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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.

Arquivos Brasileiros De Cardiologia
|January 1, 1991
PubMed
Summary
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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.

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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.