Torsade de Pointes Associated with Recurrent Ampulla Cardiomyopathy in a Patient with Idiopathic ACTH Deficiency

Abstract

cured without relapse. This cardiomyopathy is often triggered by the exposure to emotional, psychological, or physical stress, and is sometimes complicated by various endocrine disorders such as anorexia nervosa [2], pheochromocytoma [3], or adrenal insufficiency [4,5]. A small number of previous reports have referred to ampulla cardiomyopathy caused by adrenal insufficiency, which resolved promptly after steroid hormone replacement therapy without severe involvement. We herein report a patient with torsade de pointes worsening to ventricular fibrillation associated with recurrent ampulla cardiomyopathy, who was rescued by cardiopulmonary resuscitation including cardiac defibrillation and later proven to suffer from idiopathic ACTH deficiency. So far, this is the first report describing adrenal insufficiency induced torsade de pointes complicated by recurrent ampulla cardiomyopathy. Abstract. We describe here a patient with torsade de pointes associated with recurrent ampulla cardiomyopathy, who was later proven to suffer from idiopathic acTh deficiency. a 70-year-old man was admitted to our hospital for bacterial pneumonia. A cardiac examination performed on admission revealed ampulla cardiomyopathy, which improved spontaneously as the pneumonia was cured. Two months after discharge, he was transferred to our hospital for relapse of the pneumonia. After the second admission, the pneumonia subsided with antibiotic treatment and his general condition ameliorated gradually. However, on the 20 th hospital day, he was found lying on the floor in a prone position in cardiopulmonary arrest. cardiac telemetry monitoring showed torsade de pointes worsening to ventricular fibrillation, and immediate cardiac defibrillation was performed. The electrocardiogram after successful defibrillation showed inverted T waves in the chest leads with long QT intervals, and subsequent emergent coronary catherization revealed the recurrence of ampulla cardiomyopathy. Thereafter, endocrinological examinations for the diagnosis of sustained hyponatremia demonstrated secondary adrenal insufficiency caused by idiopathic acTh deficiency. The cardiomyopathy resolved promptly after steroid hormone replacement without relapse as did the hyponatremia. Patients with ampulla cardiomyopathy or ventricular fibrillation without apparent etiology should be examined for adrenal function. If begun as soon as adrenal insufficiency is diagnosed, immediate steroid replacement therapy can prevent the deterioration and relapse of cardiac involvement.

Isolated ACTH deficiency presenting with severe myocardial dysfunction

“…The apical ballooning in Takotsubo cardiomyopathy generally resolves spontaneously within several days to weeks. It remains unclear whether steroid replacement therapy is indispensable for the wall motion recovery in Takotsubo cardiomyopathy, as one of the reported cases has shown spontaneous improvement without glucocorticoid therapy [17]. As for the present case, serial electrocardiograms showed no ST-segment changes and echocardiogram demonstrated diffuse wall motion abnormalities instead of apical ballooning.…”

Prolonged QT Intervals in Isolated ACTH Deficiency: Case Report and Mini Review of Literature

“…ECG abnormalities include flat or inverted T waves, prolonged QT intervals, low voltage, prolonged PR or QRS intervals, and depressed ST segment. In some cases, these ECG changes cause lethal arrhythmia (Table 1) [5][6][7][8][9][10]. Mean QTc intervals is 0.55 seconds.…”

“…Since the reports in the 1950s, ECG abnormalities such as flat or inverted T waves, prolonged QT intervals are known to occur in adrenal insufficiency [2][3][4]. There are six reports suggesting that sudden cardiac arrest occurs in IAD due to prolonged QT intervals [5][6][7][8][9][10]. Although ECG abnormalities are modulated after hydrocortisone replacement, the mechanism by which hydrocortisone may affect ECG still remains unclear.…”

Congenital Long-QT Syndrome in Addison’s Disease: A Novel Association

“…The association between cortisol deficiency and acquired QTc syndrome has been reported as far back as the 1950s [8]. Theories of this association include functional disturbance of cardiac sodium-calcium pumps and cellular overhydration [4]. The cornerstone of chronic management for any acquired LQTS is treatment of the underlying cause.…”