Clinical Radiology (2008) 63, 605e609
CASE REPORT
Superior vena caval obstruction after complete resolution of cardiac tuberculoma G. Singh Gulatia,*, S. Singhb, C. Dey Arepallia, S. Sharmaa, S. Sunder Kotharib, B. Airanc, P. Jagiaa Departments of aCardiac Radiology, bCardiology and c Cardiothoracic and Vascular Surgery, Cardiothoracic Center, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
Introduction Superior vena cava obstruction (SVCO) is uncommon. It usually results from extrinsic compression/ invasion from mediastinal masses, with the majority of the lesions being malignant.1 Rarer causes include mediastinal lymphadenopathy, benign tumours, fibrosing mediastinitis, thyroid enlargement, aortic aneurysm, or placement of pacing leads and catheters.2,3 Cardiac involvement in tuberculosis most often manifests as tuberculous pericarditis. Involvement of the myocardium is reported in up to 0.3% of cases dying of tuberculosis.4 Association of SVCO with cardiac tuberculoma is extremely rare, with only one case reported in 1973.5 We describe the imaging findings in a patient who developed persistent SVCO after complete disappearance of a cardiac tuberculoma on anti-tuberculous therapy (ATT).
Case report A 29-year-old woman was referred with a history of fever, palpitations, facial puffiness, and mild change in voice for the past 18 months. There was no history of dyspnoea on exertion, weakness, or syncope. The patient had received a 9-month course of ATT for suspected pulmonary tuberculosis 5 years previously. On examination, her heart rate was 68 beats/ min, irregularly irregular, and her blood pressure * Guarantor and correspondent: G. Singh Gulati, Cardiac Radiology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi-110029, India. Tel.: þ91 11 26594759; fax: þ91 11 26588663. E-mail address:
[email protected] (G.S. Gulati).
was 126/74 mmHg. There was no murmur. The jugular venous pressure was raised with prominent v waves and y descent. There was no pedal oedema, the liver and spleen were not palpable, and there was no ascites. The respiratory system examination was normal. Electrocardiogram (ECG) showed atrial flutter with a variable degree of AV block. The results of laboratory investigations of the blood and urine were normal. The patient was not immunocompromised and tests for human immunodeficiency virus (HIV) antibody were negative. The chest radiograph showed cardiomegaly with enlargement of the right atrium (RA) and minimal right-sided pleural effusion. Echocardiography (ECHO) revealed a mass in the region of the superior vena cava (SVC)-right atrium (RA) junction, which could not be separately visualized from the pericardium. The lower RA was free of the mass and the inferior vena cava (IVC) was normal. The interatrial septum was intact. The pericardium elsewhere was not thickened and there was no effusion. The left and right ventricles were normal in size and function. A similar mass had been detected on echocardiography performed 10 months before admission to hospital, for which the patient had undergone a magnetic resonance imaging (MRI) examination at the time. This had revealed a 4 6 7 cm mass involving the SVCeRA junction and extending from the pericardium into the right atrial cavity in this region. A repeat contrast-enhanced MRI (Avanto, Siemens, Germany) and CT (sensation 16, Siemens, Germany) examinations were performed. On comparison with the previous MRI, the mass had now enlarged in size (5.5 7 7.5 cm) and was inseparable from the adjacent pericardium (Fig. 1a and b). The lower SVC was compressed by the
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Figure 2 Cardiac MRI image at 18 months after initiation of ATT. (a) Axial, spin-echo, T1-weighted image shows near complete resolution of the mass. The SVC lumen is better visualized (compare with Fig. 1a). (b) The mass along the right atrial wall is not visualized now (compare with Fig. 1b). Figure 1 Pre-treatment cardiac MRI and CT images. (a) Axial, spin-echo, T1-weighted MRI image shows a homogeneous isointense mass at the lower end of the SVC (arrow), which is inseparable from the adjacent pericardium. The mass is compressing the lower end of the SVC. Another similar mass is noted in the region of the left atrial appendage. (b) The lower extent of the same right-sided mass (arrow) is seen to project into the right atrial cavity. (c) Contrast-enhanced CT in the axial plane shows nearly homogeneously enhancing and non-calcified masses in the previously described locations (arrows).
mass. Another mass lesion of size 3.5 4 4.4 cm was seen in the left atrial appendage (not appreciated earlier). On MRI, the lesions were isointense to the myocardium on fast spin-echo T1-weighted and T2-weighted images, well-defined with a sharp outline, and without any flow voids. The right coronary artery was not involved by the right-sided lesion. CT did not reveal any calcification (Fig. 1c). The
Superior vena caval obstruction after complete resolution of cardiac tuberculoma
pericardium elsewhere was not thickened and the lungs were normal. On both CT and MRI images, the lesions were seen to enhance homogeneously. A CT-guided FNAC of the right-sided mass revealed an ill-defined granuloma. A complicating pneumothorax was managed conservatively. In the absence of a definitive aetiological diagnosis, a surgical exploration of this mass under cardiopulmonary bypass was performed. It revealed an extremely hard, tumour-like mass filling the upper RA and extending into the proximal SVC. Dense adhesions between the lesion and the adjacent thickened pericardium and the heart precluded the excision of the mass. Histopathological examination of the intra-operative sections showed a granulomatous lesion with necrosis and fibrosis.
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Histological staining for Mycobacterium and fungi were negative. Based upon the above findings and a history of tuberculosis, a diagnosis of cardiac tuberculoma was considered likely and ATT was started. Sequential follow-up MRI examinations showed progressive reduction in the size of both masses (Fig. 2a and b). The imaging evidence of resolution of the mass was mirrored by an improvement in the patient’s signs and symptoms, with disappearance of her arrhythmias. The patient received ATT for a total duration of 24 months. Six months after completion of therapy, the patient presented with an increase in the facial and right upper limb swelling and choking sensation while lying supine that was relieved in the head-up position. MRI
Figure 3 Cardiac MRI and MRA images at 6 months after completion of ATT. (a) Axial, spin-echo, T1-weighted image shows persistent pericardial thickening (arrow) at the lower end of the SVC. The latter itself is not visualized now. (b) Coronal image from the MRA examination shows occlusion at the lower end of the SVC (arrow). (c) Coronal image shows the dilated azygous vein (*).
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revealed no recurrence of the mass, but focal pericardial thickening at the SVCeRA junction with complete obliteration of the lower end of SVC (Fig. 3a). The distal SVC and azygous vein were dilated. MR angiography confirmed the findings (Fig. 3b and c). Phase-contrast MRI showed flow reversal in the azygous vein. There was no evidence of generalized pericardial constriction, as evidenced by the absence of any abnormal diastolic septal motion and normal septal position on deep inspiration. A superior vena cavogram through the antecubital vein approach, with simultaneous injection into the RA via a femoral vein access was performed. This revealed a 2 cm long occlusion of SVC with flow reversal in the azygous vein (Fig. 4). The mean pressure gradient across the occlusion was 10 mmHg. An angioplasty of the lesion was not performed in view of the length of the lesion precluding a safe recanalization. After discussion with the patient, it was decided to manage her condition with medical therapy (diuretics) and to consider surgical bypass/ angioplasty should her symptoms worsen.
Figure 4 Venogram with simultaneous injections into the SVC and the RA. There is complete occlusion of the SVC at its lower end (pressure gradient is 10 mmHg).
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Discussion SVCO may result from a variety of causes, the most common being malignant lesions (97%), such as bronchogenic carcinoma (85%), lymphoma, and metastatic mediastinal disease.1,2 Benign diseases account for 3e26% of the reported cases.1,6 SVCO is an uncommon finding in patients with tuberculosis and generally develops due to the dense fibrosing mediastinitis that may result from the disease.2 Rarer still is an extrinsic compression of the SVC from enlarged tuberculous mediastinal lymph nodes.2 None of these entities was present on imaging in the present case. In tuberculosis, involvement of the heart, apart from tuberculous pericarditis, is extremely rare.7 The reported overall autopsy incidence of pericardial involvement is 6%, and that of myocardial involvement ranges from 0.14e0.3%.4,8 A tuberculoma, which is a mass-like manifestation of the disease, is highly unusual and its description in the literature is confined to case reports.5,7,9,10 The lesion most commonly involves the RA, close to SVCeRA junction or the right ventricle.5,7,9,10 The differential diagnosis would usually include angiosarcoma, myxoma, and thrombus. Due to its superior contrast resolution, multiplanar imaging capability, and unrestricted field of view, MRI is advantageous over echocardiography for the detection, characterization, and complete morphological and functional evaluation of cardiac masses.11 The presence of isointensity of the lesion on both T1weighted and T2-weighted images, and lack of involvement of right coronary artery suggest that a diagnosis of angiosarcoma is highly unlikely. Myxoma is the most common cardiac tumour. However, the homogeneous signal intensity of the mass, the absence of haemorrhage or calcification (on CT) and a normal interatrial septum mitigate this diagnosis. Thrombus was not considered as there was significant pericardial involvement. The treatment for cardiac tuberculosis primarily involves the institution of ATT. Complete recovery of the patient’s signs and symptoms along with imaging evidence of resolution of cardiac involvement on ATT has been reported by several authors.10,12 The present case is unique in that the SVCO was associated with a cardiac tuberculoma and persisted and worsened despite complete resolution of the mass on treatment. SVCO in a case of cardiac tuberculoma has been described in only one previous report.5 The probable explanation for persistent SVCO in the present case could be that the mass was predominantly pericardial in nature with secondary
Superior vena caval obstruction after complete resolution of cardiac tuberculoma
infiltration of the RA, and after ATT, the focal pericardial constriction that developed in this location caused obliteration of the lower end of the SVC. This is supported by the presence of persistent pericardial thickening at the lower end of SVC on MRI. A traction injury to the lower SVC as a result of attempts made to excise the lesion during surgery may also have contributed to the occlusion. To conclude, tuberculoma is a rare manifestation of cardiac involvement in tuberculosis, and persistent SVCO may rarely develop after its treatment, particularly in patients with predominant pericardial involvement. MRI is particularly suited to suggesting the aetiology, demonstrating the associated anatomical and functional alterations, and following up the lesions on treatment.
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2. Bandhyopadhyay SK, Sarkar N, Ghosh S, et al. Tubercular mediastinal lymphadenopathy presenting as superior vena cava syndrome. J Assoc Physicians India 2002;50:1194e5. 3. Ham RJ, Bulstrode C, Eadie DGA. Saphenousejugular bypass for superior vena cava obstruction. Br J Surg 1985;72:193e4. 4. Horn H, Saphir O. The involvement of the myocardium in tuberculosis: a review of literature and report of three cases. Am Rev Tuberc 1935;32:492e506. 5. Kapoor OP, Mascarenhas E, Rananaware MM, et al. Tuberculoma of the heart: report of nine cases. Am Heart J 1973; 86:334e40. 6. Urschel HC, Paulson DL. Superior vena caval obstruction. Dis Chest 1966;49:155e64. 7. Batra R, Trehan V, Salwan R, et al. Antemortem diagnosis of cardiac tuberculoma. Indian Heart J 1998;50:87e9. 8. Rose AG. Cardiac tuberculosis: a study of 19 patients. Arch Pathol Lab Med 1987;11:422e6. 9. Jagia P, Gulati GS, Sharma S, et al. MRI features of tuberculoma of the right atrial myocardium. Pediatr Radiol 2004; 34:904e7. 10. Krishnaswami H, Cherian G. Right atrial tuberculoma: report of a case with complete recovery. Thorax 1984;39:550e1. 11. Gulati GS, Sharma S, Kothari SS, et al. Comparison of ECHO and MRI in the imaging evaluation of intracardiac masses. Cardiovasc Intervent Radiol 2004;27:459e69. 12. O’Neill PG, Rokey R, Greenberg S, et al. Resolution of ventricular tachycardia and endocardial tuberculoma following antituberculosis therapy. Chest 1991;100:1467e9.
