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Case reports |
a Department of Cardiology, University of Aberdeen, Aberdeen, Scotland AB25 2ZN, United Kingdom
b Department of Oncology, Aberdeen Royal Infirmary, Aberdeen, Scotland AB25 2ZN, United Kingdom
c Department of Cardiothoracic Surgery, Aberdeen Royal Infirmary, Aberdeen, Scotland AB25 2ZN, United Kingdom
Received 19 October 2007; received in revised form 28 November 2007; accepted 10 December 2007.
* Corresponding author. Tel.: +44 1224 552415; fax: +44 1224 550692. (Email: g.small{at}abdn.ac.uk).
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Key Words: Pericardium • Mesothelioma • Radiotherapy
Diseases of the pericardium can present with symptoms commonly associated with valvular and coronary heart disease and therefore pericardial pathology may initially be overlooked. In such cases a full clinical evaluation of the patient and readiness to use the many imaging modalities available can help the heart specialist detect pericardial disease. An appreciation of these imaging techniques can also help the cardiothoracic surgeon to determine whether pericardiectomy is indicated. This case demonstrates the importance of a multifaceted imaging approach in pericardial disease and indicates how integration of the findings from such studies may help clinicians treat rare diseases.
A 62-year-old lady presented acutely in atrial fibrillation with a pale pulseless left lower limb. The lady was a non-smoker and wife of a car mechanic. A left femoral artery embolectomy was performed, the patient was anti-coagulated and the atrial fibrillation treated by controlling her ventricular rate. Post-embolectomy she developed clinical and radiological pulmonary oedema.
Three months previously, the patient had presented to the general physicians with dyspnoea. Echocardiography had demonstrated a significant pericardial effusion without signs of cardiac tamponade. A sterile, acellular pericardial effusion had been drained. Previously in 1993, the patient had undergone a left mastectomy and axillary node clearance for breast cancer; adjuvant chemotherapy and radiotherapy had been administered. Following the finding of the pericardial effusion, a CT chest and abdomen were performed but no evidence was found to suggest recurrence of breast carcinoma. In addition blood tests for autoimmune diseases, renal function and occult infection were all unremarkable.
Cardiac examination revealed a raised JVP with rapid Y descent and Kussmaul's sign. A comprehensive echocardiogram was performed; good left ventricular systolic function was seen, there was however features consistent with diastolic dysfunction that suggested constrictive pericarditis. These included interventricular septal bounce, rapid early diastolic filling, a maintained a’ on tissue Doppler. Increased tricuspid E wave inflow velocity on first inspiratory breath with corresponding decrease in mitral E wave velocity was also observed. No significant pericardial effusion was seen.
To better delineate the pericardium a T1 weighted cardiac MRI was performed (Fig. 1a). Not only was the pericardium increased in depth it also demonstrated crescenteric thickening that suggested there might be tissue invasion.
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The patient was referred for pericardiectomy for presumed radiotherapy induced constrictive pericarditis. Pericardiectomy was performed via a midline sternotomy incision. The parietal pericardium was densely thickened but not calcified and was removed with care but with great difficulty. The visceral pericardium however was friable and closely adherent to the myocardium dissection proved highly challenging. Despite the difficulties the procedure was uncomplicated; indeed RA pressures were noted to reduce during the course of the procedure suggesting some early relief from constriction. Post operatively the patient recovered without incident.
Pericardial histology revealed pericardial epithelioid malignant mesothelioma (Fig. 2a). Typical morphology was seen with haematoxylin and eosin staining and the diagnosis confirmed by calretinin, CK 5/6 and linear staining with EMA.
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At this stage the patient elected to pursue palliative approach and she was carefully managed with diuretics and supportive care. Nine months following her presentation the patient died peacefully at a palliative care facility.
Pericardial mesothelioma is a rare tumour with a high early mortality rate, the median survival from diagnosis is 6 months [1]. It has been described more frequently in males and presents between the fourth to seventh decade [2]. Due to the paucity of cases it has been difficult to determine with certainty aetiological factors that might give rise to this rare tumour.
No definite association with asbestos exposure has been described. In this case the absence of concomitant pleural disease and the localised nature of the disease would argue against previous inhalation exposure to asbestos. Nevertheless it is possible that she may have suffered unrecognised exposure through the occupation of her spouse. Car mechanics may come into contact with asbestos through vehicle clutch linings and brake materials; although the potential for such exposure to increase mesothelioma risk is debated [3,4].
Tissue microscopy can help clarify aetiology by identifying asbestos fibres. This is particularly useful with respect to pleural mesotheliomas however histology in pericardial disease is usually unhelpful. For although three different types of pericardial mesothelioma are described: epithelioid, sarcomatous and mixed [5] the tumours are too infrequent and perhaps asbestos exposure such an indiscernible factor that no correlation between tumour type and aetiology has been noted.
In this case radiotherapy was initially considered to have induce a fibrous pericarditis [6]. The finding of pericardial epithelioid mesothelioma was unexpected but nevertheless may still be attributable to radiotherapy. Radiation therapy has previous been described in association with pericardial mesothelioma [7] and pleural mesotheliomas [8,9]. Experience from cancer centres suggests that the risks of developing pleural mesothelioma following radiotherapy for breast cancer is 0.3% [9]. The association between pericardial mesothelioma and radiotherapy remains perhaps less certain for there is a 2:1 male preponderance for this condition in spite of the obvious female preponderance for breast cancer and subsequent radiotherapy.
This rare disease demonstrates the importance of a multifaceted imaging protocol to identify and characterise cardiac abnormalities. The case highlights the important role of the cardiac surgeon in making tissue diagnoses and providing relief of symptoms. In this case although the patient's tumour load was apparently reduced by surgery the survival outcome remained poor. Newer chemotherapeutic regimens are being tried in this invariable fatal condition with some encouraging results [10].
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