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[Therapy of dilated cardiomyopathies with and without inflammation]
G Hufnagel1, S Pankuweit, B Maisch
1Abteilung Innere Medizin-Schwerpunkt Kardiologie, Philipps-Universität Marburg. hufnagel@mailer.uni-marburg.de
Insights
Diagnosing inflammatory dilated cardiomyopathy requires endomyocardial biopsies to guide specific therapies. While some treatments improve outcomes, prognosis remains poor for symptomatic cases, necessitating advanced interventions like heart transplantation.
Area of Science:
- Cardiology
- Immunology
- Virology
Context:
- Inflammatory dilated cardiomyopathy diagnosis relies on endomyocardial biopsies.
- Identifying etiological agents is crucial for targeted therapy.
- Prognosis for symptomatic cases is poor, with low complete restitution and 10-year survival rates.
Purpose:
- To review diagnostic and therapeutic strategies for inflammatory dilated cardiomyopathy.
- To highlight the importance of etiological agent identification for specific treatment.
- To discuss the prognostic implications and current treatment outcomes.
Summary:
- Histological and immunohistological examination of endomyocardial biopsies are key for diagnosing inflammatory dilated cardiomyopathy.
- Therapeutic approaches include physical activity restriction, symptomatic medical therapy (digitalis, diuretics, ACE-inhibitors, vasodilators, beta-blockers), and specific treatments based on etiological agents.
- Immunosuppressive therapy shows promise in autoimmune forms, while ongoing trials explore immunomodulation and antiviral therapies for viral myocarditis.
Impact:
- Accurate diagnosis and etiological identification enable personalized therapy, potentially improving patient outcomes.
- Current therapeutic strategies offer some benefit, but advanced interventions like cardiac assist devices or heart transplantation may be necessary for end-stage disease.
- Further research and clinical trials are essential to refine treatment protocols and enhance survival rates for inflammatory dilated cardiomyopathy.
Abstract:
Diagnosis of inflammatory dilated cardiomyopathy relies on the histological and immunohistological examination of endomyocardial biopsies. Only with the demonstration of the etiological agents in the myocardium specific therapy can be attempted. Whereas the spontaneous course of endemic myocarditis with little hemodynamic impairment is fair, the prognosis of symptomatic myocarditis and dilated cardiomyopathy is poor, with complete restitution in 35% and a 10-year survival rate of 30%. Restriction of physical activity is a validated form of therapy with normalization of the heart size in 40 to 60%. Symptomatic medical therapy consists of digitalis, diuretics, ACE-inhibitors and vasodilators and betablocker therapy, where a reduction of mortality was demonstrated in clinical (sub)studies up to 60%. Specific forms of therapy in inflammatory cardiomyopathy rely on the demonstration or lack of viral persistence or signs of autoreactivity in the myocardial tissue. Immunosuppressive therapy in autoimmune forms improved cardiac function in up to 60% of the patients in controlled trials, when compared to controls (40%). The double-blind randomized myocarditis treatment trial, which unfortunately did not distinguish viral from autoimmune myocarditis could not demonstrate such a benefit, however. Depending on the etiology of the disease, immunomodulation with immunoglobulins or interferon or antiviral therapy with hyperimmunoglobulins are presently tested in clinical treatment trials (ESETCID) in patients with enterovirus-positive or cytomegalovirus-positive and adenovirus-positive chronic myocarditis. Specific therapies are aimed to avoid the progression of the disease which may ultimately lead to heart failure with a cardiac assist device or heart transplantation as ultimate therapeutic option.