Medication
For patients with reduced heart function (low ejection fraction), guideline-directed therapy for heart failure is recommended. Medications come from four different categories: beta-blockers, afterload reducers (e.g., ACE/ARB/ARNi; commonly losartan or sacubitril-valsartan), aldosterone inhibitors, and SGLP2 inhibitors (e.g., empagliflozin). These medications aim to improve symptoms and have been shown to extend life expectancy.
Some types of cardiomyopathy have specific therapy aimed at reducing inflammation (immunosuppression in sarcoid), infiltration (iron-chelation in hemochromatosis or tafamidis in cardiac amyloid), or obstruction/fibrosis (mavacamten in hypertrophic cardiomyopathy).
Structural/Surgical Intervention:
In patients with ischemic cardiomyopathy, a type of dilated cardiomyopathy, physicians may recommend stenting a heart artery to improve blood flow to the heart muscle if there was a significant blockage.
In patients with valvular cardiomyopathy, replacement or repair of a heart valve if the valve structure/function may be recommended.
In patients with hypertrophic cardiomyopathy, surgical or alcohol myectomy may be performed to remove excess muscle blocking blood outflow.
In patients with severe symptoms and low heart function, ventricular assist devices (VAD), an electromechanical device for assisting cardiac circulation, may be implanted. In worst-case scenarios, physicians may recommend heart transplantation.
Implantable Cardioverter Defibrillator (ICD)
An implantable cardioverter defibrillator (ICD) may be recommended to protect patients from sudden cardiac arrest and death. Patients with some forms of cardiomyopathy or decreased heart function below 35% (low ejection fraction) have an increased risk of ventricular fibrillation or ventricular tachycardia. Ventricular fibrillation and tachycardia are rhythms that originate from areas of inflammation or scarring. The heart rates are often over 200 beats per minute (BPM) and do not maintain blood pressure. Defibrillation with an ICD is highly effective at restoring normal rhythm.
In about 25% of patients with heart failure and decreased ejection fraction, there is evidence of conduction block, which occurs when there is a problem with the electrical signals that control the beating of your heart. Cardiac resynchronization therapy (CRT) uses an additional pacemaker wire to coordinate the activation of each heartbeat. This can improve the timing of contraction in the heart muscle, alleviate symptoms, and extend life expectancy. CRT therapy can be coupled with an ICD (implantable cardiac resynchronization therapy device or CRT-D) or a standalone pacemaker (implantable cardiac resynchronization therapy pacemaker or CRT-P).
Catheter Ablation
Catheter ablation of abnormal heart rhythms may be recommended to treat cardiomyopathies. Patients with cardiomyopathy often present with atrial fibrillation, atrial flutter, atrial tachycardia, ventricular tachycardia, and premature ventricular complexes (PVCs). These rhythms can be treated effectively with catheter ablation and/or rhythm medication.
Shared Decision-Making
Shared decision-making is an important component of treatment. All recommendations, including device implantation, should be made with your wishes, beliefs, and expectations considered in addition to the risks and benefits.
