Background: The literature on primary cardiac tumours is relatively limited because of their rare occurrence.
Objective: To report the authors' experience with primary cardiac tumours at McGill University teaching hospitals, Montreal, Quebec. In addition, four exceedingly rare primary cardiac tumours are presented--three valve myxomas and the seventh reported case of a primary synovial sarcoma.
Methods: Twenty-one patients resected for primary cardiac tumours were retrospectively reviewed in terms of their clinical presentation, surgical treatment, histopathological findings and outcome. Postoperative success was defined by disease-free survival after resection. A survival analysis was performed using the Kaplan-Meier life-table method.
Results: The mean age at the time of resection was 45.5 years (range birth to 74 years). The most common clinical presentation in adults was dyspnea (38%) or central nervous system/embolic phenomena (24%), and in the pediatric group, it was hypoxia (50%). All but one of the tumours were visualized using transthoracic echocardiography. Coronary angiography was performed in 11 patients, with a diagnosis of coronary artery disease in five patients (45%), all of whom underwent coronary artery bypass grafting. The surgical approach was uniatrial in 10 patients (48%)--seven (33%) via the left atrium and three (14%) via the right atrium. Eight patients (38%) required a biatrial approach; the remaining three patients (14%) with ventricular tumours required a ventricular approach. Of the 21 cases, 17 were adults and four were pediatric cases. There were 16 myxomas (76%), two rhabdomyomas (10%), one fibroma (5%), one angiosarcoma (5%) and one synovial sarcoma (5%). In adults, myxoma was the most common primary cardiac tumour (88%), whereas rhabdomyoma occurred most frequently in the pediatric population (50%). Three valve myxomas, two involving the mitral valve and one involving the tricuspid valve, were encountered; all underwent conservative leaflet resection and valve repair. The probability of disease-free survival for all patients was 80.7% (95% CI 63.6 to 97.7) at one year, and 75.6% (95% CI 57.0 to 94.3) at both five and 10 years after resection. For adult benign tumours, the probability of disease-free survival was 93% (95% CI 79.4 to 100.0) at one, five and 10 years. There was a total of five deaths.
Conclusions: The results suggest that complete surgical resection of primary cardiac tumours prolongs symptom-free survival and is associated with a low recurrence rate. Benign primary cardiac tumours have an excellent prognosis in the adult population compared with in the pediatric population (93% versus 50% survival, respectively, in the present cohort). Furthermore, conservative leaflet resection with valve repair of valve myxomas, rather than radical excision and valve replacement, should be strongly considered. Early identification and diagnosis of primary cardiac tumours are necessary for expeditious surgical resection if a cure is desired, especially when encountering malignant primary cardiac sarcomas.