What are the treatment options for cardiomyopathy?

Treatment Options for Cardiomyopathy

The treatment of cardiomyopathy requires guideline-directed medical therapy tailored to the specific type of cardiomyopathy, with medications, devices, and advanced therapies selected based on cardiomyopathy subtype and symptom severity. 1, 2

General Treatment Principles

• Guideline-directed medical therapy forms the foundation for all cardiomyopathy types, with specific modifications based on the cardiomyopathy subtype 2
• Treatment should address both symptom management and reduction of morbidity and mortality 1
• Lifestyle modifications and management of comorbidities are critical components of treatment 2

Treatment by Cardiomyopathy Type

Dilated Cardiomyopathy (DCM)

• Standard heart failure with reduced ejection fraction therapies are recommended for DCM patients 1, 2:
  • Beta-blockers (metoprolol, carvedilol, bisoprolol) 1, 3
  • ACE inhibitors/ARBs/ARNI 4
  • Mineralocorticoid receptor antagonists 2
  • SGLT2 inhibitors 2
• For alcohol-related cardiomyopathy, complete abstinence from alcohol is essential in addition to standard heart failure therapy 1
• Thiamine supplementation should be considered in patients with alcoholic cardiomyopathy due to potential deficiency 1, 2

Hypertrophic Cardiomyopathy (HCM)

• First-line therapy for symptomatic obstructive HCM: non-vasodilating beta-blockers titrated to effectiveness or maximally tolerated doses 1, 2
• Second-line therapy: non-dihydropyridine calcium channel blockers (verapamil, diltiazem) for patients who cannot tolerate beta-blockers or have inadequate symptom control 1, 2, 5
• For patients with HCM who develop systolic dysfunction with LVEF <50%, guideline-directed therapy for heart failure with reduced ejection fraction is recommended 1
• Septal reduction therapy (surgical myectomy or alcohol septal ablation) should be considered for severely symptomatic patients with obstructive HCM despite optimal medical therapy 2

Management of Arrhythmias in Cardiomyopathy

• Anticoagulation with direct-acting oral anticoagulants (first-line) or vitamin K antagonists (second-line) is recommended for patients with HCM and atrial fibrillation, regardless of CHA₂DS₂-VASc score 1
• For rate control in atrial fibrillation, beta-blockers, verapamil, or diltiazem are recommended based on patient preferences and comorbidities 1, 2
• In patients with HCM and symptomatic ventricular arrhythmias or recurrent ICD shocks despite beta-blocker use, antiarrhythmic drug therapy is recommended 1
• For patients with HCM and pacing-capable ICDs, programming antitachycardia pacing is recommended to minimize risk of shocks 1

Advanced Therapies

• Cardiac resynchronization therapy (CRT) should be considered for appropriate patients with DCM 1
• Implantable cardioverter-defibrillator (ICD) therapy is recommended for high-risk patients with ventricular arrhythmias 1, 6
• For patients with HCM and recurrent poorly tolerated life-threatening ventricular tachyarrhythmias refractory to maximal antiarrhythmic drug therapy and ablation, heart transplantation assessment is indicated 1
• Mechanical circulatory support and heart transplantation should be considered for patients with advanced heart failure who fail to respond to medical therapy 1, 6

Special Considerations for Specific Cardiomyopathies

Peripartum Cardiomyopathy

• Multidisciplinary team management including cardiologists, high-risk obstetricians, and perinatologists is essential 1
• During pregnancy, medications must be carefully selected due to potential fetal risks:
  • Diuretics should be used sparingly to avoid reducing fetal blood flow 1
  • ACE inhibitors and ARBs are contraindicated during pregnancy 1
  • Hydralazine can be considered for hypertension management 1
• After delivery, standard heart failure medications can be initiated with consideration for breastfeeding status 1

Chemotherapy-Related Cardiomyopathy

• Regular cardiac functional assessment with LVEF measurement is recommended at baseline, during treatment, and after completion of cardiotoxic chemotherapy 1
• Early initiation of ACE inhibitors and beta-blockers is recommended when cardiotoxicity is detected 1
• For patients at high risk for cardiac toxicity, strategies such as continuous infusions, liposome encapsulation, or cardioprotective agents like dexrazoxane should be considered 1

Common Pitfalls and Caveats

• Beta-blockers must be started at very low doses (one-tenth to one-twentieth of doses used for angina or hypertension) in heart failure patients and gradually titrated up to avoid decompensation 3
• In cocaine-related cardiomyopathy, non-selective beta-blockers with alpha-blocking properties are preferred over selective beta-blockers to prevent unopposed alpha-agonism 1
• When treating stress-induced cardiomyopathy, catecholamine-based inotropes like dobutamine may theoretically worsen the condition; intra-aortic balloon pump and calcium-sensitizing agents are preferred 1
• For tachycardia-induced cardiomyopathy, control of the heart rate or restoration of sinus rhythm is essential for recovery of cardiac function 1