What are the pillars to treat cardiomyopathy?

Pillars of Cardiomyopathy Treatment

The pillars of cardiomyopathy treatment include symptom management with appropriate medications, arrhythmia control, prevention of disease progression, and advanced therapies for refractory cases, with specific approaches tailored to the type of cardiomyopathy. 1

General Treatment Principles

• Guideline-directed medical therapy is the foundation for all cardiomyopathy types, with specific modifications based on the cardiomyopathy subtype (hypertrophic, dilated, restrictive) 1
• An integrated approach addressing stroke risk, symptom control through rate and rhythm strategies, and modification of risk factors is essential, particularly in cardiomyopathies complicated by atrial fibrillation 1
• Lifestyle modifications and management of comorbidities (sleep apnea, obesity, physical inactivity, hypertension, hyperthyroidism, alcohol misuse) are critical to reduce disease burden 1

Medication Management by Cardiomyopathy Type

Hypertrophic Cardiomyopathy (HCM)

• First-line therapy: Non-vasodilating beta-blockers titrated to effectiveness or maximally tolerated doses for symptomatic patients with obstructive HCM 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
• Third-line therapy: Disopyramide in combination with beta-blockers or verapamil for patients with persistent symptoms despite first and second-line therapies 1, 2
• Caution: Verapamil is potentially harmful in patients with severe dyspnea at rest, hypotension, or very high resting gradients (>100 mm Hg) due to risk of hemodynamic deterioration 1, 3

Dilated Cardiomyopathy (DCM)

• Standard heart failure with reduced ejection fraction (HFrEF) therapies including beta-blockers, ACE inhibitors/ARBs/ARNI, mineralocorticoid receptor antagonists, and SGLT2 inhibitors 1
• For patients with DCM who develop systolic dysfunction with LVEF <50%, guideline-directed therapy for HF with reduced EF is recommended 1
• Diagnostic testing to assess for concomitant causes of systolic dysfunction (such as coronary artery disease) is essential in DCM management 1

Restrictive Cardiomyopathy

• Treatment focuses on symptom management as reverse remodeling is not typically a therapeutic goal 1
• Heart rate control is crucial as cardiac output is dependent on heart rate changes 1
• Beta-blockers may not be tolerated due to their negative chronotropic and inotropic effects 1

Arrhythmia Management in Cardiomyopathies

• Atrial fibrillation: Anticoagulation with direct-acting oral anticoagulants (first-line) or vitamin K antagonists (second-line) is recommended regardless of CHA₂DS₂-VASc score in HCM patients 1, 2
• Rate control: Beta-blockers, verapamil, or diltiazem are recommended for rate control in AF, with choice guided by patient preferences and comorbidities 1
• Rhythm control: For poorly tolerated AF, a rhythm control strategy with cardioversion or antiarrhythmic drugs can be beneficial 1
• Ventricular arrhythmias: Antiarrhythmic drugs (amiodarone, dofetilide, mexiletine, or sotalol) are recommended for symptomatic ventricular arrhythmias or recurrent ICD shocks despite beta-blocker use 1

Advanced Therapies

• Septal reduction therapy: For severely symptomatic patients with obstructive HCM despite optimal medical therapy, surgical myectomy or alcohol septal ablation performed at experienced centers is recommended 1
• ICD therapy: For high-risk patients with ventricular arrhythmias, with programming of antitachycardia pacing to minimize risk of shocks 1
• Catheter ablation: For patients with recurrent symptomatic sustained monomorphic VT or recurrent ICD shocks despite optimal device programming and antiarrhythmic therapy 1
• Heart transplantation: For patients with advanced heart failure symptoms or life-threatening ventricular arrhythmias refractory to maximal medical therapy and other interventions 1

Special Considerations

Alcohol-Related Cardiomyopathy

• Management should begin with total abstinence from alcohol in addition to conventional heart failure management 1
• Thiamine supplementation should be considered due to potential deficiency in chronic alcoholism 1

Chemotherapy-Related Cardiomyopathy

• Early detection and monitoring of cardiotoxicity is essential, especially with anthracycline-based therapies 1
• Standard heart failure therapies are recommended, with dose adjustments based on patient tolerance 1

Common Pitfalls and Caveats

• Vasodilators (ACE inhibitors, ARBs, dihydropyridine calcium channel blockers) should be avoided or used cautiously in obstructive HCM as they may worsen symptoms 1, 2
• Diuretics should be used cautiously in HCM, particularly in obstructive forms, as excessive preload reduction can worsen obstruction 1
• Medical therapy alone is not absolutely protective against sudden cardiac death in HCM, highlighting the importance of appropriate risk stratification for ICD therapy 4
• Verapamil can cause severe hemodynamic deterioration in patients with severe obstruction or systemic hypotension 3