ABSTRACT
Three quarters of a century have elapsed since the first description of cardiac
sarcoidosis recorded by Bernstein et al. (1) in 1929. Advances in technology and therapeutic interventions have increased our ability to diagnose and treat
this most serious complication. Yet antemortem diagnosis still remains elu-
sive in some patients and, in others, documentation is difficult evenwhen they
are suspected of having cardiac involvement. The generally good prognosis
and low mortality of sarcoidosis are adversely impacted in patients suffering
from this complication. Sarcoidosis, a multisystemic, worldwide, granuloma-
tous disease of unknown etiology occurring worldwide has a variable preva-
lence of less than 20 to 213/100,000 depending on the ethnicity, race, and geography of the population studied (2-4). The prevalence based on autopsy
studies, in general and selected populations, varies from 38.8 to 640/100.000
(5-9). The incidence of cardiac sarcoidosis is unknown. In the recent ‘‘ACase
Control Etiologic Study of Sarcoidosis’’ (ACCESS) report, the incidence of
cardiac involvement in a large group of patients studied within six months
of tissue diagnosis was 2.3% (10). Cardiac involvement in this disease, albeit uncommon, is of major significance. It is the most frequent cause of death
from sarcoidosis in Japanwhere females older than 40 years are disproportionately affected (5,11-13). Perry and Vuitch noted similar findings in their
autopsy study in the United States (14). Worldwide, this gender predilection is not seen. The age of those patients with sarcoidosis dying of cardiac invol-
vement ismostly in the fourth and fifth decade of life although the group dying
suddenly or discovered to have sarcoidosis at time of autopsy tends to be
younger. Children can present with or be affected by thismanifestation of sar-
coidosis (14-16). Cardiac involvement is the second most common cause of
death in patients with sarcoidosis (17,18). Clinically manifested cardiac dis-
ease occurs in approximately 5% of patients with sarcoidosis (12,19). A significant number of cases of myocardial sarcoidosis are discovered at the time of autopsy and are never suspected antemortem (14,20,21). A high index of
suspicion is warranted to increase our ability to establish the diagnosis of car-
diac involvement in a sarcoidosis patient. Cardiac involvement, when sus-
pected, is treated even in the absence of well-documented granulomatous
inflammation of the heart.
