Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

haemodynamically stable: no evidence of LV systolic dysfunction

1st line – supportive care ± treatment of underlying cause

Back
ACUTE
|
haemodynamically stable: no evidence of LV systolic dysfunction
1st line – 

supportive care ± treatment of underlying cause

Lymphocytic myocarditis does not require any therapy targeted at the underlying cause and the management is supportive. For those with documented influenza, antiviral agents may be beneficial. In those with SARS-CoV-2 infection, similarly, antiviral agents as well as monoclonal antibodies may be used. For more information on the treatment of influenza infection and SARS-CoV-2 infection, see Influenza infection and Coronavirus disease 2019 (COVID-19).

Giant cell myocarditis is treated with a combination of systemic corticosteroids and another immunosuppressive agent such as ciclosporin, muromonab-CD3, or azathioprine. While giant cell myocarditis is often rapidly fatal, immunosuppressive therapy has been shown to improve mortality.[10][91][94]

Systemic corticosteroids improve outcomes in patients with autoimmune-associated myocarditis.[55]

Hypersensitivity myocarditis is treated by removal of the offending agent and systemic corticosteroids.

Corticosteroid use can cause marked hyperglycaemia and increases the risk of infection. Chronic use is associated with bone mineral density loss and skin friability.

Patients with chagasic myocarditis should be treated with an appropriate antiparasitic agent; see Chagas' disease for detailed information on management. A permanent pacemaker with defibrillator is often necessary because of conduction abnormalities and ventricular arrhythmias.

Immune checkpoint inhibitor (ICI)-related myocarditis is treated by immediately stopping ICI treatment and giving high-dose intravenous methylprednisolone. This can be switched to a weaning dose of oral prednisolone if patients show ongoing signs of clinical improvement. Second-line immunosuppressive therapy is indicated for corticosteroid-resistant patients or for haemodynamically unstable patients with fulminant myocarditis. Various second-line agents are under investigation and specialist guidance is recommended. After recovery, a multidisciplinary team should review whether to restart ICI therapy.[32]

Primary options

Autoimmune-associated or hypersensitivity myocarditis or ICI-related myocarditis

methylprednisolone: children and adults: consult specialist for guidance on dose

OR

Giant cell myocarditis

methylprednisolone: children and adults: consult specialist for guidance on dose

-- AND --

ciclosporin: children and adults: consult specialist for guidance on dose

More

or

muromonab-CD3: children and adults: consult specialist for guidance on dose

or

azathioprine: children and adults: consult specialist for guidance on dose

Consider – intravenous immunoglobulin (IVIG)

Back
ACUTE
|
haemodynamically stable: no evidence of LV systolic dysfunction
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

There is insufficient evidence to routinely recommend intravenous IVIG for all patients with myocarditis. However, it may provide clinical benefit in some cases, and is frequently used to treat children in clinical practice.[24][90]

Primary options

normal immunoglobulin human: children and adults: 2 g/kg intravenously as a single dose

haemodynamically stable: evidence of LV systolic dysfunction

1st line – ACE inhibitor or angiotensin-II receptor antagonist or sacubitril/valsartan

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
1st line – 

ACE inhibitor or angiotensin-II receptor antagonist or sacubitril/valsartan

ACE inhibitors (e.g., captopril, enalapril, lisinopril) and angiotensin-II receptor antagonists (e.g., candesartan, valsartan) improve cardiac output and haemodynamics by arterial vasodilation. Chronic maladaptive cardiac remodelling is also attenuated.[57] Evidence suggests that the likelihood of progression to dilated cardiomyopathy is reduced with early initiation.[79] These medications may precipitate acute renal failure or hyperkalaemia. Serum potassium, urea and creatinine should be monitored.

The efficacy of sacubitril/valsartan for the treatment of heart failure has been well documented, and may be superior to ACE inhibitors, although data specifically for myocarditis are lacking. Sacubitril is a neprilysin inhibitor.[80]

Dose should be started low and increased gradually according to response.

Primary options

captopril: children: consult specialist for guidance on dose; adults: 6.25 to 50 mg orally three times daily

OR

enalapril: children: consult specialist for guidance on dose; adults: 2.5 to 20 mg orally twice daily

OR

lisinopril: children: consult specialist for guidance on dose; adults: 2.5 to 40 mg orally once daily

OR

candesartan: children: consult specialist for guidance on dose; adults: 4-32 mg orally once daily

OR

valsartan: children: consult specialist for guidance on dose; adults: 20-160 mg orally twice daily

Secondary options

sacubitril/valsartan: children: consult specialist for guidance on dose; adults (treatment-naive or treatment-experienced on a low dose): 24 mg (sacubitril)/26 mg (valsartan) orally twice daily initially, increase gradually according to response, maximum 97 mg (sacubitril)/103 mg (valsartan) twice daily; adults (treatment-experienced on a usual dose): 49 mg (sacubitril)/51 mg (valsartan) orally twice daily initially, increase gradually according to response, maximum 97 mg (sacubitril)/103 mg (valsartan) twice daily

More

Plus – sodium-glucose cotransporter-2 (SGLT2) inhibitor

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
Plus – 

sodium-glucose cotransporter-2 (SGLT2) inhibitor

Treatment recommended for ALL patients in selected patient group

SGLT2 inhibitors are recommended for adults who have been hospitalised with heart failure, once they are clinically stable.[81] SGLT2 inhibitors are recommended in adults with symptomatic chronic heart failure, regardless of the presence of diabetes, to reduce cardiovascular mortality and hospitalisation for heart failure.[57][82]​ Pre-clinical studies suggest an anti-inflammatory role for SGLT2 inhibitors in myocarditis.[83][84]​​

Primary options

dapagliflozin: adults: 10 mg orally once daily

OR

empagliflozin: adults: 10 mg orally once daily

Consider – treatment of underlying cause

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
Consider – 

treatment of underlying cause

Additional treatment recommended for SOME patients in selected patient group

Lymphocytic myocarditis generally does not require any therapy targeted at the underlying cause; management is supportive with treatment of the associated heart failure. For those with documented influenza, antiviral agents may be beneficial. In those with SARS-CoV-2 infection, similarly, antiviral agents as well as monoclonal antibodies may be used. For more information on the treatment of influenza infection and SARS-CoV-2 infection, see Influenza infection and Coronavirus disease 2019 (COVID-19).

Giant cell myocarditis is treated with a combination of systemic corticosteroids and another immunosuppressive agent such as cyclosporin, muromonab-CD3, or azathioprine. While giant cell myocarditis is often rapidly fatal, immunosuppressive therapy has been shown to improve mortality.[10][91][94]

Systemic corticosteroids improve outcomes in patients with autoimmune-associated myocarditis.[55]

Hypersensitivity myocarditis is treated by removal of the offending agent and systemic corticosteroids.

Corticosteroid use can cause marked hyperglycaemia and increases the risk of infection. Chronic use is associated with bone mineral density loss and skin friability.

Patients with chagasic myocarditis should be treated with an appropriate antiparasitic agent; see Chagas' disease for detailed information on management. A permanent pacemaker with defibrillator is often necessary because of conduction abnormalities and ventricular arrhythmias.

Immune checkpoint inhibitor (ICI)-related myocarditis is treated by immediately stopping ICI treatment and giving high-dose intravenous methylprednisolone. This can be switched to a weaning dose of oral prednisolone if patients show ongoing signs of clinical improvement. Second-line immunosuppressive therapy is indicated for corticosteroid-resistant patients or for haemodynamically unstable patients with fulminant myocarditis. Various second-line agents are under investigation and specialist guidance is recommended. After recovery, a multidisciplinary team should review whether to restart ICI therapy.[32]

Primary options

Autoimmune-associated or hypersensitivity myocarditis or ICI-related myocarditis

methylprednisolone: children and adults: consult specialist for guidance on dose

OR

Giant cell myocarditis

methylprednisolone: children and adults: consult specialist for guidance on dose

-- AND --

ciclosporin: children and adults: consult specialist for guidance on dose

More

or

muromonab-CD3: children and adults: consult specialist for guidance on dose

or

azathioprine: children and adults: consult specialist for guidance on dose

Consider – intravenous immunoglobulin (IVIG)

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

There is insufficient evidence to routinely recommend intravenous IVIG for all patients with myocarditis. However, it may provide clinical benefit in some cases, and is frequently used to treat children in clinical practice.[24][90]

Primary options

normal immunoglobulin human: children and adults: 2 g/kg intravenously as a single dose

Consider – beta-blocker

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
Consider – 

beta-blocker

Additional treatment recommended for SOME patients in selected patient group

Beta-blockers (e.g., carvedilol, metoprolol, bisoprolol) also attenuate maladaptive cardiac remodelling and improve survival in patients with cardiomyopathy.[57] Early treatment may decrease myocardial inflammation.[86] Carvedilol is often the preferred beta-blocker in children.

Beta-blockers may cause profound bradycardia, hypotension, or heart block. An ECG should be checked for pre-existing conduction abnormalities before commencing treatment. They may also exacerbate bronchial spasm in patients predisposed to reactive airway disease.

Dose should be started low and increased gradually according to response.

Primary options

carvedilol: children: consult specialist for guidance on dose; adults: 3.125 mg to 50 mg orally (immediate-release) twice daily

OR

metoprolol: adults: 12.5 to 200 mg orally (extended-release) once daily

OR

bisoprolol: adults: 1.25 to 10 mg orally once daily

Consider – vasodilator or inotrope

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
Consider – 

vasodilator or inotrope

Additional treatment recommended for SOME patients in selected patient group

Oral arterial vasodilators (e.g., hydralazine) and venous vasodilators (nitrates such as isosorbide dinitrate) acutely improve cardiac output and decrease pulmonary and left ventricular filling pressures.[77] This combination of therapy may also reduce morbidity and mortality in patients with heart failure who cannot tolerate ACE inhibitors, angiotensin-II receptor antagonists, or sacubitril/valsartan.[57] Hydralazine should be used in conjunction with a nitrate for the treatment of congestive heart failure. Hydralazine and nitrates are rarely used in paediatric care. If vasodilators are required in children, ACE inhibitors and related agents are often most appropriate. 

Milrinone (a parenteral positive inotrope and vasodilator) is often used early in the treatment course in children with evidence of left ventricular systolic dysfunction, even if they are haemodynamically stable. Milrinone may cause hypotension or, rarely, ventricular arrhythmias.

Dose should be started low and increased gradually according to response.

Primary options

hydralazine: adults: 25-100 mg orally three times daily

and

isosorbide dinitrate: adults: 20-40 mg orally three times daily

OR

milrinone: children: consult specialist for guidance on dose

Consider – diuretic therapy

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
Consider – 

diuretic therapy

Additional treatment recommended for SOME patients in selected patient group

Diuretics improve haemodynamics and patient comfort.

Dual therapy with a thiazide (or thiazide-like) diuretic (e.g., hydrochlorothiazide, chlorothiazide, metolazone) and a loop diuretic (e.g., furosemide) may be required in some patients for augmented therapeutic effect.[57] The thiazide diuretic should be administered at the same time as or 30 minutes prior to the loop diuretic.

Over-diuresis can cause acute renal failure. Volume status should be monitored. Diuretic therapy may also precipitate hypokalaemia or hypomagnesaemia. Serum electrolytes should be monitored. In patients who have a low cardiac index one must be careful not to administer diuretics to those with normal or low filling pressures.

Dose should be started low and increased gradually according to response.

Primary options

furosemide: children: consult specialist for guidance on dose; adults: 20-40 mg intravenously as a single dose initially, increase by 20 mg every 2 hours as needed according to clinical response; adults: 40 mg intravenously as a loading dose, followed by 10-40 mg/hour infusion

Secondary options

hydrochlorothiazide: children: consult specialist for guidance on dose; adults: 25-50 mg orally once or twice daily alone or 30 minutes prior to loop diuretic

OR

chlorothiazide: children: consult specialist for guidance on dose; adults: 250-1000 mg intravenously once or twice daily alone or 30 minutes prior to loop diuretic

OR

metolazone: children: consult specialist for guidance on dose; adults: 2.5 to 10 mg orally once daily alone or 30 minutes prior to loop diuretic

Consider – aldosterone antagonist

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
Consider – 

aldosterone antagonist

Additional treatment recommended for SOME patients in selected patient group

Aldosterone antagonists (e.g., spironolactone, eplerenone) improve survival in patients with New York Heart Association (NYHA) class II to IV heart failure.[57] Eplerenone is not recommended in children.

Aldosterone antagonists may cause hyperkalaemia. Serum potassium should be monitored. Aldosterone antagonists may also cause gynaecomastia in men.

Dose should be started low and increased gradually according to response.

Primary options

spironolactone: children: consult specialist for guidance on dose; adults: 12.5 to 50 mg/day orally given in 1-2 divided doses

OR

eplerenone: adults: 25-50 mg orally once daily

Consider – long-term anticoagulation therapy

Back
ACUTE
|
haemodynamically stable: evidence of LV systolic dysfunction
Consider – 

long-term anticoagulation therapy

Additional treatment recommended for SOME patients in selected patient group

Patients with left ventricular antero-apical akinesis or aneurysm as a result of cardiomyopathy are at increased risk for stroke or other arterial embolism secondary to left ventricular thrombus formation. Chronic anticoagulation therapy, such a warfarin, reduces this risk.[57]

Anticoagulation therapy increases the risk of significant bleed. INR should be monitored in all patients. Careful dose adjustment is necessary according to INR.

Warfarin can be safely used as a primary anticoagulant in children, including infants. Drug interactions, dosing, and appropriate INR goals should be based on consultation with a specialist.

Primary options

warfarin: children: consult specialist for guidance on dose; adults: 2-10 mg orally once daily initially, adjust dose according to target INR

More

haemodynamically unstable: adults

1st line – arterial vasodilator + invasive haemodynamic monitoring

Back
ACUTE
|
haemodynamically unstable: adults
1st line – 

arterial vasodilator + invasive haemodynamic monitoring

Sodium nitroprusside and other arterial vasodilators allow rapid titration of afterload reduction in patients with tenuous blood pressure secondary to cardiogenic shock. These medicines cause a rapid decrease in cardiac filling and pulmonary vasculature pressures and an increase in cardiac index.[77] Over-titration can result in hypotension, worsening end-organ hypoperfusion and ultimately in haemodynamic collapse.

The use of a pulmonary artery catheter or arterial catheter enables rapid titration of cardiovascular therapies and facilitates optimisation of cardiac filling pressures and cardiac output.[49] It carries the risk of procedural complications and should only be performed by experienced medical professionals.

Sodium nitroprusside may precipitate cyanide toxicity, especially in patients with renal dysfunction.

Primary options

nitroprusside: adults: 0.3 to 0.5 micrograms/kg/minute intravenously initially, titrate according to response, maximum 10 micrograms/kg/minute for 10 minutes

Consider – treatment of underlying cause

Back
ACUTE
|
haemodynamically unstable: adults
Consider – 

treatment of underlying cause

Additional treatment recommended for SOME patients in selected patient group

Lymphocytic myocarditis generally does not require any therapy targeted at the underlying cause; management is of the associated heart failure. For those with documented influenza, antiviral agents may be beneficial. In those with SARS-CoV-2 infection, similarly, antiviral agents as well as monoclonal antibodies may be used. For more information on the treatment of influenza infection and SARS-CoV-2 infection, see Influenza infection and Coronavirus disease 2019 (COVID-19).

Giant cell myocarditis is treated with a combination of systemic corticosteroids and another immunosuppressive agent such as cyclosporin, muromonab-CD3, or azathioprine. While giant cell myocarditis is often rapidly fatal, immunosuppressive therapy has been shown to improve mortality.[10][91][94]

Systemic corticosteroids improve outcomes in patients with autoimmune-associated myocarditis.[55]

Hypersensitivity myocarditis is treated by removal of the offending agent and systemic corticosteroids.

Corticosteroid use can cause marked hyperglycaemia and increases the risk of infection. Chronic use is associated with bone mineral density loss and skin friability.

Patients with chagasic myocarditis should be treated with an appropriate antiparasitic agent; see Chagas' disease for detailed information on management. A permanent pacemaker with defibrillator is often necessary because of conduction abnormalities and ventricular arrhythmias.

Immune checkpoint inhibitor (ICI)-related myocarditis is treated by immediately stopping ICI treatment and giving high-dose intravenous methylprednisolone. This can be switched to a weaning dose of oral prednisolone if patients show ongoing signs of clinical improvement. Second-line immunosuppressive therapy is indicated for corticosteroid-resistant patients or for haemodynamically unstable patients with fulminant myocarditis. Various second-line agents are under investigation and specialist guidance is recommended. After recovery, a multidisciplinary team should review whether to restart ICI therapy.[32]

Primary options

Autoimmune-associated or hypersensitivity myocarditis or ICI-related myocarditis

methylprednisolone: adults: consult specialist for guidance on dose

OR

Giant cell myocarditis

methylprednisolone: adults: consult specialist for guidance on dose

-- AND --

ciclosporin: adults: consult specialist for guidance on dose

More

or

muromonab-CD3: adults: consult specialist for guidance on dose

or

azathioprine: adults: consult specialist for guidance on dose

Consider – intravenous immunoglobulin (IVIG)

Back
ACUTE
|
haemodynamically unstable: adults
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

There is insufficient evidence to routinely recommend intravenous IVIG for all patients with myocarditis. However, it may provide clinical benefit in some cases.[24][90]

Primary options

normal immunoglobulin human: adults: 2 g/kg intravenously as a single dose

Consider – glyceryl trinitrate

Back
ACUTE
|
haemodynamically unstable: adults
Consider – 

glyceryl trinitrate

Additional treatment recommended for SOME patients in selected patient group

Intravenous glyceryl trinitrate is indicated in patients with elevated pulmonary vasculature and cardiac filling pressures, pulmonary oedema, and respiratory distress. Causes rapid decrease in pulmonary vasculature and left ventricular end-diastolic pressures.[77] This effect can rapidly improve respiratory distress secondary acute pulmonary oedema, often preventing the need for mechanical ventilation.

May cause severe hypotension.

When used in conjunction with phosphodiesterase-5 inhibitors (e.g., sildenafil) may cause precipitous drop in blood pressure and cardiovascular collapse.

Primary options

glyceryl trinitrate: adults: 5 micrograms/minute intravenously initially, increase by 5 micrograms/minute increments every 3-5 minutes according to response up to 20 micrograms/minute, if no response increase by 10-20 micrograms/minute increments every 3-5 minutes according to response, maximum 100 micrograms/minute

Consider – inotrope or vasopressor

Back
ACUTE
|
haemodynamically unstable: adults
Consider – 

inotrope or vasopressor

Additional treatment recommended for SOME patients in selected patient group

Intravenous inotropes (e.g., dobutamine, milrinone) can be considered in patients with associated non-ischaemic cardiomyopathy in whom end-organ hypoperfusion is secondary to cardiogenic shock. In this situation, inotropes increase cardiac index resulting in increased end-organ perfusion. One randomised controlled trial found no significant difference between dobutamine and milrinone in terms of outcomes including in-hospital death, resuscitated cardiac arrest, and non-fatal myocardial infarction.[96] Dobutamine and milrinone increase the risk of ventricular arrhythmias. Also, both agents can cause hypotension via their vascular effect and thus worsen end-organ perfusion. Long-term use of inotropes is associated with increased mortality.[57]

Vasopressors (e.g., noradrenaline, phenylephrine) can be used to elevate blood pressure in hypotensive patients with systolic dysfunction. Caution is required, as hypotension in the setting of cardiomyopathy is frequently caused by low cardiac output, which will be worsened with the use of vasopressors. Invasive haemodynamic monitoring with a Swan-Ganz catheter can be used to discern the aetiology of hypotension and inform the decision to use these agents. Use of vasopressors in the setting of hypovolaemia is associated with limb and mesenteric ischaemia, organ hypoperfusion, and increased mortality. The volume status of the patient must therefore be known to be normal before the use of vasopressors can be considered. Phenylephrine is the preferred agent in the setting of significant hypotension.

Primary options

dobutamine: adults: consult specialist for guidance on dose

OR

milrinone: adults: consult specialist for guidance on dose

Secondary options

noradrenaline (norepinephrine): adults: consult specialist for guidance on dose

OR

phenylephrine injection: adults: consult specialist for guidance on dose

Plus – mechanical circulatory support

Back
ACUTE
|
haemodynamically unstable: adults
|
refractory cardiogenic shock
Plus – 

mechanical circulatory support

Treatment recommended for ALL patients in selected patient group

Mechanical circulatory support with an intra-aortic balloon pump (IABP) or left ventricular assist device (LVAD [e.g., Impella®]) may be indicated in patients with refractory cardiogenic shock despite optimal aggressive medical therapy. Both devices may be used as a bridge to recovery or as a bridge to heart transplantation if no response to treatment.[57]

IABP markedly increases the risk of infection. The patient should be frequently re-evaluated to determine if IABP is still a necessity and remove it as soon as deemed safe.

IABP also increases the risk of limb ischaemia. The lower extremities must be regularly examined and the IABP must be removed at the first sign of arterial compromise.

In addition, IABP increases the risk of arterial thrombus. Heparin should be used in conjunction with this therapy if not contraindicated.

Patients should continue on their existing heart failure medicines along with the IABP.

LAVDs are associated with moderate risk for perioperative complication or mortality. They also impart a high risk of serious bleeding complication.[97]

Driveline infections are the most common LVAD-associated infection and require antibiotic therapy.[98]

LVADs also carry a high risk of arterial thromboembolic disease. Most require long-term anticoagulation.[97]

Continuation of heart failure medications with LVAD is determined on a case-by-case basis.

haemodynamically unstable: children

1st line – inotrope

Back
ACUTE
|
haemodynamically unstable: children
1st line – 

inotrope

Inotropic support may be provided with commonly used agents such as milrinone or dobutamine. These agents are considered inodilators and provide inotropy with vasodilatation.[15]

Primary options

dobutamine: children: consult specialist for guidance on dose

OR

milrinone: children: consult specialist for guidance on dose

Consider – mechanical circulatory support

Back
ACUTE
|
haemodynamically unstable: children
Consider – 

mechanical circulatory support

Additional treatment recommended for SOME patients in selected patient group

For children with acute or fulminant myocarditis who are demonstrating haemodynamic instability, consideration may be given to early mechanical circulatory support, as medical therapy during this phase is often not adequate. This is especially true if the patient is already demonstrating arrhythmias.

Depending on the age and size of the child, and centre preference, such circulatory support may be provided by extracorporeal membrane oxygenation (ECMO) or by a temporary left ventricular assist device such as with TandemHeart® or Impella®.

ECMO is recommended for children with cardiogenic shock, cardiorespiratory compromise, or significant arrhythmias.[15] Venoarterial ECMO (VA-ECMO), when used for supporting paediatric myocarditis patients, has a survival to hospital discharge of 76%.[99] This is the best survival of any indication for paediatric VA-ECMO.[99]

A durable left ventricular assist device can be used as a bridge to recovery or heart transplantation if ECMO cannot be weaned.[15]

Consider – treatment of underlying cause

Back
ACUTE
|
haemodynamically unstable: children
Consider – 

treatment of underlying cause

Additional treatment recommended for SOME patients in selected patient group

Lymphocytic myocarditis generally does not require any therapy targeted at the underlying cause; management is of the associated heart failure. For those with documented influenza, antiviral agents may be beneficial. In those with SARS-CoV-2 infection, similarly, antiviral agents as well as monoclonal antibodies may be used. For more information on the treatment of influenza infection and SARS-CoV-2 infection, see Influenza infection and Coronavirus disease 2019 (COVID-19).

Giant cell myocarditis is treated with a combination of systemic corticosteroids and another immunosuppressive agent such as cyclosporin, muromonab-CD3, or azathioprine. While giant cell myocarditis is often rapidly fatal, immunosuppressive therapy has been shown to improve mortality.[10][91][94]

Systemic corticosteroids improve outcomes in patients with autoimmune-associated myocarditis.[55]

Hypersensitivity myocarditis is treated by removal of the offending agent and systemic corticosteroids.

Corticosteroid use can cause marked hyperglycaemia and increases the risk of infection. Chronic use is associated with bone mineral density loss and skin friability.

Patients with chagasic myocarditis should be treated with an appropriate antiparasitic agent; see Chagas' disease for detailed information on management. A permanent pacemaker with defibrillator is often necessary because of conduction abnormalities and ventricular arrhythmias.

Immune checkpoint inhibitor (ICI)-related myocarditis is treated by immediately stopping ICI treatment and giving high-dose intravenous methylprednisolone. This can be switched to a weaning dose of oral prednisolone if patients show ongoing signs of clinical improvement. Second-line immunosuppressive therapy is indicated for corticosteroid-resistant patients or for haemodynamically unstable patients with fulminant myocarditis. Various second-line agents are under investigation and specialist guidance is recommended. After recovery, a multidisciplinary team should review whether to restart ICI therapy.[32]

Primary options

Autoimmune-associated or hypersensitivity myocarditis or ICI-related myocarditis

methylprednisolone: children: consult specialist for guidance on dose

OR

Giant cell myocarditis

methylprednisolone: children: consult specialist for guidance on dose

-- AND --

ciclosporin: children: consult specialist for guidance on dose

or

muromonab-CD3: children: consult specialist for guidance on dose

or

azathioprine: children: consult specialist for guidance on dose

Consider – intravenous immunoglobulin (IVIG)

Back
ACUTE
|
haemodynamically unstable: children
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

There is insufficient evidence to routinely recommend intravenous IVIG for all patients with myocarditis. However, it may provide clinical benefit in some cases, and is frequently used to treat children in clinical practice.[24][90]

Primary options

normal immunoglobulin human: children: 2 g/kg intravenously as a single dose

ONGOING

end-stage heart failure or refractory life-threatening arrhythmias

1st line – heart transplantation

Back
ONGOING
|
end-stage heart failure or refractory life-threatening arrhythmias
1st line – 

heart transplantation

Heart transplantation markedly improves survival, New York Heart Association (NYHA) functional class, and therefore quality of life in patients with end-stage heart failure or refractory life-threatening arrhythmias.[57] Post-transplant, patients are treated with appropriate immunosuppression.

2nd line – destination left ventricular assist device therapy + continue pharmacological treatment

Back
ONGOING
|
end-stage heart failure or refractory life-threatening arrhythmias
2nd line – 

destination left ventricular assist device therapy + continue pharmacological treatment

Patients who have chronic refractory New York Heart Association (NYHA) stage IV heart failure and who are not candidates for heart transplantation because of age or co-morbid conditions have improved survival, as compared with medical management, with long-term destination left ventricular assist device (LVAD) therapy.[100] Due to improved outcomes with LVAD, patients are also able to choose LVAD therapy instead of heart transplantation. 

In general, these patients are kept on their existing medication, which are adjusted as haemodynamics allow. The addition of systemic anticoagulation and use of antiplatelet therapy is required.

back arrow

Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

Use of this content is subject to our disclaimer