CardiomyopathyComparison of Survival Among Patients With Connective Tissue Disease and Cardiomyopathy (Systemic Sclerosis, Systemic Lupus Erythematosus, and Undifferentiated Disease)
Section snippets
Selection of patients
We evaluated 1,700 patients with unexplained cardiomyopathy who underwent endomyocardial biopsy (EMB) from 1983 to 2003 at The Johns Hopkins Hospital. Most of these patients were referred to us by other physicians, and some common causes of heart failure, such as ischemic heart disease, hypertension, and valvular heart disease, were likely underrepresented. At the time of referral to The Johns Hopkins Hospital for the initial evaluation, the causes of patients’ heart failure were unknown.
Clinical and hemodynamic characteristics of the study population
Specific diagnoses for all patients with CTD and cardiomyopathy are listed in Table 1. The 3 most common categories in descending order of frequency were undifferentiated CTD, SLE, and SS. The clinical and hemodynamic characteristics of patients with CTD and those with idiopathic DCM are listed in Table 2. Most patients with CTD were middle aged, white women, with a similar degree of hemodynamic compromise (Table 2). Patients with SLE and cardiomyopathy had significantly higher rates of
Discussion
Our study examined the prognosis of patients with cardiomyopathy and CTD. We explored, for the first time, long-term survival in patients with cardiomyopathy associated with SS, SLE, undifferentiated CTD, or CTD and myocarditis. The finding of a poor outcome in patients with SS relative to those with idiopathic DCM is consistent with previous reports showing a grave prognosis in subjects with SS and clinically significant cardiac involvement12, 17 and may indicate that patients with SS and
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