Pediatric Congestive Heart Failure Guidelines

Updated: Apr 23, 2019
  • Author: Gary M Satou, MD, FASE; Chief Editor: Stuart Berger, MD  more...
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Guidelines

Guidelines Summary

In 2014, the International Society for Heart and Lung Transplantation (ISHLT) published updated guidelines for the evaluation and management of heart failure in children. Due to the lack of research trials in children, the majority of the recommendations were achieved by consensus. [12]

Acute Heart Failure

The ISHLT recommendations for management of acute HF are summarized below. All recommendations have a C level of evidence unless otherwise noted. [12]

Assessment and Monitoring

Class I

  • Diagnosis of acute HF is based on signs and symptoms of HF combined with supportive evidence from chest X-ray imaging, ECG, echocardiography, and laboratory evaluations. (Level of evidence: B)
  • Severity, including degree of congestion and adequacy of perfusion should be assessed.
  • Evalute etiology of HF, with focus on identifying reversible causes (e.g., repairable CHD, myocarditis, tachycardia-induced cardiomyopathy, and hypothyroidism).
  • Coronary angiography should be performed if coronary ischemia is suspected in the presence of other potential abnormalities that cannot be definitely excluded by non-invasive imaging.  
  • Cardiac catheterization is indicated in patients with palliated or repaired CHD who present with acute HF if a non-invasive evaluation fails to establish a definitive diagnosis.  
  • Perform serial testing to monitor for electrolyte abnormalities, hemoglobin levels, end-organ perfusion, and response to therapy.   
  • In hospitalized patients: consider observation in an ICU; evaluate and monitor for arrhythmias with continuous ECG monitoring/telemetry. 
  • Transfer patients with severe acute HF to a center with pediatric HF specialists and the expertise and ability to optimize medical therapy, evaluate for heart transplant, and if necessary, provide mechanical support.

Class IIa

  • For patients with decompensated HF, place intra-arterial catheters for continuous blood pressure monitoring. 
  • Consider central venous catheters should in decompensated HF to allow for measurement of central venous pressure and/or mixed venous saturations and to administer medications and fluids. 

Class III

  • Pulmonary artery catheterization is not recommended for routine use, but may be appropriate in selected patients. 

Management

Class I

  • IV inotropic support may be used temporarily in patients presenting as cardiogenic shock with poor systemic and end-organ perfusion.
  • Vasodilators may be when hypotension is not present. Vasodilators may also be used in combination with diuretics for symptomatic relief in patients with pulmonary edema.
  • Perform ongoing assessment of fluid status in all patients admitted to the hospital.
  • Diuretics are the first-line therapy for patients with evidence of fluid overload.
  • Monitor carefully for side effects of anti-congestive therapies, including renal function, electrolytes, and hypotension.

Class IIa

  • Intravenous inotropic support may be used temporarily in patients presenting as hypotension with evidence of low CO and compromised end-organ perfusion.
  • The choice of inotropic agent in acute decompensated HF depends on clinical presentation. Milrinone and/or dobutamine can be used as first-line rescue therapy, with epinephrine playing a role in the face of refractory hypotension and poor end-organ perfusion.
  • Fluid restriction for patients with acute HF, regardless of serum sodium level.

Class IIb

  • Consider levosimendan for acute decompensated HF unresponsive to traditional inotropic therapy.

Class III

  • Use of intravenous inotropic agents in the absence of clinical evidence of hypotension, impaired perfusion, low CO, and/or decreased end-organ perfusion is potentially harmful. (Level of evidence: B)

Medical Management of Chronic Heart Failure

The major recommendations for pharmacologic therapy are summarized in the table below. [12]

Table. Recommendations for Pharmacologic Therapy for Chronic Heart Failure (Open Table in a new window)

Drug Class

Recommendation

Reduced EF (systolic HF)

Preserved EF (diastolic HF)

Diuretics

Class I

Patients with fluid retention associated with ventricular dysfunction (HF Stage C) to achieve a euvolemic state

All patients to establish a euvolemic state 

 

Class I

 

Perform close monitoring of renal function and blood pressure during initiation and up-titration of diuretic therapy

 

Class IIa

 

Consider diuretics to treatment systemic hypertension to prevent disease progression.

Ace Inhibitors

Class I

(LoE B)

For symptomatic left ventricular dysfunction (HF Stage C), use ACE inhibitors routinely unless there is a specific contraindication. Start at low doses and up-titrate to a maximum tolerated safe dose

 
 

Class IIa

(LoE B)

For asymptomatic left ventricular dysfunction (HF Stage B), use ACE inhibitors routinely unless there is a specific contraindication. 

 
 

Class III

(LoE B)

Do not routinely use for patients with single-ventricle CHD, but could be considered in specific cases such as in situations of valve regurgitation or ventricular dysfunction

 

Angiotensin antagonists

Class IIa

Generally reserved for systemic ventricular systolic dysfunction in patients who are intolerant of ACE inhibitors

 
 

Class IIb

 

Routine use is not recommended unless there is an additional indication such as hypertension.

 

Class IIb

 

May be used for control of hypertension but careful monitoring of hemodynamics and renal function is required due to the enhanced risk of hypotension and renal toxicity.

Beta- Blockers

Class IIa

(LoE B)

Consider beta-blockers in symptomatic children with systemic LV systolic dysfunction, particularly if the systemic ventricle has a LV morphology. Therapy should start at a small dose and slowly up-titrate

 
 

Class IIa

(LoE B)

Consider beta-blockers in asymptomatic children with systemic LV systolic dysfunction. Therapy should start at a small dose and slowly up-titrate

 

Calcium Channel Blockers

Class III

 

Not recommended unless there is an additional indication. 

Inotropic Agents

Class III

Use of intermittent or chronic inotropic therapy, other than as a bridge to transplant, is not recommended.

 

Nesiritide

Class IIb

Not recommended for routine use although it may be considered in select patients where other interventions to lower central venous pressure have been unsuccessful.

 

Device-Based Therapies

The recommendations for device-based therapies are summarized below. [12]

Class I

  • Permanent pacemaker implantation for advanced second- or third-degree atrioventricular block associated with ventricular dysfunction. (Level of evidence: B)
  • In the pediatric survivor of cardiac arrest after evaluation to define the cause of the event and to exclude any reversible/treatable causes, perform ICD implantation. (Level of evidence: B)
  • In patients with tachycardia-induced cardiomyopathy when medical therapy fails, ablation therapy. (Level of evidence: B)

​Class IIa

  • Cardiac resynchronization therapy (CRT) for patients with a systemic LV with an EF < 35%, complete left bundle branch block pattern, QRS duration (native or paced) > ULN for age, NYHA Class II-IV on GDMT. (Level of evidence: B)
  • ICD implantation can be useful in the following groups:

    • Patient with unexplained syncope and at least moderate LV dysfunction and DCM. 
    • Adolescents with hypertrophic cardiomyopathy (HCM) and 1 or more major risk factors for sudden cardiac death (SCD).

    • Adolescent with AVC with 1 or more risk factors for SCD. 

    • Adolescent with a familial cardiomyopathy associated with SCD. 

  • In adolescents with tachycardia-induced cardiomyopathy, consider ablation therapy for primary therapy. (Level of evidence: B)

Class IIb

  • Consider CRT for patients with:
    • Systemic RV, with an EF < 35%, complete right bundle branch block pattern, QRS duration (native or paced) > ULN for age, NYHA Class II-IV on GDMT. 
    • Single ventricle, with an EF < 35%, complete bundle branch pattern, QRS duration (native or paced) > ULN for age, NYHA Class II-V on GDMT. 

  • Consider ICD therapy for the following groups:
    • Patients with DCM who have an LVEF < 35% and who are in NYHA Class II or III. 
    • Patients with CHD with syncope in the presence of ventricular dysfunction. 

    • Adolescents with LVNC and moderately depressed ventricular function. 

    • Non-hospitalized patients with non-sustained or sustained ventricular tachycardia who required a VAD. 

  • Consider ablation therapy in patients with frequent premature ventricular contraction and cardiomyopathy of unknown etiology when medical therapy fails. (Level of evidence: B)

Mechanical Circulatory Support

The recommendations for mechanical circulatory support (MCS) are summarized below. [12]

Class I

  •  For children who are unable to be weaned from inotropic support and are showing early, reversible dysfunction of at least 1 other major organ system, consider implantation of a durable ventricular assist devices (VAD) as a bridge to transplantation 

Class IIa

  • For a patient in cardiac arrest or cardiogenic shock with pulmonary compromise, ECMO should be considered for emergency cardiovascular support, as a bridge to recovery of function. 
  • For a patient with isolated cardiac failure that is believed to be reversible, consider ECMO or a temporary VAD as a bridge to recovery of function. If recovery does not occur, then transition to a chronic VAD for bridge to transplantation or for destination therapy is reasonable. 

  • For cardiogenic shock that is not believed to be due to a reversible underlying cause, consider use of a temporary VAD or ECMO for resuscitation of end-organ function rather than directly implanting a chronic VAD system. 

Class IIb

  • Consider implantation of a chronic VAD system as long-term support for patients who are not eligible for transplantation, provided a system is available that permits discharge to home with regular outpatient follow-up.
  • Use of BiVAD support should be reserved for patients who appear unlikely to achieve adequate hemodynamics from LVAD support alone.
  • Children who are supported on a chronic VAD system may be considered for a recovery protocol and weaning from VAD if recovery of cardiac function is documented.