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Eur J Cardiothorac Surg 2001;20:1002-1006
© 2001 Elsevier Science NL

Primary cardiac tumours: when is surgery necessary?

^a Department of Pediatric Cardiology, Deutsches Herzzentrum, Augustenburger Platz 1D-13353, Berlin, Germany
^b Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum, Berlin, Germany
^c Department of Pathology, Deutsches Herzzentrum, Berlin, Germany
^d Department of Pediatric Cardiology, Charité Berlin, Germany

Received 19 June 2001; received in revised form 25 July 2001; accepted 30 July 2001.

Corresponding author. Tel.: +49-30-45932800; fax:+49-30-45932900
e-mail: stiller{at}dhzb.de

Objective: Primary cardiac tumours are rare. The literature predominantly contains series on myxomas in adults and only a few long-term series that involve the very different primary cardiac tumours in early childhood. As foetal ultrasonography has continued to improve, cardiac tumours are increasingly detected early before significant symptoms develop. It is a challenge for paediatric cardiologists and surgeons to ascertain which patients need surgery and which will benefit from conservative follow-up. Methods: A retrospective review of a 10-year period revealed 51 tumours in 26 children (median age: 1 month). Analysis was by presentation, location, associated findings, interventions, histological findings, and clinical course. Results: The most common tumours were rhabdomyomas (29), fibromas (nine), teratomas (two), and haemangiomas (two). The tumour location was the right ventricle in 24 and the left ventricle in 22 patients. The symptoms varied between abnormal heart murmur (20), arrhythmia and conduction abnormalities (ten), obstruction of the outflow tract >30 mmHg (nine), severe cyanosis (three) and congestive heart failure (two). Fourteen children with haemodynamic compromises underwent surgery. There was one post-operative death and one heart transplantation after bridging with an assist device. There was no tumour recurrence even when resection was incomplete. Nine of 13 children with rhabdomyomas had spontaneous tumour regression without intervention. Conclusions: Most of the cardiac tumours in children are benign. Spontaneous regression is possible not only in rhabdomyoma. Surgical intervention is only required for children who develop relevant clinical symptoms. Total resection of the tumour is not the only therapeutic aim; more important is the restoration of the best possible heart function.

Key Words: Cardiac tumour • Children • Rhabdomyoma • Fibroma • Teratoma

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