Multi-disciplinary evaluation of a 5-month-old with hypertrophic cardiomyopathy related to a functional adrenocortical tumor

Laura M. Nally, Erin Conner, Sharon Paige, Kelly L. Mooney, Urs Naber, Rebecca Richards, Gail Wright

Research output: Contribution to journalArticlepeer-review

Abstract

Hypertrophic cardiomyopathy (HCM) in childhood is a rare diagnosis, and associations with adrenocortical tumors (ACTs) have been rarely reported in the pediatric literature. We present a case of a 5-month-old who presented with HCM and during the evaluation for hypertension was found to have elevated glucocorticoids, mineralocorticoids, androgens and urine metanephrines. During preoperative evaluation, he developed shock followed by cardiogenic collapse requiring extracorporeal membrane oxygenation (ECMO); however, he did not survive. Pathology revealed an ACT with hormone production that contributed to his demise. Adrenocortical tumors associated with hypertrophic cardiomyopathy can be life-threatening. We discuss the complex interplay of unrestricted cortical hormone production in the setting of hypertrophic cardiomyopathy that may lead to rapid decline and poor clinical outcomes.

Original languageEnglish (US)
Pages (from-to)1371-1376
Number of pages6
JournalJournal of Pediatric Endocrinology and Metabolism
Volume31
Issue number12
DOIs
StatePublished - Dec 1 2018
Externally publishedYes

Keywords

  • Cushing's syndrome
  • adrenal tumor
  • adrenocortical tumor
  • hypertension
  • hypertrophic cardiomyopathy
  • infant

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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