Left atrial cavernous haemangioma presenting with cardiac tamponade in a patient with Cowden syndrome

What is it about?

Cowden Syndrome (CS) is a rare disorder characterised by multiple noncancerous, tumuor-like growths called hamartomas. The syndrome is associated with development of cancer of the breast, endometrium, kidneys, skin and rarely the brain. We report a rare case of symptomatic cardiac haemangioma in a patient with CS. A 54-year-old female with CS presented with dyspnoea and orthopnoea in the setting of cardiac tamponade. Echocardiography revealed large haemopericardium and tamponade physiology secondary to a pericardial mass. The patient underwent urgent cardiopulmonary bypass with removal of the mass. Histopathology confirmed a benign cavernous haemangioma. We postulate that tumours involving the heart/pericardium may be an additional manifestation of Cowden Syndrome. This case further highlights the necessity to consider pericardial/cardiac manifestations in patients with hamartomatous syndromes who present with cardiorespiratory symptoms, so that opportunistic investigation and treatment may be instituted.

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