Heart Failure Guidelines

Please note that the entire Guidelines section outlines select guidelines and recommendations. The reader is invited to consult the original guidelines for more detailed information.

Heart Failure Criteria, Classification, and Staging

Guideline contributor: Henry H Ooi, MD, MRCPI, Director, Advanced Heart Failure and Cardiac Transplant Program, Nashville Veterans Affairs Medical Center; Assistant Professor of Medicine, Vanderbilt University School of Medicine.

Heart failure criteria, classification, and staging

Framingham classification

In the Framingham classification, the diagnosis of heart failure is based on the concurrent presence of either two major criteria or one major and two minor criteria. ^[1]

Major criteria comprise the following:

• Paroxysmal nocturnal dyspnea
• Weight loss of 4.5 kg or more in 5 days in response to treatment
• Neck vein distention
• Rales
• Acute pulmonary edema
• Hepatojugular reflux
• S ₃ gallop
• Central venous pressure greater than 16 cm water
• Circulation time of 25 seconds or longer
• Radiographic cardiomegaly
• Pulmonary edema, visceral congestion, or cardiomegaly at autopsy

Minor criteria (accepted only if they cannot be attributed to another medical condition) are as follows:

• Nocturnal cough
• Dyspnea on ordinary exertion
• A decrease in vital capacity by one third the maximal value recorded
• Pleural effusion
• Tachycardia (rate of ≥120 bpm)
• Hepatomegaly
• Bilateral ankle edema

New York Heart Association (NYHA) classification

The NYHA functional classification of heart failure is widely used in practice and in clinical studies. It is based on symptom severity and the amount of exertion needed to provoke symptoms. NYHA heart failure classes are as follows ^[2] :

• Class I: No limitation of physical activity
• Class II: Slight limitation of physical activity, in which ordinary physical activity leads to fatigue, palpitation, or dyspnea; the person is comfortable at rest
• Class III: Marked limitation of physical activity, in which less-than-ordinary activity results in fatigue, palpitation, or dyspnea; the person is comfortable at rest
• Class IV: Inability to carry on any physical activity without discomfort but also symptoms of heart failure at rest, with increased discomfort if any physical activity is undertaken

American College of Cardiology Foundation/American Heart Association (ACCF/AHA) staging systems

The 2013 ACCF/AHA staging system complements the NYHA classification to reflect the progression of disease and comprises four stages, as shown in Table 3. below. ^[3]

Table 3. 2013 American College of Cardiology Foundation/American Heart Association (ACCF/AHA) Heart Failure Staging System (Open Table in a new window)

2022 ACC/AHA/Heart Failure Society of America classification

The updated ACC/AHA/HFSA disease-staging terminology characterizes the syndrome as a continuum. ^{[4, 5, 6, 7]}

Table 4. 2022 ACC/AHA/Heart Failure Society of America (HFSA) Heart Failure Staging System (Open Table in a new window)

Table 5. 2022 ACC/AHA/HFSA Classification of Heart Failure (HF) by Left Ventricular Ejection Fraction (LVEF) (Open Table in a new window)

2022 American College of Cardiology/American Heart Association/Heart Failure Society of America (ACC/AHA/HFSA) recommendations for genetic evaluation and testing

Genetic screening and counseling are recommended for first-degree relatives of selected individuals with genetic or inherited cardiomyopathies to detect cardiac disease and to encourage review of therapies for lowering HF progression and sudden death. ^[4]

It is reasonable to refer select patients with nonischemic cardiomyopathy for genetic counseling and testing to identify conditions that could guide treatments for patients and family member. ^[4]

2021 European Society of Cardiology guidelines for genetic counseling and testing

Arrhythmogenic cardiomyopathy (ACM)

Offer genetic counseling and testing all patients with suspected ACM and all first-degree adult relatives of patients with ACM and a disease-causing mutation, regardless of their phenotype to preclinically identify genetically affected individuals. ^[68]

Genetic family screening may also be used for arrhythmic risk stratification. ^[68]  

First-degree relatives with the same definite disease-causing mutation as the patient should undergo clinical evaluation, electrocardiography (ECG), echocardiography, and possibly cardiac magnetic resonance imaging (CMRI). ^[68]  In the setting of no definite identified genetic mutation in the patient or no genetic testing is undertaken, consider clinical evaluation in first-degree adult relatives with ECG and echocardiography, and repeat every 2-5 years or less if nondiagnostic abnormalities are present.

Dilated cardiomyopathy (DCM) or hypokinetic nondilated cardiomyopathy (HNDC)

All patients with suspected DCM or HNDC and all first-degree adult relatives of such patients and a definite disease-causing mutation, regardless of their phenotype, should undergo genetic counseling and testing to preclinically identify genetically affected individuals. ^[68]  Repeat the evaluation every 5 years or less in first-degree adult relatives when aged younger than 50 years or in the presence of nondiagnostic abnormalities.

All first-degree relatives of patients should undergo clinical evaluation, ECG, echocardiography, and possibly CMRI. ^[68]

Findings from clinical evaluation, genetic testing, and imaging studies noted above can identify patients with DCM or HNDC who have the greatest risk of arrhythmia and/or deserve other specific treatments. ^[68] Early identification of asymptomatic relatives may allow early treatment and prevent progression to HF,  as well as appropriate genetic counseling.

Hypertrophic cardiomyopathy (HCM)

Offer genetic counseling and testing all patients with suspected HCM and all first-degree adult relatives of patients with HCM and a disease-causing mutation, regardless of their phenotype to preclinically identify genetically affected individuals. ^[68]

First-degree relatives with the same definite disease-causing mutation as the patient should undergo clinical evaluation with ECG and echocardiography. ^[68]  In the setting of no definite identified genetic mutation in the patient or no genetic testing is undertaken, consider clinical evaluation in first-degree adult relatives with ECG and echocardiography, and repeat every 2-5 years or less if nondiagnostic abnormalities are present.

2013 ACC Foundation/AHA guidelines for screening and genetic testing for DCM

Familial DCM (DCM with two close relatives who meet the criteria for idiopathic DCM) ^[3]

• First-degree relatives not known to be affected should undergo periodic, serial echocardiographic screening with assessment of LV function and size.
• Although the screening frequency is uncertain, every 3-5 years is reasonable.
• Consider genetic testing in conjunction with genetic counseling.

Idiopathic DCM  ^[3]

• Inform first-degree relatives of index diagnosis.
• Relatives should discuss with their clinicians whether they should undergo echocardiographic screening.
• Although the value of genetic testing is unclear in this setting, it is potentially valuable in patients with significant cardiac conduction disease and/or a family history of premature sudden cardiac death.

2011 Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA) select recommendations for genetic testing for channelopathies and cardiomyopathies

Long QT syndrome (LQTS) ^[263]

Comprehensive or LQT1-3 (KCNQ1, KCNH2, and SCN5A)–targeted LQTS genetic testing is recommended for the following:

• Individuals with a strong clinical index of suspicion for LQTS based on the patient's clinical history, family history, and expressed electrocardiographic (ECG) (resting 12-lead ECGs and/or provocative stress testing with exercise or catecholamine infusion) phenotype
• Asymptomatic individuals with idiopathic QT prolongation on serial 12-lead ECGs defined as QTc over 480 ms (prepuberty) or longer than 500 ms (adults); may also be considered in asymptomatic individuals with idiopathic QT prolongation on serial 12-lead ECGs for QTc values over 460 ms (prepuberty) or longer than 480 ms (adults)

Mutation-specific genetic testing is recommended for family members following identification of the LQTS mutation in an index case.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) ^[263]

• Comprehensive or CPVT1 and CVPT2 ( RYR2 and CASQ2)–targeted CPVT genetic testing is recommended for any individual with a clinical index of suspicion for CPVT based on the patient's clinical history, family history, and expressed ECG phenotype during provocative stress testing with cycle, treadmill, or catecholamine infusion.
• Mutation-specific genetic testing is recommended for family members following identification of the CPVT mutation in an index case.

Brugada syndrome (BrS) ^[263]

• Consider comprehensive or BrS1 ( SCN5A)–targeted BrS genetic testing for individuals with a clinical index of suspicion for BrS based on the patient's clinical history, family history, and expressed ECG (resting 12-lead ECGs and/or provocative drug challenge testing) phenotype.
• Mutation-specific genetic testing is recommended for family members following identification of the BrS mutation in an index case.
• Genetic testing is not indicated in individuals with an isolated type 2 or type 3 Brugada ECG pattern.

Cardiac conduction disease (CCD) ^[263]

• Consider genetic testing as part of the diagnostic evaluation for individuals with either isolated CCD or CCD with concomitant congenital heart disease, particularly in cases of a documented positive family history of CCD.
• Mutation-specific genetic testing is recommended for family members following identification of the CCD mutation in an index case. 

Short QT syndrome (SQTS) ^[263]

• Consider comprehensive or SQT1-3 ( KCNH2, KCNQ1, and KCNJ2)–targeted SQTS genetic testing for individuals with a clinical index of suspicion for SQTS based on the patient's clinical history, family history, and ECG phenotype.
• Mutation-specific genetic testing is recommended for family members following identification of the SQTS mutation in an index case.

HCM  ^[263]

• Comprehensive or targeted ( MYBPC3, MYH7, TNNI3, TNNT2, TPM1) HCM genetic testing is recommended for individuals with a clinical diagnosis of HCM based on the patient's clinical history, family history, and ECG/echocardiographic phenotype.
• Mutation-specific genetic testing is recommended for family members following identification of the HCM mutation in an index case.

​ACM / arrhythmogenic right ventricular cardiomyopathy (ARVC) ^[263]

• Comprehensive or targeted ( DSC2, DSG2, DSP, JUP, PKP2, and  TMEM43) ACM/ARVC genetic testing can be useful for individuals who fulfill the task force diagnostic criteria for ACM/ARVC.
• Consider genetic testing for patients with possible ACM/ARVC (1 major or 2 minor criteria) based on the 2010 task force criteria. ^[264]
• Mutation-specific genetic testing is recommended for family members following identification of the ACM/ARVC mutation in an index case.
• Genetic testing is not recommended for patients with only a single minor criterion according to the 2010 task force criteria. ^[264]  

DCM  ^[263]

• Comprehensive or targeted ( LMNA and  SCN5A) DCM genetic testing is recommended for individuals with DCM and significant cardiac conduction disease (ie, first-, second-, or third-degree heart block) and/or a family history of premature unexpected sudden death.
• Mutation-specific genetic testing is recommended for family members following identification of the DCM mutation in an index case.
• Genetic testing can be useful for individuals with familial DCM to confirm the diagnosis, identify those at highest risk of arrhythmia/syndromic features, facilitate cascade screening among family members, and aid in family planning.

Left ventricular noncompaction (LVNC) ^[263]

• Genetic testing may be useful for individuals with a clinical diagnosis of LVNC based on the patient's clinical history, family history, and ECG/echocardiographic phenotype
• Mutation-specific genetic testing is recommended for family members following identification of the LVNC mutation in an index case.

Restrictive cardiomyopathy (RCM) ^[263]

• Consider genetic testing for individuals with a suspected clinical diagnosis of RCM based on the patient's clinical history, family history, and ECG/echocardiographic phenotype.
• Mutation-specific genetic testing is recommended for family members following identification of the RCM mutation in an index case.

2010 Heart Failure Society of America (HFSA) recommendations for genetic evaluation of cardiomyopathy

Note the following ^[9] :

• For all patients with cardiomyopathy, obtain a detailed family history for at least 3 generations (HCM, DCM, arrhythmic right ventricular dysplasia [ARVD], LVNC, RCM, and cardiomyopathies associated with extracardiac manifestations)
• Carefully assess the patient's medical history as well as that of asymptomatic first-degree relatives, with special focus on heart failure symptoms, arrhythmias, presyncope, and syncope.
• Screen asymptomatic first-degree relatives for cardiomyopathy (HCM, DCM, ARVD, LVNC, RCM, and cardiomyopathies associated with extracardiac manifestations)
• Screen for cardiomyopathy at intervals in asymptomatic at-risk relatives who are known to carry the disease-causing mutation(s) (For details, see Recommendations 17.2e and 17.2f in  HFSA Guideline Approach to Medical Evidence for Genetic Evaluation of Cardiomyopathy ^[9] )
• Screen for cardiomyopathy in asymptomatic at-risk first-degree relatives who have not undergone genetic testing or in whom a disease-causing mutation has not been identified.

Note: Due to the complexity of genetic evaluation, testing, and counseling of patients with cardiomyopathy, it is recommended that patients be referred to centers with expertise in these matters and in family-based management. ^[9]

Guidelines for the diagnosis and management of heart failure have been issued by the following organizations:

• American College of Cardiology Foundation/American Heart Association (ACCF/AHA) ^[3]
• American College of Cardiology (ACC)/AHA/Heart Failure Society of America (HFSA) ^{[4, 5]}
• HFSA ^[9]
• European Society of Caridiology (ESC) ^{[8, 68]}

The ACC/AHA/HFSA guidelines, ^{[3, 4, 5, 61]}  HFSA guidelines, ^[9] ​ and ESC guidelines ^{[8, 68]}  all recommend the following basic laboratory tests and studies in the initial evaluation of patients with suspected heart failure:

• Complete blood count (CBC), which may indicate anemia or infection as potential causes of heart failure
• Urinalysis (UA), which may reveal proteinuria, which is associated with cardiovascular disease
• Serum electrolyte levels, which may be abnormal owing to causes such as fluid retention or renal dysfunction
• Blood urea nitrogen (BUN) and serum creatinine levels, which may indicate decreased renal blood flow
• Fasting blood glucose levels, because elevated levels indicate a significantly increased risk for heart failure (diabetic and nondiabetic patients
• Liver function tests (LFTs), which may show elevated liver enzyme levels and indicate liver dysfunction due to heart failure
• Electrocardiography (ECG) (12-lead), which may reveal arrhythmias, ischemia/infarction, and coronary artery disease (CAD) as possible causes of heart failure

In addition, these guidelines recommend measuring B-type natriuretic peptide (BNP) and N-terminal pro-B-type (NT-proBNP) natriuretic peptide levels, which are increased in heart failure and useful for risk stratification. ^{[3, 4, 5, 8, 9, 61]}  Baseline measurements correlate closely with the New York Heart Association (NYHA) heart failure functional classification and can be useful for prognosis in acutely decompensated patients. ^[61] The ACCF/AHA, HFSA, and ESC also indicate obtaining BNP or NT-proBNP levels in the workup of heart failure particularly when the diagnosis is unclear. ^{[3, 8, 9]} The HFSA recommends this test in all cases of suspected heart failure, particularly in ambiguous cases. ^[9]

The ACC/AHA recommendations also include obtaining a lipid profile and thyroid stimulating hormone (TSH) level. ^{[3, 4, 5]}  These tests reveal potential cardiovascular or thyroid disease as causes of heart failure. If the clinical presentation also suggests an acute coronary syndrome, the ESC recommends obtaining levels of troponin I or T ^{[8, 68]} ; increased troponin levels indicate injury to the myocytes and the severity of heart failure.

The ACCF/AHA, ACC/AHA, HFSA, and ESC also recommend the following imaging studies and procedures ^{[3, 4, 5, 8, 9, 68]} :

• Chest radiography (posterior-anterior, lateral), which may show pulmonary congestion, an enlarged cardiac silhouette, or other potential causes of the patient's symptoms
• Two-dimesional echocardiographic and Doppler flow ultrasonographic studies, which may reveal ventricular dysfunction and/or valvular abnormalities
• In the setting of inadequate echocardiography, alternative imaging (cardiac magnetic resonance imaing [CMRI], cardiac computed tomography [CCT], radionuclide imaging), to assess left ventricular (LV) ejection fraction (EF) ^{[4, 5]}
• Coronary arteriography in patients with a history of exertional angina or suspected ischemic LV dysfunction, which may reveal CAD
• Maximal exercise testing with/without respiratory gas exchange and/or blood oxygen saturation, which assesses cardiac and pulmonary function with activity, the inability to walk more than short distances, and a decreased peak oxygen consumption reflect more severe disease
• Noninvasive stress imaging (stress echocardiography, single-photon emission CT [SPECT], CMRI, or positron emission tomography [PET]), for detecting myocardial ischemia in those with HF and CAD who are candidates for coronary revascularization

Other studies may be indicated in selected patients, ^[3]  such as the following:

• Screening for hemochromatosis, in which iron overload affects cardiac function
• Screening for sleep-disturbed breathing, which affects neurohormonal activation
• Screening for human immunodeficiency virus (HIV), which may result in heart failure from possible direct infectious effects, from disease treatment effects causing CAD, or from other causes
• Testing for rheumatologic diseases, amyloidosis, or pheochromocytoma, all of which may cause cardiomyopathy
• Serum and urine electrophoresis for light-chain disease
• Genetic screening, testing, and/or counseling for at-risk patients with a first-degree relative who has been diagnosed with a cardiomyopathy leading to heart failure, which may aid in detecting early disease onset and guide treatment ^{[4, 5, 34]}
• Holter monitoring, which may reveal arrhythmias or abnormal electrical activity (eg, in patients with heart failure and a history of myocardial infarction (MI) who are being considered for electrophysiologic study to document ventricular tachycardia [VT] inducibility) ^{[8, 9]}

Routine use of invasive hemodynamic monitoring is not recommended in HF. ^{[4, 5]} However, invasive hemodynamic monitoring may help to guide management in selected patients with HF who have persisitent or worsening symptoms, signs, diagnostic parameters, and in whom hemodynamics are uncertain. ^{[4, 5]}

The 2022 ACC/AHA/HFSA and the 2020 Canadian Cardiovascular Society (CCS) and Canadian Heart Failure Society (CHFS) recommend that when cardiac amyloidosis (CA) is suspected, rule out light-chain amyloidosis (AL amyloidosis) using serum free light chains (kappa and lambda), and serum and urine protein electrophoresis with immunofixation. ^{[4, 5, 265]} Accurate identification of the amyloid subtype is essential to initiate specific treatment and avoid inappropriate application of therapy.

When the suspicion is high for cardiac amyloidosis but without evidence of serum/urine monoclonal light chains, perform bone scintigraphy to confirm the presence of transthyretin cardiac amyloidosis (ATTR-CA). ^{[4, 5]} For a positive diagnosis of ATTR-CA, perform genetic testing with TTR gene sequencing to differentiate the hereditary variant from wild-type ATTR-CA.

The following are practical tips regarding CA by the CCS/CHFS ^[265] :

• In the setting of undifferentiated CA, the presence of light chains does not confirm the diagnosis of light chain cardiac amyloidosis (AL-CA) because monoclonal gammopathy of unknown significance and ATTR-CA can coexist. In such settings, tissue biopsy is often necessary to exclude AL-CA.
• Perform technetium-labeled scintigraphy, where available, to diagnose ATTR-CA when plasma cell dyscrasias have been ruled out.
• Patient selection for tafamidis, a transthyretin tetramer stabilizer, should reflect the inclusion criteria for the Transthyretin Amyloidosis Cardiomyopathy Trial (ATTR-ACT) that showed clinical benefits of tafamidis over placebo with respect to mortality and cardiovascular hospitalization, including established ATTR-CA and objective evidence of HF (with elevated natriuretic peptides, where available).
• Do not routinely consider treatment with tafamidis for patients with New York Heart Association (NYHA) class IV symptoms or severe functional disability, measured using a 6-minute walk test < 100 m. (These patient were excluded from ATTR-ACT.) Subgroup analysis from the ATTR-ACT trial suggested that the reduction in cardiovascular hospitalizations seen with tafamidis might be limited to patients with less severe symptoms (NYHA class I or II).
• Because of the complexity in diagnosing CA and the potential for offering advanced or experimental treatment options, consider referring patients with CA to experienced centers. Other agents are currently under investigation, which might modify current treatment recommendations.

Catheterization and angiography

According to the ACCF/AHA, HFSA, and ESC cardiac catheterization and coronary angiography should be considered for patients with heart failure in the following situations ^{[3, 8, 9]} :

• When symptoms worsen without a clear cause in patients with heart failure, no angina, and known CAD
• In heart failure caused by systolic dysfunction in association with angina or regional wall-motion abnormalities and/or scintigraphic evidence of reversible myocardial ischemia when revascularization is being considered
• When the pretest probability of the underlying ischemic cardiomyopathy is high and surgical coronary procedures are being considered
• Before cardiac transplantation or LV assist device placement
• In cases of heart failure secondary to postinfarction ventricular aneurysm or other mechanical complications of MI

Endomyocardial biopsy

According to the 2013 ACCF/AHA and 2022 ACC/AHA/HFSA guidelines, routine endomyocardial biopsy (EMB) is not recommended in all cases of HF given the risk of complications. However, it may be considered in the following situations ^{[3, 4, 5]} :

• In rapidly progressive heart failure or worsening ventricular dysfunction that persists despite appropriate medical therapy
• In suspected cases of acute cardiac rejection status after heart transplantation or myocardial infiltrative processes
• In rapidly progressive and unexplained cardiomyopathy for which active myocarditis, especially giant cell myocarditis, is being considered
• When a specific diagnosis is suspected and EMB would influence therapy

The ESC guidelines recommend considering EMB in rapidly progressive HF despite appropriate medical therapy when there is a probability of a specific diagnosis that can be confirmed only in mycardial samples and there is an effective specfic therapy available. ^[8]  EMB is also indicated in patients with suspected phenotypes that require specific treatments, such as giant cell myocarditis, eosinophilic myocarditis, sarcoidosis, vasculitis, systemic lupus erythematosus, other systemic autoimmune inflammatory conditions, or storage diseases. ^[68]

The HFSA suggests that EMB be considered in patients with rapidly progressive clinical heart failure or ventricular dysfunction, despite appropriate medical therapy, as well as in patients suspected of having myocardial infiltrative processes (eg, sarcoidosis, amyloidosis) or in patients with malignant arrhythmias out of proportion to their LV dysfunction (eg, sarcoidosis, giant cell myocarditis). ^[9]

Assessment of functional capacity

The ACCF/AHA indicates the 6-minute walk test may be indicated in patients with heart failure whose adequacy of rate control is in question ^[3] ; the HFSA indicates it is a good indicator of functional status and prognosis in patients with heart failure. ^[9]

The ACCF/AHA and HFSA do not recommend routine maximal exercise stress testing. ^{[3, 9]}  HFSA guidelines indicate it may be useful in situations such as the following with measurement of gas exchange ^[9] :

• To assess the disparity between symptomatic limitation and objective indicators of disease severity
• To distinguish non HF-related causes of functional limitation, specifically cardiac versus pulmonary
• To consider whether patients are candidates for cardiac transplantation or mechanical circulatory support
• To determine the prescription for cardiac rehabilitation

ACCF/AHA and ESC guidelines note that values of peak oxygen consumption of less than 50% of predicted or less than 14 mL/kg/min reflect poor cardiac performance and a likelihood of 1-year survival less than 50%, facilitating referral for cardiac transplantation or mechanical circulatory device placement. ^{[3, 8]}

By definition, stage A patients are at high risk for heart failure but do not have structural heart disease or symptoms of heart failure. For these individuals, guidelines from the American College of Cardiology Foundation/American Heart Association (ACCF/AHA), Heart Failure Society of America (HFSA), ACC/AHA/HFSA, and the European Society of Cardiology recommend nonpharmacologic management focused on prevention through reduction of risk factors. Measures include the following ^{[3, 4, 8, 9, 68]} :

• Treat hypertension and lipid disorders
• Encourage smoking cessation
• Discourage heavy alcohol intake and illicit drug use
• Control and/or prevent diabetes mellitus
• Encourage physical activity
• Encourage weight reduction if obese or overweight

For patients with chronic heart failure, the ACCF/AHA, HFSA, and ESC recommend regular aerobic exercise to improve functional capacity and symptoms. ^{[3, 8, 9]}  However, ACCF/AHA cautions that limitation of activity is appropriate during acute heart failure exacerbations and in patients with suspected myocarditis. Most patients should not participate in heavy labor or exhaustive sports. ^[3]

The ACCF/AHA and ESC recommend specific patient education to facilitate self-care and close observation and follow-up are important aspects of care. Close supervision, including surveillance by the patient and family, home-based visits, telephone support, or remote monitoring should be provided to improve adherence. ^{[3, 9]}

Dietary sodium should be restricted to 2-3 g/day according the ACCF/AHA and HFSA, ^{[3, 9]}  although the ACCF/AHA notes that evidence to support this recommendation is inconclusive. ^[3]

Fluid restriction to 2 L/day is recommended for patients with evidence of hyponatremia (Na <  130 mEq/dL) and for those whose fluid status is difficult to control despite sodium restriction and the use of high-dose diuretics. ^{[3, 8, 9]}

The ACCF/AHA, HFSA, and ESC guidelines recommend caloric supplementation for patients with evidence of cardiac cachexia. ^{[3, 8, 9]}  The HFSA recommends against the use of anabolic steroids for these patients. ^[9]

The HFSA recommends against naturoceutical use for relief of symptomatic heart failure or for the secondary prevention of cardiovascular events. ^[9] Avoid natural or synthetic products containing ephedra (ma huang), ephedrine, or its metabolites, as well as products that have significant drug interactions with digoxin, vasodilators, beta blockers, antiarrhythmic drugs, and anticoagulants. ^[9]

The 2022 ACCF/AHA/HFSA recommendations for nonpharmacologic management of HF focuses on self-care support ^[4] :

• Multidisciplinary teams should provide care to those with HF, to facilitate guideline-directed medical therapy, address potential barriers to self-care, lower the risk of rehospitalization for HF, and improve survival.
• Provide specific education and support to those with HF to facilitate multidisciplinary HF self-care.
• To reduce mortality, it is reasonable to vaccinate patients with HF against respiratory illnesses.
• It is reasonable to screen adults with HF for risk factors for poor self-care (eg, depression, social isolation, frailty, low health literacy) to improve management.

2022 ACC/AHA/HFSA Recommendations

The American College of Cardiology, American Heart Association, and Heart Failure Society of America (ACC/AHA/HFSA) replaced their 2013 and 2017 guidelines on the management of heart failure (HF) with significant and paradigm-shifting additional treatment options that include new/repurposed drug therapies that benefit almost without regard to ejection fraction (EF). ^{[4, 5]}

• Four core foundational medication classes (sodium-glucose cotransporter-2 inhibitors [SGLT2Is], beta blockers, mineralocorticoid receptor antagonists [MRAs], and renin-angiotensin system [RAF] inhibitors) are now included in guideline-directed medical therapy (GDMT) for HF with reduced EF (HFrEF). Angiotensin receptor-neprilysin inhibitors (ARNIs), angiotensin-converting enzyme inhibitors (ACEIs), or angiotensin receptor blockers (ARBs) are recommended as first-line agents in HFrEF.
• SGLT2Is are a class 2a (moderate) recommendation in HF with mildly reduced ejection fraction (HFmrEF), whereas ARNIs, ACEIs, ARBs, MRAs, and beta blockers are class 2b (weak) recommendations for this patient population.
• There are new recommendations for HF with preserved EF (HFpEF) for SGLT2Is (class 2a), MRAs (class 2b), and ARNIs (class 2b). Renewed recommendations include those for treatment of hypertension (class 1 [strong]) and of atrial fibrillation (AF) (class 2a); use of ARBs (class 2b); as well as avoidance of the routine use of nitrates or phosphodiesterase-5 (PDE5) inhibitors (class 3 [no benefit]).

At risk for HF (stage A) (class I recommendations)

In hypertensive patients at risk for HF, control blood pressure with GDMT for hypertension to prevent symptomatic HF.

Use SGLT2i's in those with type 2 diabetes and either established cardiovascular (CV) disease or at high CV disease risk to prevent hospitalizations for HF.

Pre-HF (stage B) (class I recommendations)

• Patients with an LVEF of 40% or less: Use an ACEI to prevent symptomatic HF and lower mortality; or use beta blockers to prevent symptomatic HF.
• Those with a recent or remote history of myocardial infarction (MI) or acute coronary syndrome (ACS): Use statins to prevent symptomatic HF and adverse CV events.
• Those with a recent or remote history of MI or ACS and an LVEF of 40% or less: Use evidence-based beta blockers to lower mortality
• Those with a recent MI and an LVEF of 40% or less who are not able to tolerate ACEIs: Use angiotensin receptor blockers (ARBs) to prevent symptomatic HF and lower mortality.
• Patients who are a minimum of 40 days post-MI with an LVEF of 30% or less and New York Heart Association (NYHA) class I symptoms while on GDMT and with a reasonable expectation of meaningful survival for longer than 1 year: The ACC/AHA/HFSA recommend an implantable cardioverter-defibrillator (ICD) for primary prevention of sudden cardiac death to lower total mortality.

HFrEF

Pharmacotherapy for (NYHA class II-IV HFrEF (LVEF ≤40%) to reduce the risk of HF hospitalization and death:

• Use an ACEI, an MRA, or dapagliflozin or empagliflozin for those with HFrEF
• Use a beta-blocker for those with stable HFrEF
• Use sacubitril/valsartan as a replacement for ACEI
• Use an ARB in symptomatic patients unable to tolerate and ACEI or ARNI (they should also receive a beta blocker and an MRA)

Use loop diuretics in those with HFrEF who have signs/symptoms of congestion to alleviate HF symptoms, improve exercise capacity, and reduce HF hospitalizations.

HFmrEF

Pharmacotherapy for (NYHA class II-IV HFmrEF includes the following:

• Use diuretics in patients with congestion and HFmrEF to alleviate signs/symptoms.
• An ACEI, ARB, beta blocker, MRA, or sacubitril/valsartan may be considered to reduce the risk of HF hospitalization and death.

HFpEF

Use diuretics in patients with congestion and HFpEF to alleviate signs/symptoms.

2016 ACC/AHA/HFSA Recommendations

In 2016, the ACC/AHA/HFSA  published a focused update on new pharmacologicaly therapy for heart failure ^[63]  which were developed in collaboration with the International Society for Heart and Lung Transplantation (ISHLT). The recommendations are aligned with those of the 2016 ESC guidelines ^[8]  and the 2017 ACC/AHA focused updates to the 2013 guidelines, ^[61] and are summarized below.

Class I

Reduction of morbidity and mortality

In patients with chronic heart failure with reduced ejection fraction (HFrEF),  one of the following agents should be administered in conjunction with evidence-based beta blockers: 

• Angiotensin-converting enzyme  inhibitors (ACEIs) (Level of evidence: A)
• Angiotensin receptor blockers (ARBs) (Level of evidence: A) 
• Angiotensin receptor–neprilysin inhibitor (ARNI) (Level of evidence: B-R) 

In patients with prior or current symptoms of chronic HFrEF, ACEIs are beneficial. (Level of evidence: A) 

In patients with prior or current symptoms of chronic HFrEF who are intolerant to ACEIs because of cough or angioedema, ARBs are recommended. (Level of evidence: A) 

In patients with chronic symptomatic HFrEF New York Heart Association (NYHA) class II or III, replace an ACEI or ARB with an ARNI. (Level of evidence: B-R) 

Class IIa

Ivabradine can reduce heart failure hospitalization for patients receiving guideline-directed evaluation and management who have symptomatic (NYHA class II-III) stable chronic HFrEF (left ventricular ejection fraction of ≤35%) and who are in sinus rhythm with a heart rate of at least 70 bpm at rest.(Level of evidence: B-R) 

Class III

ARNI should not be given in the following situations:

• Concomitantly with or within 36 hours of the last dose of an ACEI
• Patients with a history of angioedema

2020 CCS/CHFS Guidelines

The 2020 Canadian Cardiovascular Society (CCS) and Canadian Heart Failure Society (CHFS) presented new evidence for angiotensin receptor neprilysin inhibitors (ARNIs) in HFpEF as well as for SGLT2 inhibitors and HF. ^[265]

New evidence for ARNIs in HFpEF

The Prospective Comparison of ARNI (angiotensin receptor-neprilysin inhibitors) with ARB (angiotensin-receptor blockers) Global Outcomes in Heart Failure With Preserved Ejection Fraction (PARAGON-HF) trial, which compared sacubitril/valsartan with valsartan in HFpEF patients, showed a modest but nonsignificant 13% reduction in the primary outcome of first and recurrent HF hospitalizations and cardiovascular death. A secondary end point analysis revealed improvement in quality of life and renal function, which suggested potential benefits with sacubitril/valsartan compared with valsartan. The data further suggest heterogeneity in the treatment response with greater benefit in women and in individuals with a lower LVEF.

The CCS/CHFS indicate that the statistically negative results of the primary end point analysis preclude any recommendation for the general use of sacubitril/valsartan in patients with HFpEF.

New evidence for SGLT2 inhibitors and HF

The CCS/CHFS recommend use of SGLT2 inhibitors (eg, empagliflozin, canagliflozin, dapagliflozin) for treatment of patients with type 2 diabetes and atherosclerotic cardiovascular disease to reduce the risk of HF hospitalization and death (strong recommendation).

SGLT2 inhibitors, such as dapagliflozin, are recommended for use in patients with the following features:

• Type 2 diabetes, aged >50 years, with additional risk factors for atherosclerotic cardiovascular disease, to reduce the risk of HF hospitalization (strong recommendation)

• Mild to moderate HF due to reduced LVEF (≤ 40%) and concomitant type 2 diabetes, to improve symptoms and quality of life and to reduce the risk of hospitalization and cardiovascular mortality (strong recommendation)

• Mild to moderate HF due to reduced LVEF (≤ 40%) and without concomitant diabetes, to improve symptoms and quality of life and to reduce the risk of hospitalization and cardiovascular mortality (conditional recommendation)

The CCS/CHFS recommend SGLT2 inhibitors, such as canagliflozin, be used in patients aged >30 years with type 2 diabetes, and macroalbumineric renal disease, to reduce the risk of HF hospitalization and progression of renal disease (strong recommendation).

Practical tips

Note that SGLT2 inhibitors are currently contraindicated for patients with type 1 diabetes.

The most common adverse effects of SGLT2 inhibitors are genital mycotic infections (GMIs), with the highest risk in women (10%-15% risk), those with previous GMIs, and uncircumcised men. GMIs can generally be managed with antifungal drugs and do not require discontinuation of therapy.

SGLT2 inhibitors might result in an up to 15% temporary reduction of estimated glomerular filtration rate (eGFR) (usually resolves within 1-3 months). These drugs have also been associated with acute kidney injury, and increased monitoring is warranted in those at risk.

SGLT2 inhibitors do not cause hypoglycemia in the absence of concomitant insulin and/or secretagogue therapy. Background therapies might need adjustment to prevent hypoglycemia.

SGLT2 inhibitors should be held in the setting of concomitant dehydrating illness as part of “sick day” management. Patients should be educated on “sick day” management.

These agents have been associated with diabetic ketoacidosis (incidence 0.1%). Patients might present with normal or only modestly elevated blood glucose level (< 14 mmol/L). Rarely, SGLT2 inhibitors might be associated with normal anion gap acidosis (best detected with measurement of serum ketones). Nonspecific symptoms associated with diabetic ketoacidosis include dyspnea, nausea/vomiting, abdominal pain, confusion, anorexia, excessive thirst, and lethargy.

Exercise caution when combining SGLT2 inhibitors, ARNIs, and diuretics because of their concomitant effects to promote diuresis.

The 2010 Heart Failure Society of America (HFSA) guidelines indicate that device therapy is an integral part of the treatment of heart failure and that considerations such as the nature and severity of the condition and any patient comorbidities are essential in optimizing the use of this therapy. ^[9] The Committee for Practice Guidelines (CPG) of the European Society of Cardiology (ESC) as well as the American College of Cardiology, American Heart Association, and Heart Rhythm Society (ACC/AHA/HRS) emphasized the importance of medical devices in heart failure in their respective 2010 and 2012 focused updates on these interventions. ^{[116, 266]}

More recently, the 2022 ACC/AHA/HFSA guidelines also provided the following level recommendations for implantable cardioverter-defibrillators (ICDs) for primary prevention of sudden cardiac death to lower total mortality: ^[4]

• Individuals with nonischemic dilated cardiomyopathy (DCM) or ischemic heart disease that is a minimum of 40 days post myocardial infarction (MI), who have a left ventricular (LV) ejection fraction (EF) of 35% or less and aNew York Heart Association (NYHA) class II or III symptoms while on chronic guideline-directed medical therapy (GDMT), as well as having a reasonable expectation of meaningful survival for longer than 1 year
• Patients with a minimum of 40 days post-MI who have an LVEF of 30% or less and NYHA class I symptoms while on GDMT, with a reasonable expectation of meaningful survival for longer than 1 year

Cardiac resynchronization therapy (CRT) is recommended for individuals with an LVEF of 35% or less, sinus rhythm, left bundle branch block (LBBB) with a QRS duration of at least 150 ms, and a NYHA class II, III, or ambulatory IV symptoms on GDMT to lower total mortality and hospitalizations as well as improve symptoms and quality of life.

Pacemakers

Because right ventricular (RV) pacing may worsen heart failure due to an increase in ventricular dysynchrony, the 2010 HFSA Practice Guidelines recommend against placement of a dual-chamber pacemaker in heart failure patients in the absence of symptomatic bradycardia or high-degree atrioventricular (AV) block. ^[9]

The ACC/AHA heart failure guidelines recommend consideration of CRT for patients with heart failure who have indications for permanent pacing (eg, first implant, upgrading of a conventional pacemaker) and NYHA class III-IV symptoms or those who have an LVEF below 35% despite being on optimal heart failure therapy and who may have a dependence on RV pacing. ^{[3, 266]}  These recommendations also include patients with NYHA class II symptoms and the presence of LBBB with a QRS duration that is at least 150 ms. The ESC guidelines have similar recommendations. ^[8]  

Implantable cardioverter-defibrillators

ACC Foundation (ACCF)/AHA guidelines recommend placing an ICD in virtually all patients with an LVEF below 35%. The ACCF/AHA and ESC recommend ICD placement for the following categories of heart failure patients ^{[3, 8, 267]} :

• Patients with LV dysfunction (LVEF ≤35%) from a previous MI who are at least 40 days post-Ml
• Patients with nonischemic cardiomyopathy; with an LVEF of 35% or less; in NYHA class II or III; receiving optimal medical therapy; and expected to survive longer than 1 year with good functional status
• Patients with ischemic cardiomyopathy who are at least 40 days post-MI; have an LVEF of 30% or less; are in NYHA functional class I; are on chronic optimal medical therapy; and are expected to survive longer than 1 year with good functional status
• Patients who have had ventricular fibrillation (VF)
• Patients with documented hemodynamically unstable ventricular tachycardia (VT) and/or VT with syncope; with an LVEF below 40%; on optimal medical therapy; and expected to survive longer than 1 year with good functional status

Cardiac resynchronization therapy/biventricular pacing

The 2013 ACCF/AHA guidelines recommend CRT for patients in sinus rhythm or atrial fibrillation with a QRS duration of 120 ms or longer (the greatest benefit is in patients with a QRS >150 ms) and an LVEF of 35% or less with persistent, moderate-to-severe heart failure (NYHA class III and functional NYHA class IV) despite optimal medical therapy. ^[3]  A 2012 update of ACC/AHA/HRS guidelines on CRT expanded class I indications to patients with NYHA class II symptoms and LBBB duration of 150 ms or longer. ^[266]  Additional CRT recommendations include ^{[3, 266]} :

• Patients with a reduced LVEF and a QRS of 150 ms or longer who have NYHA I or II symptoms
• Patients with a reduced LVEF who require chronic pacing and in whom frequent ventricular pacing is expected
• CRT is not recommended for patients with NYHA class I or II symptoms and non-LBBB pattern with a QRS duration shorter than 150 ms
• CRT is not indicated in patients who are not expected to survive for more than 1 year due to their comorbidities or frailty

The 2022 ACC/AHA/HFSA guidelines indicate an "upgrade" to CRT should be considered for patients with an LVEF of 35% or less who have received a conventional pacemaker or an ICD and who then develop worsening HF despite optimal medical therapy and who have a signficant proportion of RV pacing.

The ESC guidelines gives class I recommendations for the use of CRT in the following groups ^[8] :

• Symptomatic patients in sinus rhythm with a QRS duration of 150 ms or longer, LBBB QRS morphology and an LVEF of 35% or less despite optimal medical therapy. (Level of evidence: A)
• Symptomatic patients in sinus rhythm with a QRS duration of 130-149 ms or longer, LBBB QRS morphology and an LVEF of 35% or less despite optimal medical therapy. (Level of evidence: B)
• CRT rather than RV pacing for patients with heart failure with reduced ejection fraction (HFrEF) regardless of NYHA class, including patients with atrial fibrillation who have an indication for ventricular pacing and a high degree AV block. (Level of evidence: A)

CRT should be considered for the following groups ^[8] :

• Symptomatic patients in sinus rhythm with a QRS duration of 150 ms or longer, non-LBBB QRS morphology and an LVEF of 35% or less despite optimal medical therapy. (Class IIa; level of evidence: B)
• Patients with LVEF of 35% or less in NYHA Class III-IV despite optimal medical therapy, if they are in atrial fibrillation and have a QRS duration of 130 ms or longer provided a strategy to ensure biventricular capture is in place or the patient is expected to return to sinus rhythm. (Class IIa; level of evidence: B)

CRT may be considered for the following groups ^[8] :

• Symptomatic patients in sinus rhythm with a QRS duration of 130-149 ms, non-LBBB QRS morphology and with an LVEF of 35% or less despite optimal medical therapy. (Class IIb; level of evidence: B)
• Patients with HFrEF who have received a conventional pacemaker or an ICD and subsequently develop worsening heart failure despite optimal medical therapy and who have a high proportion of RV pacing. (Class IIb; level of evidence: B)

CRT is contraindicated in patients with a QRS duration below 130 ms. (Class III; level of evidence: A)

The American College of Cardiology Foundation/American Heart Association (ACCF/AHA), Heart Failure Society of America (HFSA), and European Society of Cardiology (ESC) guidelines recommend coronary artery bypass graft (CABG) and percutaneous coronary intervention (PCI) revascularization procedures in selected patients with heart failure and coronary artery disease (CAD) to improve symptoms and survival. ^{[3, 8, 9]} In patients who are at low risk for CAD, findings from noninvasive tests such as exercise electrocardiography (ECG), stress echocardiography, and stress nuclear perfusion imaging should determine whether subsequent angiography is indicated.

In selected patients with heart failure, reduced ejection fraction (EF) (ef ≤35%), and a suitable coronary anatomy, surgical revascularization and guideline-directed medical therapy helps improve symptoms, cardiovascular hospitalizations, and long-term all-cause mortality. ^{[4, 5]}

The ACCF/AHA guidelines recommend revascularization procedures for the following heart failure patients ^[3] :

• CABG or PCI for those on medical therapy with angina and suitable coronary anatomy, especially significant left main stenosis (>50%) or left main equivalent
• CABG to improve survival in patients with mild to moderate left ventricular (LV) systolic dysfunction (EF OF 35%-50%) and significant (≥70% stenosis) multivessel CAD or proximal left anterior descending (LAD) artery stenosis in the presence of viable myocardium
• CABG to improve morbidity and survival for patients with an LVEF of 35% or less, heart failure, and significant multivessel CAD
• CABG may also be considered in patients with ischemic heart disease, severe LV systolic dysfunction (EF < 35%), and operable coronary anatomy, regardless of whether or not viable myocardium is present

The ESC guidelines are in general agreement with those of ACCF/AHA, with the choice between CABG and PCI individualized for each patient. ^[8] In addition, the ESC points out that the benefit-risk balance of revascularization in patients without angina and without viable myocardium remains uncertain.

The American College of Cardiology Foundation/American Heart Association (ACCF/AHA) recommends aortic valve replacement for patients with critical aortic stenosis and predicted surgical mortality of 10% or less, as well as transcatheter aortic valve replacement for selected patients who are considered to be inoperable. ^[3] The benefit of transcatheter mitral valve repair or mitral valve surgery for functional mitral insufficiency is unclear and should only be considered after careful candidate selection.

The 2022 ACC/AHA/Heart Failure Society of America (HFSA) has the following level I recommendations for valvular heart disease in those with heart failure (HF) ^[4] :

• A multidisciplinary management strategy according to clinical practice guidelines for valvular heart disease to prevent worsening HF and adverse clinical outcomes
• Optimization of guideline-directed medical therapy (GDMT) for those with chronic severe secondary mitral regurgitation (MR) and HF with reduced ejection fraction (EF) (HFrEF) before any intervention for secondary MR related to left ventricular (LV) dysfunction

The HFSA indicates that isolated mitral valve repair or replacement for severe mitral regurgitation secondary to ventricular dilatation in the presence of severe LV systolic dysfunction is not generally recommended. ^[9]

Although the European Society of Cardiology (ESC) recommends optimized medical treatment for aortic stenosis, it also cautions that vasodilators may cause hypotension and should be used with caution. Surgical decision making should not be delayed. For patients unfit for surgery, transcatheter aortic valve replacement should be considered. Additional valvular surgery recommendations include ^[8] :

• Aortic valve repair or replacement in all symptomatic patients with severe aortic regurgitation as well as asymptomatic patients with an LV EF of 50% or less who are fit for surgery.
• Consider a combination valve and coronary surgery for secondary mitral regurgitation in symptomatic patients with an LVEF below 30% with suitable arteries for revascularization. Surgery is also recommended for those with severe mitral regurgitation with an LVEF over 30% undergoing coronary artery bypass grafting.
• Isolated mitral valve surgery in patients with severe functional mitral regurgitation and severe LV systolic dysfunction (LVEF < 30%) who cannot be revascularized or have non-ischemic cardiomyopathy is questionable; conventional medical and device therapy are preferred. In selected cases, consider repair to avoid or postpone transplantation.

The 2020 Canadian Cardiovascular Society (CCS) and Canadian Heart Failure Society (CHFS) recommendations for percutaneous mitral valve repair for HF and reduced EF and severe functional mitral regurgitation include the following ^[265] :

• The CCS/CHFS recommends maximally tolerated guideline-directed medical therapy (GDMT), including cardiac resynchronization therapy and revascularization where appropriate, be implemented before consideration of percutaneous mitral valve repair (PMVR) for patients with HF and reduced ejection fraction (HFrEF) and severe functional mitral regurgitation (FMR) (strong recommendation).
• It is suggested that patients with symptomatic HF (HFrEF) despite maximal GDMT and severe mitral regurgitation be evaluated for PMVR (weak recommendation).
• The CCS/CHFS recommends that a multidisciplinary dedicated heart team (including interventionalists, cardiac surgeons, imaging specialists, and HF specialists) evaluate and manage the care of potential candidates for PMVR (strong recommendation).

Practical tips by the CCS/CHFS include the following ^[265] :

• Use caution when treating FMR in patients with HFrEF.
• Patients with HFrEF and FMR who have severe left ventricular (LV) dilatation (typically LV end diastolic dimension >70 mm) and less than severe mitral regurgitation may be poor candidates for PMVR with MitraClip.
• Patients with FMR should first receive maximally tolerated GDMT, including pharmacologic and nonpharmacologic HF therapies (eg, cardiac resynchronization therapy where applicable) for a reasonable minimum period (eg, 3 months), before PMVR is considered.
• Refer patients considered for PMVR to centers experienced in evaluating patients with advanced HF, have high volumes of patients with valve disease managed medically and surgically, and have a high likelihood of achieving the volume of PMVR (eg, 2-4 per month) required for developing and maintaining competence in well-selected patients.

The following organizations have released guidelines for the utilization of mechanical circulatory support (MCS):

• Society for Cardiovascular Angiography and Interventions, American College of Cardiology, Heart Failure Society of America, and Society for Thoracic Surgeons (SCAI/ACC/HFSA/STS)
• International Society of Heart and Lung Transplantation (ISHLT)
• American Heart Association (AHA)

Historically, the intra-aortic balloon bump (IABP) and extracorporeal membrane oxygenation (ECMO) devices had been the only MCS devices available to clinicians, but axial flow pumps (eg, Impella) and left atrial to femoral artery bypass pumps (eg, TandemHeart) have more recently entered clinical practice. ^[268]

The 2015 SCAI/ACC/HFSA/STS clinical expert consensus-based recommendations include the following ^[268] :

• Percutaneous circulatory assist devices provide superior hemodynamic support (reduce left ventricular [LV] pressures, LV volumes, LV stroke volume) compared with pharmacologic therapy; this is particularly apparent for the Impella and TandemHeart devices.
• In those with cardiogenic shock who fail to stabilize or show signs of improvement after initial interventions, consider early placement of an appropriate MCS.
• For profound cardiogenic shock, IABP is less likely to provide benefit than continuous flow pumps (including the Impella CP and TandemHeart). ECMO may also be beneficial, particularly for patients with impaired respiratory gas exchange.
• Consider MCS for isolated acute right ventricular (RV) failure complicated by cardiogenic shock.
• MCS can be beneficial in high-risk percutaneous coronary intervention (PCI) (eg, multivessel, left main, or last patent conduit interventions), particularly if the patient is inoperable or has severely reduced ejection fraction or elevated cardiac filling pressures
• MCS can be utilized when patients fail to wean off of cardiopulmonary bypass.
• Early MCS may benefit patients with acute decompensated heart failure when they continue to deteriorate despite initial interventions.
• MCS can be used in severe biventricular failure via both right- and left-sided percutaneous devices or venoarterial ECMO.

However, there was insufficient evidence to support or refute routine use of MCS as an adjunct to primary revascularization in the setting of large acute MI (myocardial infarction) to reduce reperfusion injury or infarct size. ^[268]

In its 2013 guidelines for mechanical circulatory support, the ISHLT recommended long-term MCS for the following patients in acute cardiogenic shock (class IIa) ^[269] :

• Those whose ventricular function is considered unrecoverable or unlikely to recover without long-term device support (level of evidence: C)
• Those considered too ill to maintain normal hemodynamics and vital organ function with temporary MCS, or who cannot be weaned from temporary MCS or inotropic support (level of evidence: C)
• Those with the capacity for meaningful recovery of end-organ function and quality of life (level of evidence: C)
• Those without irreversible end-organ damage (level of evidence: C)
• Those who are dependent on inotropic agents (level of evidence: B)
• Those with end-stage systolic heart failure who do not fall into one of the recommendations: Routine risk stratification at regular intervals to determine the need for and optimal timing of MCS (level of evidence: C)

Additional recommendations for heart failure therapy include ^[269] :

• Diuretic agents for the management of volume overload during MCS (class I; level of evidence: C)
• An angiotensin-converting enzyme inhibitor (ACEI) or an angiotensin receptor blocker (ARB) for managing hypertension or for risk reduction in patients with vascular disease and diabetes (class I; level of evidence: C.)
• Beta-blockers for hypertension or for rate control in patients with tachyarrhythmias (class I; level of evidence: C.)
• Mineralocorticoid receptor antagonists to limit the need for potassium repletion in patients with adequate renal function and for potential beneficial antifibrotic effects on the myocardium (class I; level of evidence: C.)
• Digoxin, potentially, for treating atrial fibrillation with rapid ventricular response (class II; level of evidence: C.)

The 2012 AHA guidelines on heart device strategies, patient selection, and postoperative care focuses on risk stratification and early referral of high-risk patients with heart failure to centers that can implant MCS. ^[270] The specific recommendations for MCS include ^[270] :

• Consider MCS as a bridge to transplantation (BTT) for eligible patients with end-stage heart failure who are failing optimal medical, surgical, and or device therapies and are at high risk for dying before receiving heart transplantation.
• Early referral for MCS before development of advanced heart failure is preferred.
• Durable, implantable MCS devices is beneficial as permanent or destination therapy for patients with advanced heart failure, high 1-year mortality resulting from HF, and the absence of other life-limiting organ dysfunction; who are failing medical, surgical, and/or device therapies; and who are not heart transplant candidates.
• Consider patients who are ineligible for heart transplantation because of pulmonary hypertension related to heart failure alone for bridge to potential transplant eligibility with durable, long-term MCS.
• Consider urgent nondurable MCS in hemodynamically compromised patients with heart failure and end-organ dysfunction and/or relative contraindications to heart transplantation/durable MCS that are expected to improve with restoration of an improved hemodynamic profile.
• Long-term MCS is not recommended in patients with advanced kidney disease in whom renal function is unlikely to recover despite improved hemodynamics.
• Consider long-term MCS as a bridge to heart-kidney transplantation on the basis of the availability of outpatient hemodialysis.

According to the American College of Cardiology Foundation/American Heart Association (ACCF/AHA) and Heart Failure Society of America (HFSA) guidelines, selected patients with refractory end-stage heart failure, debilitating refractory angina, ventricular arrhythmia, or congenital heart disease that cannot be controlled despite pharmacologic, medical device, or alternative surgical therapy should be evaluated for heart transplantation. ^{[3, 9]}

The European Society of Cardiology (ESC) guidelines recommend heart transplantation be considered for patients with progressive end-stage heart failure despite maximal medical therapy who have a poor prognosis and no viable alternative form of treatment; these patients must be well informed, motivated, and emotionally stable, and they must be capable of complying with intensive medical treatment. ^[8]

The ESC considers the following conditions as contraindications for heart transplantation ^[8] :

• Active infection
• Severe peripheral arterial or cerebrovascular disease
• Current alcohol and/or drug abuse
• Malignancy (collaborate with oncologists for risk stratification of tumor recurrence)
• Irreversible renal dysfunction (creatinine clearance < 30 mL/min)
• Pharmacologically irreversible pulmonary hypertension (consider placing a left ventricular assist device and then reevaluating eligibility)
• Multiorgan systemic disease
• Other serious comorbidity with a poor prognosis
• Pretransplant body mass index above 35 kg/m ²
• insufficient social support in the outpatient setting to achieve compliant care

Note that the HFSA does not recommend partial left ventriculectomy (Batista operation) to treat nonischemic cardiomyopathy. ^[9]

The Heart Failure Society of America (HFSA) guidelines recommend the following treatment goals for patients with acute decompensated heart failure (ADHF) ^[9] :

• Symptomatic improvement (ie, congestion, low output)
• Restoration of normal oxygenation
• Optimization of volume status
• Identification of the etiology and addressing precipitating factors
• Optimization of long-term oral therapy
• Minimization of side effects
• Identification of patients in whom revascularization or device therapy may be beneficial
• Risk stratification for venous thromboembolism and potential need for anticoagulation
• Patient education regarding medications and self-management of heart failure
• Initiation of a disease management program, where possible

HFSA indications for hospital admission in patients with ADHF are as follows ^[9] :

• Evidence of severely decompensated heart failure, including hypotension, worsening renal function, and altered mentation
• Dyspnea at rest
• Hemodynamically significant arrhythmia, including new onset of rapid atrial fibrillation
• Acute coronary syndromes

The American College of Cardiology Foundation/American Heart Association (ACCF/AHA) comments regarding adjustment of maintenance heart failure medications in patients admitted with ADHF are as follows:

• Oral therapy should be continued, or even uptitrated, in most patients with reduced ejection fraction heart failure.
• Most patients tolerate well the continuation of angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) and beta-blockers; this also results in better outcomes.
• Only consider withholding or reducing beta-blockers in patients hospitalized after a recent beta-blocker initiation or increase in beta-blocer therapy, or in those with marked volume overload or marginal/low cardiac output.
• In patients with significant worsening of renal function, consider a reduction in, or temporary discontinuation of, ACEIs, ARBs, and/or aldosterone antagonists until renal function improves.

Pharmacologic therapy

The ACCF/AHA, HFSA, and European Society of Cardiology (ESC) agree that diuretics remain the cornerstone of standard therapy. ^{[3, 8, 9]} The aim of diuretic therapy is to achieve and maintain euvolaemia with the lowest achievable dose. ^[8] Intravenous (IV) administration of a loop diuretic (eg, furosemide, bumetanide, torsemide) is preferred initially. ^{[3, 8, 9]}  In patients with hypertensive heart failure who have mild fluid retention, thiazide diuretics (eg, bendroflumethiazide, hydrochlorothiazide, metolazone) may be preferred because of their more persistent antihypertensive effects. ^[8]

When diuresis is inadequate, the ACCF/AHA, HFSA and ESC guidelines recommend higher doses or the addition of a second diuretic (eg, a thiazide). ^{[3, 8, 9]}  Careful monitoring to avoid hypokalemia, renal dysfunction, and hypovolemia is required. The ACC/AHA and ESC suggest the use of ultrafiltration for fluid reduction when diuretic therapies are unsuccessful. ^{[3, 8]}

Vasodilators (eg, nitroprusside, nitroglycerin, or nesiritide) are recommended as an adjuvant to diuretics for relief of symptoms. ^{[3, 8, 9]} However, the ESC cautions against their use in patients with a systolic blood pressure below 90 mm Hg or those with significant mitral or aortic stenosis. ^[8]

The ACCF/AHA, HFSA, ESC recommend that in hospitalized patients, beta-blocker therapy should be initiated after optimization of volume status and successful discontinuation of IV diuretics, vasodilators, and inotropic agents. ^{[3, 8, 9]} Beta-blockers should be started at a low dose and only in stable patients, and should be used cautiously in patients who have required inotropes during their hospital course. ^{[3, 8, 9]}

Additional recommendations from the 2013 ACC/AHA and 2010 HSFA guidelines include the following ^{[3, 9]} :

• If symptomatic hypotension is absent, consider IV nitroglycerin, nitroprusside, or nesiritide an adjuvant to diuretic therapy for relief of dyspnea in hospitalized patients.
• Administer venous thromboembolism prophylaxis with an anticoagulant medication for patients admitted to the hospital, if the risk-benefit ratio is favorable.

Invasive hemodynamic monitoring

The 2013 ACCF/AHA and 2010 HSFA guidelines found no benefit found for the routine use of invasive hemodynamic monitoring in normotensive patients with acute decompensated heart failure and congestion with symptomatic response to diuretics and vasodilators. ^{[3, 9]}  The HSFA guidelines include a recommendation for consideration of invasive hemodynamic monitoring for patients with any of the following ^[9] :

• Heart failure refractory to initial therapy
• Unclear volume status and cardiac filling pressures
• Clinically significant hypotension (systolic blood pressure < 80 mm Hg) or worsening renal function during therapy
• Need for assessment of degree and reversibility of pulmonary hypertension, as part of the evaluation for possible cardiac transplantation
• Need for documentation of an adequate hemodynamic response to inotropic therapy, when considering long-term outpatient infusion

Ventilation

The HFSA recommends routine administration of supplemental oxygen only in the presence of hypoxia; noninvasive positive pressure ventilation (NIPPV) should be considered for severe dyspnea and clinical evidence of pulmonary edema. ^[9]  The ESC recommends noninvasive ventilation as an adjunctive therapy to improve outcomes in patients with acute respiratory failure due to hypercapnic exacerbation of chronic obstructive pulmonary disease or heart failure in the setting of acute pulmonary edema. ^[8]