**4. Clinical management of dilated cardiomyopathy**

#### **4.1 Heart failure**

Heart failure management should be in accordance with guidelines, such as those of the ESC, which are summarized below [1]. An angiotensin converting enzyme inhibitor (ACEi) or angiotensin receptor blocker (ARB) is indicated in left ventricular systolic dysfunction. If symptomatic, i.e. New York Heart Association functional classification (NYHA-class) 2 or above, a beta-blocker should be added to treatment with the ACEi/ARB. In patients who remain symptomatic with systolic

**175**

diabetes mellitus [15].

in atrial fibrillation [3].

**4.3 Prevention of sudden cardiac death**

**4.2 Arrhythmia**

*Familial Dilated Cardiomyopathy: Risk Stratification for Sudden Cardiac Death*

ejection fraction ≤35% despite the highest tolerable evidence-based doses of ACEi or ARB as well as a beta-blocker, a mineral receptor antagonist is recommended with maximum tolerated evidence-based dose. If the patient is still symptomatic with systolic ejection fraction ≤35% it is recommended to initiate treatment with an angiotensin receptor neprilysin inhibitor that replaces the ACEi/ARB [1]. Further treatment modalities that should be considered include the addition of ivabradine in patients with sinus rhythm ≥70 beats per minute, or the implantation of a cardiac device to allow for cardiac resynchronization therapy in those with left bundle branch block and QRS ≥130 ms or without left bundle branch block but QRS ≥150 ms. These mentioned treatment modalities have shown increased survival in randomized controlled trials [1]. Digoxin could be considered if symptoms remain, however reduced mortality has not been shown but rather reduced need for hospitalization. The small therapeutic window of digoxin should be kept in mind, most commonly digoxin is used for rate control in atrial fibrillation that is common in heart failure. Loop-diuretics such as furosemide should be considered in patients with heart failure to relieve symptoms and signs of congestion, but this has not been shown to reduce mortality. The dosage of diuretics should be kept as low as possible and cessation of treatment might often be possible. In end-stage heart failure, transplant might be considered or mechanical left ventricular assist devices that can be used as destination therapy, bridge-to-decision, or bridge to transplant [1].

Lately inhibition of sodium-glucose transporter protein 2 (SGLT2i) has proved

There is an increased risk for both brady- and tachyarrhythmia in DCM, these arrhythmias can also be a contributing factor worsening heart failure. In symptomatic sinus node disease or in high-degree atrioventricular (AV)-block without a reversible cause a pacemaker is indicated in order the relieve symptoms and/or increase survival [3]. Beta-blockers are often indicated for the treatment of symptomatic heart failure but also have antiarrhythmic properties making it useful for both rhythm and rate control. With the exception of beta-blockers currently available antiarrhythmic drugs have not consistently been shown in randomized clinical trials to improve survival in the primary management of arrhythmia. Amiodarone have shown some positive results and is highly useful to control symptoms, to terminate tachyarrhythmia and prevent recurrence [3]. In heart failure with reduced ejection fraction most other antiarrhythmic agents are contraindicated, this includes flecainide and dronedarone otherwise frequently used for rhythm control

An implantable cardioverter defibrillator (ICD) is an effective way to prevent sudden cardiac death in those at risk for developing ventricular tachycardia or ventricular fibrillation [3]. An ICD offers both antitachycardia pacing, rapid ventricular pacing (preferably bursts), that can terminate ventricular tachycardia, as well as

an interesting treatment modality for heart failure with reduced ejection fraction. In the EMPA-REG trial in 2015, the SGLT2i empagliflozin showed reduced cardiovascular mortality, reduction in all-cause mortality, and reduced need for hospitalization for heart failure in patients with type 2 diabetes mellitus [14]. In 2019 the results of the DAPA-HF trial showed that the SGLT2i dapagliflozin reduced cardiovascular and all-cause mortality as well as risk of worsening heart failure in patients with heart failure with reduced ejection fraction even if they did not have

*DOI: http://dx.doi.org/10.5772/intechopen.94437*

#### *Familial Dilated Cardiomyopathy: Risk Stratification for Sudden Cardiac Death DOI: http://dx.doi.org/10.5772/intechopen.94437*

ejection fraction ≤35% despite the highest tolerable evidence-based doses of ACEi or ARB as well as a beta-blocker, a mineral receptor antagonist is recommended with maximum tolerated evidence-based dose. If the patient is still symptomatic with systolic ejection fraction ≤35% it is recommended to initiate treatment with an angiotensin receptor neprilysin inhibitor that replaces the ACEi/ARB [1]. Further treatment modalities that should be considered include the addition of ivabradine in patients with sinus rhythm ≥70 beats per minute, or the implantation of a cardiac device to allow for cardiac resynchronization therapy in those with left bundle branch block and QRS ≥130 ms or without left bundle branch block but QRS ≥150 ms. These mentioned treatment modalities have shown increased survival in randomized controlled trials [1]. Digoxin could be considered if symptoms remain, however reduced mortality has not been shown but rather reduced need for hospitalization. The small therapeutic window of digoxin should be kept in mind, most commonly digoxin is used for rate control in atrial fibrillation that is common in heart failure. Loop-diuretics such as furosemide should be considered in patients with heart failure to relieve symptoms and signs of congestion, but this has not been shown to reduce mortality. The dosage of diuretics should be kept as low as possible and cessation of treatment might often be possible. In end-stage heart failure, transplant might be considered or mechanical left ventricular assist devices that can be used as destination therapy, bridge-to-decision, or bridge to transplant [1].

Lately inhibition of sodium-glucose transporter protein 2 (SGLT2i) has proved an interesting treatment modality for heart failure with reduced ejection fraction. In the EMPA-REG trial in 2015, the SGLT2i empagliflozin showed reduced cardiovascular mortality, reduction in all-cause mortality, and reduced need for hospitalization for heart failure in patients with type 2 diabetes mellitus [14]. In 2019 the results of the DAPA-HF trial showed that the SGLT2i dapagliflozin reduced cardiovascular and all-cause mortality as well as risk of worsening heart failure in patients with heart failure with reduced ejection fraction even if they did not have diabetes mellitus [15].
