Sarcoidosis is a multisystem granulomatous disease of unknown etiology. Symptomatic cardiac involvement is present in up to 5% of patients, mostly as heart blocks.1 Massive pericardial effusion leading to cardiac tamponade is extremely rare. Only 2 cases of cardiac tamponade as a presenting symptom of sarcoidosis were reported in the literature.2,3.
A 32 year old African American obese male presented to the hospital with insidious onset of dyspnea over 6 week period. He denied fever, chills, cough, hemoptysis, weight loss. Past medical, social and family history was non-contributory. There was no tuberculosis exposure. His PPD was negative. Physical examination revealed a well-developed man in mild respiratory distress with blood pressure of 105/60. He was afebrile with respiratory rate of 24 breaths per minute. Jugular venous distention, distant heart sounds and pulsus paradoxus were present. EKG showed electrical alternans. CBC and chemistry were normal. Chest X-ray revealed massive cardiomegaly with mediastinal adenopathy (Figure 1). Transthoracic echocardiogram was positive for massive pericardial effusion with right ventricular and left atrial collapse in diastole. Pericardiocentesis drained 1500 cc of bloody exudative fluid. CT-guided pericardial biopsy was negative for AFB and malignancy. Biopsy of mediastinal lymph nodes by anterior mediastinoscopy showed granulomatous disease and ACE level was 63. Extensive work up with AFB cultures and multiple viral, fungal and HIV serologies remained negative. Patient remained asymptomatic over next 14 months without any treatment. He then presented with a month long gradual onset of dyspnea, cough and blood-tinged sputum. He denied fever, chills or weight loss. He was afebrile with normal physical exam. Chest X-ray and CT scan showed extensive right lung infiltrate with enlarged mediastinal lymph nodes (Figure 2). ACE level was 101. Bronchoscopic transbronchial biopsy revealed multiple non-caseating granulomas. Patient was started on Prednisone 80 mg daily with complete resolution of symptoms and radiological findings as well as a drop of ACE level to 29 after 2 months.
The usual clinical features of cardiac sarcoidosis, in order of decreasing frequency, are complete heart block, ventricular irritability, congestive heart failure, sudden death, first-degree heart block or bundle branch block, supraventricular arrhythmias, mitral valve dysfunction and pericarditis.1 Prevalence of asymptomatic small pericardial effusion in diagnosed cases of sarcoidosis is 19% to 21% detected by echocardiography.4 Sarcoidosis involves the inflammation of both visceral and parietal pericardium, resulting in gross thickening of the pericardium and accumulation of pericardial fluid.1 Pathologically, tiny nodules may be seen in a patchy distribution which may explain the presence of a negative pericardial biopsy in our patient.Massive effusions are rare, with only about 10 cases found in the literature.1-3 Effusion may be secondary to systemic or pericardial disease, cor pulmonale, pericardial granuloma or left ventricular dysfunction.2 The previous documentation of pulmonary involvement helps to establish the diagnosis. Steroids have been the mainstay of medical treatment of sarcoidosis, including pericardial effusions, but it is difficult to assess its role in treating pericardial effusions. Most patients with cardiac tamponade due to sarcoidosis were placed on steroids but the control of their pericardial effusion required surgical intervention.1-3.
Cardiac tamponade is a rare manifestation of sarcoidosis. Our case stands out since it is the third documented case in literature of cardiac tamponade as the presenting feature of sarcoidosis.
Dmitry Lvovsky, None.