Therapeutic Targets
- Improvement of quality of life or expectancy.
- Avoidance of complications (eg, malignant arrhythmogenic events/life-threatening cardiac arrhythmias)).
Therapy recommendations
Dilated Cardiomyopathy (DCM)
This is an abnormal enlargement (dilation) of the heart muscle, especially the left ventricle (heart chamber). For therapy:
- Causal (cause-related) therapy:
- Cardiomyopathy caused by viruses can be treated with interferon (immunostimulation drug) (see myocarditis below).
- In autoimmune disease with proven auto-Ak against the beta1-adrenergic receptor in the myocardium (heart muscle):
- Immunadsorption (removal of auto-Ak) or.
- Neutralization of auto-Ak by aptamers (binding molecules).
- If necessary, immunosuppressive therapy
- In peripartum (“around birth”) cardiomyopathy: bromocriptine (so far only in studies).
- In the presence of heart failure (cardiac insufficiency): guideline-based therapy (see below heart failure* ).
- For thromboembolism prophylaxis (preventive measures to prevent thrombosis/vascular disease in which a blood clot (thrombus) forms in a vessel): Anticoagulants (drugs that inhibit blood clotting) in atrial fibrillation (VHF) or when intracavitary (located in a cavity) thrombi (blood clots) are detected.
* Note: A good response to therapy usually indicates “remission” only; after discontinuation of therapy, relapse occurred in 44% of cases (11 of 25 patients) within 6 months in one study:
- Decrease in LVEF by >10% and below 50%.
- LVEDV increase by > 10% and above normal value.
- NT-proBNP increase by a factor of two and to > 400 ng/l
- Clinical signs of heart failure (cardiac insufficiency).
In the control group, which continued to receive treatment, no deterioration was detectable in any patient based on the four criteria. Thereafter, the pharmacotherapy of the patients in the control group was also successively discontinued, with the result that in the following 6 months in 36% of these patients also deteriorated. Thus, in total, 20 patients (40%) out of 50 patients ended up with an objectifiable deterioration.
Hypertrophic (enlarged) cardiomyopathy (HCM)
- Drug therapy for heart failure (therapy for heart failure):
- Beta-blockers – Caution: calcium antagonists and beta-blockers must not be administered together because of the risk of AV block (atrioventricular block; disorders caused by conduction disturbances from the atrium to the ventricle)
- In case of beta-blocker intolerance: non-dihydropyridine-type calcium antagonists (e.g., verapamil, diltiazem).
- In atrial fibrillation (see below VHF): anticoagulants.
- Hypertrophic obstructive cardiomyopathy (HOCM).
- Mavacamten (myosin modulator): efficacy was demonstrated in a phase III EXPLORER-HCM trial (“breakthrough therapy”): mavacamten reduced hypercontractility with consequent:
- 65.0% of patients improved by at least one NYHA class compared with 31.3% with placebo (p ˂ 0.0001)
- Quality of life and exercise capacity increased substantially.
- Obstruction (obstruction) of the left ventricular outflow tract (LVOT) decreased significantly.
- Complete remission occurred in nearly one-third, i.e., no clinical, radiological, or other signs of the disease were detectable.
- The following drugs are contraindicated (contraindications):
- Inotropic substances (increase the contractile force of the heart) such as digitalis and sympathomimetics.
- Potent afterload lowering agents (to relieve the heart) such as ACE inhibitors.
- Nitrates (cause an increase in systolic stenosis (narrowing)).
- Mavacamten (myosin modulator): efficacy was demonstrated in a phase III EXPLORER-HCM trial (“breakthrough therapy”): mavacamten reduced hypercontractility with consequent:
Restrictive (limited) cardiomyopathy (RCM)
- Early therapy of heart failure (see there due toTherapy) with diuretics (draining agents); caveat: no digitalis!
- Thromboembolism prophylaxis
Arrhythmogenic right ventricular cardiomyopathy (ARVCM)
- Symptomatic therapy:
- Treatment of arrhythmias (cardiac arrhythmias) or prophylaxis.
- Beta blocker
Isolated (ventricular) noncompaction cardiomyopathy (NCCM)
- Therapy of heart failure (see there due toTherapy).
- Prophylaxis of thromboembolism: anticoagulants.
Pregnancy cardiomyopathy/peripartum cardiomyopathy
- Standard therapy as for heart failure
- 5 mg bromocriptine for 2 weeks, then 2.5 mg for 6 weeks: after therapy, 7% of all participants still had severe limitation of left ventricular function after six months (<35%) and only 3% subsequently suffered from heart failure
TTR-associated systemic amyloidosis (ATTR).
- In a phase III study (ATTR-ACT), tafamidis was shown to significantly reduce the risk of mortality in patients by 30%.
- 2020: The EU Commission approved Tafamidis 61 mg for the treatment of transthyretin amyloidosis with cardiomyopathy (ATTR-CM). The drug is indicated for the treatment of ATTR-CM in adults with both the wild-type and hereditary forms.
