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Cardiac function in children with congenital adrenal hyperplasia

Tuğba Kontbay Çetin1,2, Abdulkadir Akkuş3, Müge Atar1,4

  • 1Department of Paediatric Endocrinology, Şanlıurfa Training and Research Hospital, Şanlıurfa, Türkiye.

Journal of Pediatric Endocrinology & Metabolism : JPEM
|April 8, 2025
PubMed

Insights

Conventional hydrocortisone treatment for congenital adrenal hyperplasia (CAH) may negatively impact cardiac function in children. This study found impaired systolic function and altered diastolic function in pediatric CAH patients on long-term therapy.

Area of Science:

  • Pediatric Endocrinology
  • Cardiovascular Physiology
  • Genetics and Inherited Diseases

Background:

  • Congenital adrenal hyperplasia (CAH) involves glucocorticoid imbalance and hyperandrogenism, potentially leading to cardiovascular issues.
  • Failure to mimic physiological circadian rhythms in treatment can exacerbate cardiovascular risks.
  • Conventional hydrocortisone therapy is standard but its long-term cardiac effects in children are not fully understood.

Purpose of the Study:

  • To assess cardiac function in pediatric patients with CAH receiving conventional hydrocortisone treatment.
  • To investigate the relationship between hydrocortisone dosage, therapy duration, and cardiac function in CAH.
  • To compare cardiac function in pediatric CAH patients with age- and gender-matched healthy controls.

Main Methods:

  • Enrolled 23 pediatric patients with CAH (ages 3-16) and 21 healthy controls.
  • Utilized physical examination, electrocardiogram (ECG), conventional echocardiography, and tissue Doppler imaging (TDI).
  • Analyzed systolic myocardial velocity (Sm), systolic excursion, early diastolic myocardial velocity (Em), and E/Em ratio.

Main Results:

  • Pediatric CAH patients showed significantly lower mean Sm and systolic excursion compared to controls.
  • Tricuspid-derived Em and E/Em ratio were significantly higher in the CAH group.
  • No evidence of left ventricular hypertrophy or dilatation was observed via ECG or echocardiography.

Conclusions:

  • Long-term conventional hydrocortisone therapy, even within recommended doses, may adversely affect cardiac function in children with 21-hydroxylase deficiency.
  • Findings suggest a need for closer cardiac monitoring in pediatric CAH patients.
  • Further research into optimized hydrocortisone regimens is warranted.
Abstract

Keywords:
M-mode echocardiographyadrenal insufficiencyhydrocortisone treatment

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