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[Cardiac rhabdomyomas]
J L Jacob1, S Hassen Sobrinho, A M Lorga
1Instituto de Moléstias Cardiovasculares (IMC), Sáo José do Rio Perto.
Insights
Cardiac rhabdomyoma, a rare tumor, caused fatal cyanosis and respiratory issues in a newborn. Early echocardiography is crucial for potential surgical intervention in infants with these heart tumors.
Area of Science:
- Pediatric Cardiology
- Oncology
Background:
- Cardiac rhabdomyoma is a rare benign tumor of the heart, often associated with tuberous sclerosis.
- It can cause significant hemodynamic compromise in neonates.
Observation:
- A case report details a 1-2 day old infant presenting with severe cyanosis and respiratory insufficiency.
- Radiological findings included significant cardiomegaly, and electrocardiography revealed a left bundle branch block.
- Two-dimensional echocardiography identified multiple intracavitary tumors within both ventricles and the right atrium.
Findings:
- Post-mortem examination confirmed the presence of multiple cardiac rhabdomyomas.
- Microscopic analysis revealed characteristic 'spider cells' with vacuolation and glycogen presence.
- The diagnosis of cardiac rhabdomyoma was definitively established.
Implications:
- Early diagnosis via echocardiography is critical for managing cardiac rhabdomyoma in neonates.
- Surgical resection of intracavitary tumors causing outflow tract obstruction may be life-saving.
- Further investigation for tuberous sclerosis and potential renal tumors is warranted, though not performed in this case.
Abstract:
Case report of cardiac rhabdomyoma in one two days old infant with important cyanosis and respiratory insufficiency with evolution to death in few hours. The radiologic feature was a great enlargement of the heart and the electrocardiographic study showed left branch block. The two-dimensional echocardiography study showed multiple intracavitary tumours in both ventricles and in the right atrium. These features were confirmed in the post-mortem study. The microscopic study of the tumors showed proliferation of the spider cells, and vacuolation with the presence glycogen. The diagnosis of cardiac rhabdomyoma was confirmed. The brain and the kidney were not studied for the diagnosis of tuberous sclerosis or kidney tumors. It is very important the early diagnosis by echocardiography, due to the possibility of surgical resection of the intracavitary tumors that cause severe obstruction to the blood flow.