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Johns Hopkins Hospital Baltimore, MD 21287
Johns Hopkins University School of Medicine Baltimore, MD
21287
Sarcoidosis is a granulomatous disease that infiltrates any area of the body, but most of its morbidity and mortality are due to invasion of the cardiac system. We present a case of a biopsy-proven interventricular septal mass detected on MR imaging.
A 33-year-old African American woman with no significant medical history underwent a routine physical examination. ECG findings were abnormal. She was referred for an echocardiogram that revealed normal left ventricular function with an abnormal septum.
Cardiac MR imaging (1.5 T) confirmed the presence of a broad-based mass located in the basilar part of the interventricular septum, protruding into the right ventricle. On T1-weighted images (double inversion recovery–blood suppression technique; TR/TE 900/10), the mass was nearly isointense to adjacent myocardium (Fig. 1A). On T2-weighted images (double inversion recovery–blood suppression technique; 1800/80; chemical shift fat suppression), the lesion was slightly hyperintense compared with normal myocardium. On first-pass perfusion images (0.1 mmol/kg of gadodiamide; 7/1.4; inversion prep time, 175 msec; flip angle, 20°), the mass appeared hypointense to normal myocardium (Fig. 1B). The mass was hyperintense on delayed gradient-echo images (10/ 4.6; flip angle, 20°) (Fig. 1C). A moderate pericardial effusion was also present (Fig. 1A). Transvenous endomyocardial biopsy was nondiagnostic.
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After 2 months, the patient presented with fatigue, diaphoresis, and a pounding sensation in her chest. ECG findings revealed ventricular tachycardia. Repeated MR imaging showed no significant change in the size or characteristics of the mass, but several pulmonary nodules were visualized. Open biopsy revealed noncaseating granulomata without evidence of malignancy (Fig. 1D). The final diagnosis was cardiac and pulmonary sarcoidosis.
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Cardiac sarcoidosis has never been described as a mass seen on MR imaging. The differential diagnosis for this patient included primary or metastatic neoplasm. Mural thrombus and asymmetric septal hypertrophy were ruled out on the basis of enhancement characteristics of the mass. The location of this mass in the basal septum and the presence of pulmonary nodules were suggestive of cardiac sarcoidosis.
Although biopsy is the only definitive diagnostic tool for cardiac sarcoidosis, radiologic techniques continue to play an important role. Transvenous endomyocardial biopsy has low sensitivity, as shown in this patient, especially because biopsies are often taken from the apical septum, whereas the basal septum is more likely to be affected by cardiac sarcoidosis [1]. Open biopsies carry even greater risks, despite their increased sensitivity. As cardiac MR imaging continues to be refined, it will become a major tool for the diagnosis of cardiac sarcoidosis.
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  J. Vogel-Claussen, C. E. Rochitte, K. C. Wu, I. R. Kamel, T. K. Foo, J. A. C. Lima, and D. A. Bluemke Delayed enhancement MR imaging: utility in myocardial assessment. RadioGraphics, May 1, 2006; 26(3): 795 - 810. [Abstract] [Full Text] [PDF]
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 E. Tadamura, M. Yamamuro, S. Kubo, S. Kanao, T. Saga, M. Harada, M. Ohba, R. Hosokawa, T. Kimura, T. Kita, et al. Effectiveness of Delayed Enhanced MRI for Identification of Cardiac Sarcoidosis: Comparison with Radionuclide Imaging Am. J. Roentgenol., July 1, 2005; 185(1): 110 - 115. [Abstract] [Full Text] [PDF]
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