Circulation, Vol 88, 2198-2205, Copyright © 1993 by American Heart Association
J Narula, P Chopra, KK Talwar, KS Reddy, RS Vasan, R Tandon, ML Bhatia and JF Southern
BACKGROUND. Carditis is the only component of rheumatic fever that leads to
permanent disability. The diagnosis of carditis is presently made by using
composite clinical criteria based on the revised Jones' criteria. Since
myocardial involvement is an important component of rheumatic carditis,
right ventricular endomyocardial biopsies were performed in 54 patients
with clinical acute rheumatic fever and quiescent rheumatic heart disease
to evaluate the role of biopsy for the diagnosis of rheumatic carditis.
METHODS AND RESULTS. In 11 of the 54 patients, clinical consensus was
certain about rheumatic fever and carditis based on the revised Jones'
criteria (group 1). Histomorphological abnormalities in these patients were
scarce. The diagnostic features of rheumatic myocarditis including Aschoff
nodules or histiocytic aggregates were encountered in 3 patients (27%).
Lymphocytic infiltration was sparse. A majority of patients demonstrated
myocyte degeneration, interstitial degeneration, or occasional interstitial
mononuclear cell infiltration, but since these histopathological lesions
may occur in other conditions also, they were considered nondiagnostic. In
33 of the 54 patients with preexisting rheumatic heart disease, the
diagnosis of carditis was suspected based on varied clinical presentations.
Since previous cardiac findings were not available in these patients, the
clinical diagnosis of carditis could not be made without equivocation
(group 2). Twenty-three patients presented with unexplained acute onset of
congestive heart failure and evidence of recent streptococcal infection
(group 2A). While 13 of them had one or more other major manifestations, 10
patients had only minor manifestations. Mimetic carditis was suspected in
the remaining 10 of 33 patients based on carditis having occurred in
previous episodes of rheumatic fever (group 2B). The endomyocardial biopsy
provided confirmatory evidence of rheumatic myocarditis in 9 patients of
group 2A but in none of the 10 patients with suspected mimetic carditis.
Nondiagnostic myocyte or interstitial alterations were frequently observed
in group 2. Ten of the 54 patients had no clinical evidence of active
carditis (group 3). No histological alterations diagnostic of rheumatic
carditis were noted in these patients. Twenty-two follow-up biopsies were
performed in the first 10 consecutive patients. Diagnostic histiocytic
aggregates or Aschoff nodules were observed in initial biopsies in 4 of 10
patients, and nonspecific myocyte or interstitial alterations were observed
in 9. All patients with diagnostic changes in initial biopsy demonstrated
fibrohistiocytic nodules in 6- or 12-week biopsy samples. Nondiagnostic
alterations, similar to those seen in acute cases, were present in 5 of 8
patients at 6 weeks, 5 of 8 patients at 12 weeks, and 3 of the 6 patients
at 24 weeks despite the presumed adequate immunosuppressive therapy. No
complications related to biopsy were encountered. CONCLUSIONS. The present
study highlights the low frequency of diagnostic features in the biopsy
specimens of patients with definite clinical rheumatic carditis. Although
such alterations are not observed in patients with chronic rheumatic heart
disease, endomyocardial biopsy does not appear to provide additional
diagnostic information where clinical consensus is certain about diagnosis
of rheumatic carditis. Our study, however, substantiates the concept of
carditis underlying unexplained congestive heart failure of acute onset in
patients with preexisting rheumatic heart disease and elevated
antistreptolysin-O titers.
ARTICLES
Does endomyocardial biopsy aid in the diagnosis of active rheumatic carditis?
All India Institute of Medical Sciences, Delhi.
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