The only causal (cause-related) therapy for cardiomyopathies is heart transplantation (abbreviated HTX; English heart transplantation).
Dilated (dilated) cardiomyopathy (DCM)
- In terminal heart failure: temporary mechanical heart replacement (left ventricular assist device (LVAD)) – For further details, see “Heart failure (heart failure)/Operative therapy“.
- Heart transplantation (Ultima ratio).
Hypertrophic (enlarged) cardiomyopathy (HCM)
- Transaortic subvalvular myectomy (TSM): excess muscle tissue (= myectomy) in the outflow tract of the left ventricle (heart chamber) is removed through the aortic valve.
- The success rate is >90%, and the lethality (mortality based on the total number of people with the disease) is <2%
- Indications:
- Failure of medical therapy
- Ineffective DDD pacing or ablation (surgical ablation of the myocardial tissue responsible for the disorder).
- Probably improve the prognosis
- Percutaneous alcohol septal ablation (percutaneous transluminal septal myocardial ablation, PTSMA; Engl. Transcoronary Ablation of Septal Hypertrophy; synonym: transcoronary ablation of septal hypertrophy; selection of the coronary branch by cardiac catheter, supplying the hypertrophied area and placing an artificial infarct using alcohol (100% ethanol) in the area of hypertrophy): the procedure has achieved comparable results to myectomy for over 20 years.
- Indications: see below TSM
- Possible complications: Atrioventricular block (impaired transmission of impulses between the atria and ventricles, causing arrhythmias), ventricular septal defect (hole in the ventricular septum), and residual obstructionNote: In 10-20% of patients, subsequent implantation of a pacemaker is required after PTSMA because of AV block.
- Heart transplantation
- Indication: dilated progression (the severity of heart failure (cardiac insufficiency)) increases; NYHA stages III and IV.
Note: TSM and PTSMA procedures have not been compared in randomized controlled trials to date.
Restrictive (limited) cardiomyopathy (RCM)
- Heart transplantation
- Indication: for terminal heart failure.
Arrhythmogenic right ventricular cardiomyopathy (ARVCM)
- Heart transplantation
- Indication: for right heart failure
Isolated (ventricular) noncompaction cardiomyopathy (NCCM)
- Cardiac transplantation, if necessary
Other notes
- Severe ischemic cardiomyopathy (ICM): restoration of cardiac function by ventricular and/or mitral valve reconstruction without heart transplantation (HTX) or implantation of a left ventricular assist device (LVAD): median survival of 102 patients at 8 years was 64.3% with an “in-hospital” mortality of 2.9% (n = 3).
