Key result
Tumor resection and postoperative glucocorticoid management led to the normalization of body weight and myocardial mass in an infant with severe hypertrophic obstructive cardiomyopathy.
Why the study?
Does tumor resection and glucocorticoid management improve hypertrophic obstructive cardiomyopathy in an infant with an adrenocortical tumor?
Case Report (n=1)
Does tumor resection and glucocorticoid management improve hypertrophic obstructive cardiomyopathy in an infant with an adrenocortical tumor?
This is the first report describing severe hypertrophic obstructive cardiomyopathy caused by an adrenocortical tumor in an infant, which resolved after tumor resection and glucocorticoid management.
Answers are grounded in this paper and cite their sources.
May identify a reversible cause of infantile hypertrophic obstructive cardiomyopathy; hypothesis-generating and requires confirmation in larger cohorts.
Nonfamilial cardiomyopathies in childhood have been only sporadically ascribed to endocrine disorders. We report on a 4-month-old male infant presenting with Cushing's syndrome associated with excessive body weight (8.9 kg; >97th percentile) and features of virilization (Tanner stage 2 for pubic hair development). Abdominal sonography showed a large adrenal tumor. Echocardiography revealed myocardial hypertrophy with severe subaortic obstruction. Blood tests showed excessive androgen and cortisol serum levels with absent circadian rhythm as well as suppressed corticotropin. Urine catecholamine levels were within the normal range. Tumor resection with general anesthesia was performed after preparation with antihypertensive and anticongestive drug therapy. Continuous intravenous hydrocortisone substitution was started intraoperatively and subsequently tapered and switched to oral administration after 12 days. A gradual reduction in glucocorticoid substitution and its discontinuation after a total duration of 9 months were well tolerated. Histopathologic workup revealed an adrenocortical tumor of intermediate dignity. Postoperative tumor staging excluded both residual primary tumor and metastases. Both a normalization of body weight and myocardial mass were observed. The present article is, to our knowledge, the first to describe severe hypertrophic obstructive cardiomyopathy caused by an adrenocortical tumor and provides novel detailed data on postoperative glucocorticoid management.
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Hauser et al. (2013) conducted a case report in Hypertrophic Obstructive Cardiomyopathy and Adrenocortical Tumor (n=1). Tumor resection and glucocorticoid substitution was evaluated on Normalization of body weight and myocardial mass. Tumor resection and postoperative glucocorticoid management led to the normalization of body weight and myocardial mass in an infant with severe hypertrophic obstructive cardiomyopathy.
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