Key result
Corticosteroid replacement rapidly reverses LV dysfunction and resolves heart failure in adrenal insufficiency-associated cardiomyopathy.
Why the study?
Reversible cardiomyopathy is a rare and potentially life-threatening complication of Addison's disease with limited pediatric case reports and unclear pathophysiology.
High-dose hydrocortisone and fludrocortisone may reverse pediatric Addison's cardiomyopathy; extends sparse case reports but leaves open prospective validation.
A 13 year-old girl with clinical features of Addison's disease developed acute cardiac failure after initiation of treatment and after initial clinical improvement. Large doses of i.v. hydrocortisone and oral fludrocortisone, in addition to inotropic and ventilatory support, were required to achieve cardiovascular stability. The cardiomyopathy improved over one week and her condition then remained stable on oral glucocorticoid and mineralocorticoid replacement therapy. Reversible cardiomyopathy is a rare and potentially life-threatening complication of Addison's disease. The second reported paediatric patient is presented, the only one reported to require ventilatory support.
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Conwell et al. (2003) conducted a review in Adrenal Insufficiency-associated Cardiomyopathy (n=24). Glucocorticoid and mineralocorticoid replacement therapy was evaluated on Recovery of cardiac function and resolution of heart failure symptoms. Glucocorticoid and mineralocorticoid replacement therapy rapidly reversed left ventricular dysfunction and resolved heart failure symptoms in patients with adrenal insufficiency-associated cardiomyopathy.
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