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CLINICAL STUDY: PRIMARY MYOCARDIAL DISEASE

A clinical and histopathologic comparison of cardiac sarcoidosis and idiopathic giant cell myocarditis

Yuji Okura, MD, PhD^*, G. William Dec, MD, FACC, Joshua M. Hare, MD, FACC^||, Makoto Kodama, MD^¶, Gerald J. Berry, MD^#, Henry D. Tazelaar, MD, Kent R. Bailey, PhD and Leslie T. Cooper, MD, FACC^*,*

^* Division of Cardiovascular Diseases, Mayo Clinic and Foundation, Rochester, Minnesota, USA
Division of Anatomic Pathology, Mayo Clinic and Foundation, Rochester, Minnesota, USA
Department of Biostatistics, Mayo Clinic and Foundation, Rochester, Minnesota, USA
Cardiovascular Division, Massachusetts General Hospital, Boston, Massachusetts, USA
^|| Cardiovascular Division, Johns Hopkins Hospital, Baltimore, Maryland, USA
^¶ First Department of Internal Medicine, Niigata University School of Medicine, Niigata, Japan
^# Department of Pathology, Stanford University Medical Center, Stanford, California, USA

Manuscript received March 26, 2002; revised manuscript received August 30, 2002, accepted October 14, 2002.

^* Reprint requests and correspondence: Dr. Leslie T. Cooper, Jr., Cardiovascular Division/Mayo East 16B, Mayo Clinic, Rochester, Minnesota 55905, USA.
cooper.leslie{at}mayo.edu

OBJECTIVES: The goal of this study was to determine the prognostic value of clinical data available at presentation and histology in cardiac sarcoidosis (CS) and idiopathic giant cell myocarditis (IGCM).

BACKGROUND: The prognosis of patients with nonischemic cardiomyopathy is partly dependent on the histologic diagnosis. Survival in IGCM is poor. The prognosis of a histologically related entity, cardiac sarcoidosis (CS), is less well established, and the prognostic value of the distinction between CS and IGCM on endomyocardial biopsy (EMB) is unknown.

METHODS: We identified 115 patients from the Multicenter IGCM Registry with CS (n = 42) and IGCM (n = 73). We compared the clinical data for these two groups using Cox proportional-hazards models to assess the association between histologic diagnosis and survival. In order to determine whether histologic features could reliably differentiate these two entities, two cardiac pathologists semiquantitatively graded the inflammatory infiltrate components and compared the results between groups.

RESULTS: Black race was more frequent in the CS group (31% vs. 4%, p < 0.0001). Syncope and atrioventricular block were also more frequently observed in CS than IGCM (31% vs. 5%, p = 0.0002 and 50% vs. 15%, p < 0.0001, respectively). Left-sided heart failure was more common in IGCM (40% vs. 64%, p = 0.013). In CS patients diagnosed by EMB, the five-year transplant-free survival after diagnosis was 69.8% versus 21.9% for IGCM (p < 0.0001, log-rank test). In multivariate models, presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS. Eosinophils, myocyte damage, and foci of lymphocytic myocarditis were more frequent in IGCM, while granulomas and fibrosis were more frequent in CS.

CONCLUSIONS: Transplant-free survival is better for patients with CS than for IGCM diagnosed by EMB. Presentation with heart failure predicted IGCM, and presentation with heart block or more than nine weeks of symptoms predicted CS.

Abbreviations and Acronyms   CHF   congestive heart failure   CI   confidence interval   CS   cardiac sarcoidosis   EMB   endomyocardial biopsy   IGCM   idiopathic giant cell myocarditis   JHH   Johns Hopkins Hospital   MCR   Mayo Clinic Rochester   MGH   Massachusetts General Hospital   NUH   Niigata University Hospital   SUMC   Stanford University Medical Center

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