INTRODUCTION: Cardiac tamponade due to sarcoidosis is rare. To our knowledge, there are only eleven case reports of sarcoid associated tamponade. The patients were 27-59 years old, nine were women and eight presented 2-60 months after being diagnosed with sarcoidosis. Only three patients had tamponade as the first presentation of their disease (1,2). We present a case of a young healthy woman who came to us in tamponade and was found to have sarcoidosis.
CASE PRESENTATION: A 30 year old African-American woman presented to the ER with severe dyspnea and tachycardia. Her symptoms were insidious and progressive over three months. The CXR showed an enlarged heart. 2-D echocardiogram revealed a large circumferential pericardial effusion with right-sided diastolic collapse. An emergent pericardial drain removed 800 cc of straw colored fluid with significant clinical improvement. Post procedure EF was 30%. An endomyocardial biopsy showed non-caseating granulomas suggesting sarcoidosis, mycobacterial or fungal infection. TB and fungal infection was ruled out by a non-reactive ppd, negative serum IGRA , negative AFB and fungal stain and cultures from the pericardial fluid, myocardial biopsy and sputum, negative PCR for TB from the pericardial fluid, negative serum galactomannan and beta-D-Glucan. A CT scan showed multiple perilymphatic lung nodules and hepatic and splenic lesions compatible with sarcoidosis. Prednisone was started and slowly tapered over 8 months. The patient had complete normalization of her cardiomyopathy, pericardial effusion and spleen and liver lesions. She is asymptomatic with only mild obstructive lung disease.
DISCUSSIONS: Only 5 percent of sarcoidosis patients have symptomatic cardiac disease, but in autopsy series 30 percent show cardiac involvement. Sudden cardiac death causes 25-65% of cardiac sarcoidosis related mortality and can be the first presentation. First-degree heart blocks and bundle branch blocks are common and can progress to complete heart block. Ventricular premature beats and ventricular tachycardia are seen in 22% . Granulomatous infiltration of the myocardium can cause systolic or diastolic heart failure and is responsible for 25 -75 percent deaths. Perfusion defects can be seen. Valve involvement can cause regurgitation defects. SVT's, aortic aneurysms, pericarditis and pericardial effusions are rare. The optimal diagnostic strategy for cardiac sarcoidosis is unclear due to variable presentation as well as the low sensitivity and specificity of diagnostic tests. These include endomyocardial biopsy, CXR, EKG, Holter monitoring, exercise stress test, echocardiography and nuclear imaging. Echocardiography may show hyperechogenicity in tissue with extensive granulomas or wall motion abnormalities not in the normal distribution of the coronary vessels. Myocardial radionuclide imaging may show perfusion defects that actually decrease in size with excercise. Nuclear imaging can be used to follow up patients. Steroids are the mainstay of treatment and decrease 5 year mortality from 90% to 25%. When steroids are weaned, relapse is common and is treated by restarting from high doses of prednisone. Alternative agents such as methotrexate are used in patients unresponsive to steroids. ICD/ pacemakers should be placed if indicated. Cardiac transplant can be done in advanced cases with good results. Recurrent sarcoidosis can occur in the transplant.
CONCLUSION: To our knowledge, our case is the fourth case of cardiac tamponade as initial presentation of sarcoidosis to be reported in literature. Sarcoidosis should be considered in any young or healthy patient with cardiac symptoms. Although most patient with cardiac sarcoidosis will die from it, early initiation of treatment leads to 89% 5-year survival (1). Since there is no established method for screening for sarcoidosis, physician awareness is of utmost importance.
DISCLOSURE: Basel Altaqi, No Financial Disclosure Information; No Product/Research Disclosure Information