Endomyocardial fibrosis

INTRODUCTION: Giant cell myocarditis (GCM) is a rare, rapidly fatal myocarditis with histologic features that have some similarities to cardiac sarcoidosis (CS). We report a case of cardiac saecoidosis which initially was diagnosed as GCM. Howevere after a carefull review of the histopathlogical feature of the endomyocardial biopsy, and the presence of pulmonary granulomas on the transbronchial biopsy the diagnosis confirmed as a cadiac sarcoiodosis.

CASE PRESENTATION: 38 Year-old African American male, with no known medical problems, was transferred from an outside hospital to our institution for further workup of his ventricular arrhythmias. He had a syncopal episode while walking to the TV in his living room. He lost consciousness for 5-6 minutes and woke up without confusion, but he was complaining of chest pain. On admission the patient was noted to have multiple arrhythmias including second degree AV Block, nonsustained ventricular tachycardia, and torsades. He was complaining of some chest discomfort, cough, dyspnea, and 10 lbs weight loss. His physical exam was remarkable for elevated JVD; third heart sound, systolic murmur over the apex, bibasilar end inspiratory crackles, and 2 + pitting edema over the lower extremities. The electrolytes were within normal limits. Urine drug screen was negative and his TSH as well. Arterial blood gas on room air showed moderate degree of hypoxemia. Chest Ct with PE protocol did not show pulmonary embolus, but it showed diffuse pulmonary nodules with hilar and mediastinal lymphadenopathy. He underwent a cardiac catheterization which showed normal coronaries and ejection fraction of 15%. He had an endomyocardial biopsy as well and initially showed Giant Cell Myocarditis. Subsequently he had a bronchoscopy with transbronchial biopsy showing non-caseating granulomas suggestive of sarcoidosis (see figure 1). In view of the lung biopsy results the pathologist reevaluated his endomyocardial biopsy (see figure 2)and due to presence of non-caseating granulomas on endomyocardial biopsy, he revised the diagnosis to cardiac sarcoidosis. Patient underwent a placement of a cardiac defibrilator for his arrhythmias. Prednisone of 60mg a day was given with a marked improvement of his heart failure symptoms.

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DISCUSSIONS: Idiopathic giant-cell myocarditis IGCM is a frequently fatal type of myocarditis. The clinical course, unlike that of sarcoid myocarditis CS, is usually characterized by progressive congestive heart failure, frequently associated with refractory ventricular arrhythmia. Most patients die of congestive heart failure. On the bases histopathology CS and IGCM may have similar characteristic. Eosinophils, myocyte damage, and foci of lymphocytic myocarditis were more frequent in IGCM, while granulomas and fibrosis were more frequent in CS. The differentiation between CS and IGCM is clinically important, because the natural history of the disease, survival, and the treatment are different. For example, CS has better prognosis than IGCM, the 5 years survival is 80% for CS versus 20% for IGCM.The treatment for CS is corticosteroids. However, the treatment for IGCM is the combination of corticosteroids and cyclosporine which showed a survivor benefit compared with corticosteroids only.

CONCLUSION: The clinical cardiac involvement occurs in fewer than 5% of patients with sarcoidosis.CS and IGCM can look similar on the histopathology. Lung biopsy may help to Cardiac sarcoid patients treated with had 5-year survival rates 59-89%. However, the combination of corticosteroids and cyclosporine for patients with Giant Cell Myocarditis had 5-year survival rates of 10-18%.

REFERENCES:

(1) Cooper et al. New England Journal of Medicine 1997;336(26):1860-1866.

(2) Okura et al. American College of Cardiology. 41(2):322-9, 2003 Jan 15.

DISCLOSURE: Bassel Ramadan, None.

Bassel Ramadan MD * Emory University, Atlanta, GA

COPYRIGHT 2005 American College of Chest Physicians
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