Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2013) 32 P256 | DOI: 10.1530/endoabs.32.P256

ECE2013 Poster Presentations Clinical case reports – Pituitary/Adrenal (57 abstracts)

Isolated ACTH deficiency with Brugada syndrome: a combination increasing the risk of fatal arrhythmia

Naoko Kumagai 1 , Kazufumi Honda 1, , Yohei Muroya 1, , Masanori Shimodaira 1, , Kaoru Tsuzawa 1, , Erisa Sorimachi 1, , Akira Imamura 1 & Keita Ishii 1

1Moriya Keiyu Hospital, Ibaraki, Japan; 2Tokyo Metropolitan Hiro-o Hospital, Tokyo, Japan.

Introduction: Brugade syndrome (BS) is a cardiac disorder characterized by typical electrocardiograph (ECG) alterations and is known to have a high risk of sudden cardiac death (SCD). Isolated ACTH deficiency (IAD) is often associated with fetal arrhythmia. Previous reports have suggested that certain endocrine diseases might cause Brugada-like ECG forms. We present a case of IAD with BS who died suddenly.

Case presentation: Forty-one year-old man was referred to our department because of severe hypoglycemia in July 2011. An arrhythmia was pointed out at his annual medical checkup in 2010 and he was diagnosed as having BS by cardiologists. The catheter ablation was done twice. After his second ablation, he lost consciousness due to severe hypoglycemia and was referred to our department. The endocrinological investigation revealed severe disturbance in ACTH secretion and mild disturbance in GH secretion. Slight elevations of thyrotropin (TSH) were observed but anti-thyroid antibodies were negative. Both TSH and GH secretion were normalized after the replacement with hydrocortisone and so he was diagnosed as IAD. Five months after the beginning of the replacement therapy, he died suddenly.

Discussion: BS is based on genetic defect concerning cardiac ion channels. On the other hand, several reports indicate that changes of thyroid hormone and cortisol and/or hyponatremia caused by adrenal insufficiency could induce Brugada-like ECG forms through the functional disorders in cardiac ion channels. In addition, abnormalities of ion channel function have been found in some patients with Schmidt syndrome. These reports suggest that IAD could influence in cardiac ion chancel function in this case, though the detail of functional connection between these hormone and myocardial ion channels remains unclear in humans.

Conclusion: This case shows us possibility that IAD can increase risk of fatal arrhythmia through its actions to cardiac ion channels.

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