Am Fam Physician. 1998 Sep 1;58(3):660-662.
to the editor: Sarcoidosis is a multisystem granulomatous disorder. Symptomatic cardiac involvement is present in up to 5 percent of patients with sarcoidosis; postmortem studies reveal cardiac involvement in 20 to 30 percent of cases. While small effusions occur in 19 percent of cases, massive pericardial effusion leading to cardiac tamponade secondary to sarcoidosis is exceedingly rare and only a few cases have been reported.1,2 We report here a patient with sarcoidosis who presented with cardiac tamponade.
A 30-year-old black man was admitted with complaints of nonproductive cough and a 9.08-kg (20-lb) weight loss over the past two months. He was treated with oral antibiotics on two different occasions with no relief of symptoms. A previous admission one year earlier for transbronchial biopsy revealed a diagnosis of sarcoidosis. History was negative for medication use, tuberculosis exposure, smoking, alcoholism or drug abuse. Physical examination revealed a well-developed man in mild respiratory distress with a blood pressure of 100/60 mm Hg, a pulse rate of 96 beats per minute, a temperature of 38.1°C (100.6°F) and a respiratory rate of 22 breaths per minute. There was significant pulsus paradoxus of 20 mm Hg and obvious jugular venous distention.
The pulmonary examination revealed bilateral vesicular breath sounds. Heart sounds were distant with a three-component pericardial rub over the left parasternal area. Radiographic examination revealed mild cardiomegaly with clear lung fields. The electrocardiogram showed normal sinus rhythm. Routine laboratory parameters were within normal limits.
Transthoracic echocardiogram revealed a large pericardial effusion with early diastolic collapse of the right ventricle free wall suggestive of impending tamponade. During urgent pericardiocentesis, 600 mL of fluid was drained, and pericardial biopsy revealed fibrinous pericarditis. The pericardial fluid was exudative, and negative for acid fast bacilli and malignancy. The patient was started on treatment with prednisone, 60 mg per day orally, which was slowly tapered over the next few months. The patient showed a significant response to the treatment. Subsequent repeat echocardiograms over the next nine months did not show any pericardial effusion, and the patient has remained asymptomatic.
The clinical features of sarcoid heart disease include congestive heart failure, ventricular arrhythmia, conduction disturbances, ventricular aneurysms, pericardial effusion and sudden death, particularly in younger patients.1,3–5 Arrhythmias are the most predictive indicators of cardiac involvement.6 Echocardiography is being increasingly used in patients with sarcoidosis to establish the cardiac involvement echo. Features include focal wall abnormalities, mitral valve prolapse, pericardial effusions, increased septal thickness, aneurysmal dilatation and atypical nonischemic ventricular wall motion abnormalities.
Massive effusions are rare, with only 10 such cases found during review of the medical literature, and they are exudative in nature. Effusion may be secondary to systemic or pericardial disease, cor pulmonale, pericardial granuloma or left ventricular dysfunction.4,5 A patchy distribution may lead to negative pericardial biopsies.4 Fortunately, the previous documentation of pulmonary involvement helped to establish the diagnosis. Treatment of cardiac sarcoidosis is still evolving. Steroids have been the mainstay of medical treatment including pericardial effusion. Refractory arrhythmias or heart block may necessitate surgical or pacemaker placement.
REFERENCESshow all references
1. Verkleeren JL, Glover MU, Bloor C, Joswig BC. Cardiac tamponade secondary to sarcoidosis. Am Heart J. 1983;106:601–3....
2. Israel RH, Poe RH. Massive pericardial effusion in sarcoidosis. Respiration. 1994;61:176–80.
3. Bower SP, Thomson A. Two dimensional echocardiography and left ventriculography in cardiac sarcoidosis. Aust N Z J Med. 1989;19:724–6.
4. Kinney E, Murthy R, Ascunce G, Donohoe R, Zelis R. Pericardial effusions in sarcoidosis. Chest. 1979;76:476–8.
5. Pesola G, Teirstein AS, Goldman M. Sarcoidosis presenting with pericardial effusion. Sarcoidosis. 1987;4:42–4.
6. Silverman KJ, Hutchins GM, Bulkley BH. Cardiac sarcoid: a clinicopathologic study of 84 unselected patients with systemic sarcoidosis. Circulation. 1978;58:1204–11.
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