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Until the 1950s, cardiac tumours were merely a curiosity. Diagnosis was academic and outlook poor. With the advent of cardiopulmonary bypass, however, surgical management became possible, particularly of intracavity tumours. More recently, the development of echocardiography, compututed tomography, and magnetic resonance imaging has contributed greatly to the process of preoperative diagnosis.
Epidemiology and nomenclature
Primary cardiac tumours are rare, with a necropsy incidence of 0.05%.1 Secondary deposits are seen more frequently, in 1% of postmortem examinations, but usually in the setting of widely disseminated malignancy.2 The relative incidence of presentation is shown in table 1, and demonstrates that atrial myxoma is by far the most common primary cardiac tumour in adults, and rhabdomyosarcoma is the most common in children. A quarter of all cardiac tumours are malignant, the majority of which are angiosarcomas or rhabdomyosarcomas.
General clinical features
Cardiac tumours are diverse in clinical presentation, and atrial myxomas in particular may cause systemic symptoms mimicking collagen vascular disease, malignancy or infective endocarditis. There are several clinical features, however, that are seen commonly with many cardiac tumours:
Embolisation—This occurs frequently. Either the tumour itself, or adherent thrombus may dislodge and migrate; hence the age old aphorism that all retrieved emboli should be examined histologically. Multiple small emboli may mimic vasculitis or endocarditis, while larger fragments may lead to cerebrovascular events. Right sided tumours naturally embolise to the lungs producing pleuritic symptoms and possibly right heart failure.
Obstruction—Atrial tumours, once they are large enough, may result in obstruction of atrioventricular valvar flow, and, in particular, may mimic valvar stenosis. Symptoms are often markedly paradoxical and may relate to body positions. Ventricular tumours, though in general less frequent, may obstruct outflow tracts leading to chest pain, breathlessness or syncope.
Arrhythmias—Intramyocardial and …