An 11-year-old female adolescent who was previously healthy was admitted to the emergency department presenting severe tachypnea and dyspnea, pale skin and mucosa, tachycardia, slightly muffled heart sounds, mild mitral regurgitation murmur, normal blood pressure, and hepatomegaly.
The chest X-ray (see Figure 1) showed significant cardiomegaly, the electrocardiogram (ECG) was normal, and Doppler echocardiography (see Figure 1) showed large pericardial effusion with signs of cardiac tamponade, cardiac chambers of normal size, mitral and aortic valves with normal morphology, mild mitral insufficiency, and no vegetation in the valves.
Pericardiocentesis was performed and 1,200ml of exudate was drained; culture of this fluid was negative for fungi, bacteria, and mycobacterium. Blood cultures were negative. Serology tests for HIV, cytomegalovirus (CMV), rubella, toxoplasmosis, and mononucleosis were also negative. Clinical and laboratory investigations for tuberculosis, neoplasm, and collagen diseases were negative. The laboratory tests revealed C-reactive protein (CPR) of 105.1mg/dl, antistreptolysin O (ASO) of 200IU/ml, and an erythrocyte sedimentation rate (ESR) of 55mm/hour.
The patient was initially treated with antibiotics (oxacillin and gentamicin). Fever persisted even after 12 days of antibiotic therapy, and more intense mitral regurgitation murmur, onset of aortic regurgitation murmur, and worsening of heart failure signs were observed. Doppler echocardiography showed mild pericardial effusion, severe mitral insufficiency, mild to moderate aortic insufficiency, and enlarged left cardiac chambers. CRP was 82.6mg/l, ASO 600IU/ml, and ESR 45mm/hour, and the PR interval was prolonged on the ECG.
Treatment for heart failure was intensified and the patient was initiated on prednisone (50mg per day). She presented a marked improvement in general status and was afebrile within less than 72 hours, and CRP and ESR normalized. The patient was discharged after one week with the following prescription: penicillin G benzathine (PGB) to be taken every 21 days, prednisone, captopril, and furosemide.
At the follow-up visit she was asymptomatic with good general status, presenting moderate mitral regurgitation murmur, and medications were withdrawn except for PGB. Tests performed after 45 days using prednisone showed normal CRP and ESR and ASO of 400IU/ml, and Doppler echocardiography showed moderate enlargement of the left cardiac chambers, thickened mitral and aortic valves, moderate to severe mitral insufficiency, and mild aortic insufficiency.
Figure 2 shows the mitral valve with thickened leaflets, moderate mitral insufficiency, and mild aortic insufficiency on Doppler echocardiography performed after two years.
Discussion
The case reported met the Jones criteria for diagnosis of acute rheumatic fever (ARF)1 due to the following manifestations: carditis, fever, increased ESR and CRP, prolonged PR interval on ECG, and proven recent streptococcal infection. The diagnosis of ARF was made late, and it was defined only upon intensification of mitral insufficiency murmur, onset of aortic insufficiency murmur, maintenance of fever, progressive worsening of valvitis, elevated CRP and ERS, and ASO titers. The delayed diagnosis of ARF with acute pericarditis, with large pericardial effusion, signs of cardiac tamponade, and drainage of 1,200ml of exudate, was based on the rare presentation.
There are few reports in the literature of ARF associated with pericarditis with large pericardial effusion requiring pericardiocentesis.2,3 In rheumatic carditis, the isolated involvement of the pericardium is not described and, if there is no valvitis, other etiologies of pericarditis must be investigated.4,5
Pericarditis is the less common finding in rheumatic carditis.6–8 Pericarditis is more often diagnosed in necropsy and on Doppler echocardiography than clinically, since it usually presents as mild pericardial effusion with no clinical signs.9,10 Pericarditis in rheumatic carditis classically presents a predominantly fibrinous and sterile exudate, described as ‘bread and butter’ pericarditis, which often organizes itself leaving no important functional sequelae. In ARF, pericarditis is generally associated with severe carditis and resolution of pericarditis occurs with an anti-inflammatory treatment.2
In contrast to the habitual picture, pericarditis manifested early with cardiac tamponade and was similar to bacterial pericarditis, but the pericardial fluid drained was not purulent and the patient had a significant improvement after being treated with prednisone. The literature contains only two documented cases of cardiac tamponade related to ARF.9,10
The diagnosis of ARF must be considered among the possible etiologies of pericarditis with pericardial effusion, even if significant and with cardiac tamponade, particularly in children and adolescents between five and 15 years of age living in countries with a high prevalence of rheumatic fever and with mitral and aortic insufficiency murmur. Valvitis may present few or no clinical signs and may be underdiagnosed at the initial phase.