Inflammatory cardiomyopathy and myocarditis are considered acquired forms of dilated cardiomyopathy. Whereas consensus documents on the diagnosis of myocarditis and perimyocarditis do exist, guidelines on the specific treatment have been established only for the management of pericardial diseases, which at least partly can be applied in analogy to myocarditis. Presently, feasible clinical pathways are available, which can lead to a correct diagnosis and specific treatment. This is illustrated with two cases of fulminant myocarditis, in one with successful diagnosis and treatment of a cardiac sarcoid and another one in which diagnostic nihilism led to a lethal outcome in giant cell myocarditis at necropsy. A case of active parvo B19-positive myocarditis demonstrates the role of immunoglobulin treatment under these conditions.

We monitored reverse left ventricular (LV) remodeling and LV function during the first 6 months of cardiac resynchronization therapy (CRT) in 34 patients (mean age = 55.3 +/- 13.6 years, 28 men) with dilated cardiomyopathy (DCM), left bundle branch block, in stable New York Heart Association class III, and on fixed drug regimen who underwent implantation of CRT systems with or without cardioverter defibrillator back-up. QRS-complex duration was reduced from 169.69 +/- 19.6 ms (SD) to 144.1 +/- 23.4 ms during CRT. Parasternal M-mode and apical 2D-echocardiography was performed before and 3 and 6 months after device implantation. LV enddiastolic (EDD) and endsystolic (ESD) diameters were measured, and biplane LV enddiastolic (EDV), and endsystolic (ESV) volumes and ejection fractions (EF) were calculated using a modified Simpson formula. Significant decreases in LVEDD (P = 0.0064 at 3 months and P = 0.021 at 6 months), LVESD (P = 0.023 at 3 months, and P = 0.003 at 6 months), and LVESV (P = 0.006 resp. P = 0.007), and increases in LVEF (P = 0.003 at 3 months and P < 0.001 at 6 months) were observed. Mean LVEF increased from 23% at baseline to 39% at 6 months. CRT induced prominent reverse LV remodeling and significantly increased LVEF within a few months in patients with DCM.

In the European Study on Epidemiology and Treatment of Cardiac Inflammatory Disease (ESETCID) pts with autoreactive (virus-negative) myocarditis (AM) and an ejection fraction <45% were randomised for 6 months of treatment with azathioprin (2mg/kg BW/day for 1 m and 0.85mg/kg BW/day for 5 m) + prednisolone (1.25mg/kg BW/ for 1 m and 0.3mg/kg BW/day for 5 m) or placebo on top of their heart failure treatment and followed-up for up to 8 years. Patients: 3149 pts with dilated cardiomyopathy were screened, 103 pts (mean age 47± 9 years, 81 male, 22 female) with AM and an EF 14 infiltrating cells/mm 2 ), persistence of viral or bacterial genomes for Parvo B19, coxsackie-, influenza-, adeno-, cytomegalo-, HHV 6, EBV, chlamydia and borrelia were excluded from the analysis. 56 pts (45 m) with AM were treated with verum, 47 pts ( 38m) with placebo. MACE are defined as cardiac death, heart transplantation, ICD implantation or hospitalisation for cardiac decompensation. Time to MACE is given in days to the event. Results: Inflammation was eradicated in 63% in the treatment group, but it also vanished spontaneously in 40% in the placebo arm(p<0,05). After 12 months the Kaplan Mayer MACE curves began to diverge. At 4 years freedom from MACE was 50% in the verum and 40% in the placebo group. Respective data at 8 years were 40% and 20% freedom from MACE. NYHA-association class and Minnesota Heart Failure Score improved in treatment and placebo arms to a similar extend. Ejection fraction by echo and radioventriculography improved in both arms with a trend for immunosuppression(IS). Independent from IS pts with no inflammation in the follow-up biopsy (n=45) showed a better NYHA-class (p<0,05), ejection fraction and superior long term freedom from MACE than those with persistent inflammation.. Conclusion: Immunosuppression is superior to conventional heart failure treatment in the acute eradication of the inflammatory infiltrate iDCM patients and bears better long-term prognosis. Results

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