LETTER TO THE EDITOR
Reply
Alida L. P. Caforio, MD, PhD, FESC*,*,
Niall G. Mahon, MD* and
William J. McKenna, MD, FRCP, FACC, FESC*
a Division of Cardiology, Department of Clinical and Experimental Medicine, Padua University Medical School, Policlinico Universitario, Centro "V. Gallucci", via N. Giustiniani, 2, 35128 Padua, Italy
* Reprint requests and correspondence: Dr. Alida L.P. Caforio, Division of Cardiology, Department of Clinical and Experimental Medicine, Padua University Medical School, Policlinico Universitario, Centro "V. Gallucci", via N. Giustiniani, 2, 35128 Padua, Italy.
alida.caforio{at}unipd.it
We thank Dr. Noutsias and colleagues for their comments regarding our report (1). Although dilated cardiomyopathy (DCM) is classified as idiopathic, familial/genetic, viral and/or immune (2), in the real world we lack consensus diagnostic criteria for viral and/or immune DCM. In relation to the key question, as to whether immunosuppression is indicated in DCM patients and their relatives with left ventricular enlargement (LVE) (2), we would suggest that the rational base is the establishment of accepted consensus diagnostic criteria. This should set the groundwork for future controlled studies of immunosuppressive therapy in DCM. We agree with Dr. Noutsias and co-workers that autoimmune DCM is defined by lack of viral genome by polymerase chain reaction (PCR) and myocardial inflammation by immunohistochemistry. Although the study by Wojnicz et al. (3) has shown beneficial hemodynamic effects of immunosuppression after two-years, it failed to show a favorable effect on mortality, possibly because it was statistically underpowered. Conversely, the IMAC trial failed to demonstrate efficacy in recent-onset DCM and myocarditis, but patients were not stratified in terms of pathogenesis (4). Thus, multicenter studies enrolling adequate numbers of patients using consensus criteria for viral versus immune inflammation are needed. Meanwhile, on the basis of the Polish study (3) a short course of immunosuppression may be considered in patients with established inflammatory DCM, with no replicating virus. In these patients the prognosis remains poor, and the potential benefit of halting disease progression is high.
It is, however, premature to administer immunosuppression for asymptomatic LVE relatives with myocardial inflammation. Although such therapy has the potential to prevent disease progression, the absolute risk of progression in LVE needs to be quantified. Data from an initial cohort demonstrated progression in 27% of subjects over three years (5), but longer follow-up in a larger cohort is required. Second, five-year follow-up has revealed that serum detection of cardiac-specific antibodies (6) with or without LVE at baseline is also a noninvasive predictor of disease progression (7). The clinical challenge is to identify more accurately, ideally with noninvasive markers, asymptomatic relatives at risk. We believe it is necessary to obtain such data before considering a potentially deleterious therapy such as immunosuppression in asymptomatic relatives with preserved systolic function.
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References
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- Mahon NG, Madden BP, Caforio ALP, et al. Immunohistologic evidence of myocardial disease in apparently healthy relatives of patients with dilated cardiomyopathy. J Am Coll Cardiol. 2002;39:455–462[Abstract/Free Full Text]
- Richardson P, McKenna WJ, Bristow M, et al. Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the definition and classification of cardiomyopathies. Circulation. 1996;93:841–842[Free Full Text]
- Wojnicz R, Nowalany-Kozielska E, Wojciechowska C, et al. Randomized, placebo-controlled study for immunosuppressive treatment of inflammatory dilated cardiomyopathy: two-year follow-up results. Circulation. 2001;104:39–45[Abstract/Free Full Text]
- McNamara DM, Holubkov R, Starling RC, et al. Controlled trial of intravenous immune globulin in recent-onset dilated cardiomyopathy. Circulation. 2001;103:2254–2259[Abstract/Free Full Text]
- Baig MK, Goldman JH, Caforio ALP, et al. Familial dilated cardiomyopathy: cardiac abnormalities are common in asymptomatic relatives and may represent early disease. J Am Coll Cardiol. 1998;31:195–201[Abstract/Free Full Text]
- Caforio ALP, Keeling PJ, Zachara E, et al. Evidence from family studies for autoimmunity in dilated cardiomyopathy. Lancet. 1994;344:773–777[CrossRef][Medline]
- Caforio ALP, Mahon NG, Baig MK, et al. Cardiac autoantibodies are independent predictors of progression to dilated cardiomyopathy in symptom-free relatives. (abstr)Eur Heart J. 2001;22(Suppl):637
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