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Primary cardiac tumours: when is surgery necessary?
1Department of Pediatric Cardiology, Deutsches Herzzentrum, Augustenburger Platz 1D-13353, Berlin, Germany. stiller@dhzb.de
Insights
Pediatric cardiac tumors are often benign and may regress spontaneously. Surgery is reserved for symptomatic cases, focusing on heart function restoration rather than complete resection.
Area of Science:
- Pediatric Cardiology
- Pediatric Cardiac Surgery
- Developmental Biology
Background:
- Primary cardiac tumors are rare in children, with limited long-term data compared to adult myxomas.
- Improved fetal ultrasonography leads to earlier detection of pediatric cardiac tumors.
- Distinguishing between surgical and conservative management for pediatric cardiac tumors presents a clinical challenge.
Purpose of the Study:
- To analyze the presentation, characteristics, and outcomes of primary cardiac tumors in a pediatric cohort.
- To evaluate the efficacy of surgical versus conservative management strategies.
- To identify factors predicting tumor behavior, including spontaneous regression.
Main Methods:
- Retrospective review of 51 cardiac tumors in 26 children over a 10-year period.
- Analysis included tumor presentation, location, histology, interventions, and clinical course.
- Median patient age was 1 month.
Main Results:
- Rhabdomyomas were the most common (29 cases), followed by fibromas (9).
- Tumors frequently occurred in the ventricles, presenting with murmurs, arrhythmias, or outflow tract obstruction.
- Surgical intervention was performed in 14 children with hemodynamic compromise; 9 rhabdomyomas showed spontaneous regression.
Conclusions:
- Most pediatric cardiac tumors are benign, with potential for spontaneous regression beyond rhabdomyomas.
- Surgical intervention is indicated for clinically significant symptoms, not solely for tumor removal.
- Restoring optimal cardiac function is the primary therapeutic goal, superseding complete resection.
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.