Yonsei Med J.  2012 May;53(3):658-661. 10.3349/ymj.2012.53.3.658.

Male Pseudohermaphroditism Presented with Sudden Cardiac Arrest

  • 1Division of Cardiology, Severance Cardiovascular Hospital, Yonsei University College of Medicine, Seoul, Korea. cby6908@yuhs.ac


Torsades de Pointes is a life-threatening arrhythmia associated with a number of causes, but is very rare among endocrinologic disorders. We report a case of male pseudohermaphroditism with hyperaldosteronism due to a 17alpha-hydroxylase deficiency presented with sudden cardiac arrest.


Torsades de Pointes; pseudohermaphroditism; 17alpha-hydroxylase deficiency

MeSH Terms

46, XY Disorders of Sex Development/*diagnosis/drug therapy
Death, Sudden, Cardiac/*etiology/*pathology


  • Fig. 1 ECG and arterial blood pressure recording. (A) The 12-lead ECG at the emergency room shows a markedly prolonged QT interval (QTc=635 ms). (B) Spontaneous termination of Torsades de Pointes. Note the short-long-short initiation of polymorphic ventricular tachycardia. (C) Torsades de Pointes with hemodynamic compromise, which was terminated by biphasic shock of 100 J. The ABP waveform became flat after the occurrence of Torsades de Pointes. ABP, arterial blood pressure; ECG, electrocardiogram.

  • Fig. 2 Abdominal computed tomography showed bilateral hyperplasia of adrenal glands (arrows).

  • Fig. 3 Clinical course of the patient. Serial variations in plasma ACTH, systolic blood pressure (SBP), corrected QT interval (QTc), serum cortisol, and potassium (K+) are shown. ACTH, adrenocorticotropic hormone; HD, hospital day.


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