediatric to adult services.
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SUMMARY
HF in children is complex with multiple diverse etiologies, severities of presentation, and fluctuating clinical courses over time. Morbidity and mortality are high. Treatment approaches are based predominantly on expert consensus guidelines extrapolated from adult evidence-based literature. The goal is to encourage reverse remodeling of the myocardium that is failing due to maladaptive changes related to the underlying HF pathophysiology. Establishing guidelines directed medical therapy is key concurrent with addressing other factors contributing to ventricular dysfunction and HF. Care requires diligent management by a multi-disciplinary HF team that can adapt to the needs of the different phases of childhood and adolescence with ongoing reassessment to maximize survival and quality of life across the patient’s lifespan.
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