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Hypertrophic obstructive cardiomyopathy in an infant with an adrenocortical tumor

Jakob Hauser1, Stefan Riedl, Ina Michel-Behnke

  • 1Division of Pediatric Cardiology, Department of Pediatrics and Adolescent Medicine, and cDepartment of Pediatric Surgery, Medical University of Vienna, Vienna, Austria. jakob.hauser@meduniwien.ac.at

Pediatrics
|July 10, 2013
PubMed

Insights

A rare adrenal tumor caused Cushing's syndrome in an infant, leading to heart problems. Surgical removal resolved the condition, normalizing weight and heart function.

Area of Science:

  • Pediatric Endocrinology
  • Cardiology
  • Oncology

Background:

  • Nonfamilial cardiomyopathies in children are rarely linked to endocrine disorders.
  • Adrenocortical tumors can secrete hormones, causing systemic effects.

Observation:

  • A 4-month-old infant presented with Cushing's syndrome, virilization, and severe hypertrophic obstructive cardiomyopathy.
  • Diagnostic imaging revealed a large adrenal tumor with elevated androgen and cortisol levels.

Findings:

  • Surgical resection of the adrenocortical tumor was successful.
  • Postoperative management included careful glucocorticoid substitution and tapering.
  • Histopathology confirmed an intermediate-grade adrenocortical tumor without metastasis.

Implications:

  • This case highlights the potential for adrenocortical tumors to cause severe cardiomyopathy in infants.
  • Successful surgical and endocrine management can lead to complete recovery.
  • Provides valuable insights into postoperative glucocorticoid management in pediatric cases.

Keywords:
Cushing’s syndromeLi-Fraumeni syndromeTP53adrenocortical tumorhypertrophic obstructive cardiomyopathy

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