Autoimmune polyendocrine syndrome II presenting paradoxically as Takotsubo cardiomyopathy: a case report and reappraisal of pathophysiology

Abstract

Background Takotsubo cardiomyopathy (TCM) is a rare disease entity characterized by acute, non-ischemic, reversible myocardial dysfunction that mimics acute myocardial infarction. Activation and excessive outflow of sympathetic nervous system are believed to be central to the figure in the disease pathogenesis. Adrenocortical hormones potentiate the systemic actions of sympathetic nervous system and accordingly are essential for regulation of myocardial function. We present an unusual case of a middle-aged woman with primary adrenal insufficiency who presented paradoxically with TCM. Case presentation A 50-year-old woman with past history of hypothyroidism presented to emergency department with history of acute chest pain and syncope. There was no significant drug history or history of an emotional or physical stimulus prior to admission. Prominent pigmentation over the tongue and skin creases of hands were noted. On presentation, she was in shock and had ventricular tachycardia which required electrical cardioversion. The subsequent electrocardiogram demonstrated diffuse T-wave inversions with prolonged QTC. There was apical hypokinesia on echocardiogram, and cardiac biomarkers were elevated. There was persistent inotropic requirement. She had marked postural symptoms, and a postural blood pressure drop of 50 mm Hg was present. Initial laboratory parameters were significant for hyperkalemia (7.8 mEq/L) and hyponatremia (128 mEq/L). These findings prompted evaluation for adrenal insufficiency which was confirmed with appropriate tests. Autoimmune polyendocrine syndrome II was thus diagnosed based on the above findings. Coronary angiography revealed normal coronaries. The diagnoses of TCM was established in accordance with the International Takotsubo Diagnostic Criteria. She was started on stress dose steroid replacement therapy and improved dramatically. At one month of follow-up, the patient is asymptomatic, and there was normalization of her left ventricular function. Conclusions Intricate relationship and interplay exist between the steroid hormones and catecholamines in the pathogenesis of TCM. Steroid hormones not only potentiate the actions of catecholamines, but they also regulate and channelize catecholaminergic actions preventing their deleterious effects on the cardiac tissue. Hence, both steroid deficiency and exogenous steroid replacement may precipitate TCM. Evidence from more such cases and larger perspective studies in future will further improve our understanding of this complex disease process and its myriad associations.

Takotsubo Syndrome After Alcohol Withdrawal in a Patient With Suspected Alcoholic Cardiomyopathy

“…The other unique aspect was that takotsubo syndrome further developed under the condition of suspected alcoholic cardiomyopathy. Takotsubo syndrome resembles anterior acute myocardial infarction in initial symptoms and ECG changes [ 1 - 5 ]. In the present case, computed tomography had already revealed the absence of significant coronary artery disease during the first hospitalization.…”

Postoperative Apical Ballooning Syndrome Following Orthotopic Liver Transplantation

“…The typical variant of ABS is characterized by mid and apical left ventricle (LV) segments hypokinesis in the absence of any evidence of coronary artery ischemia along with preserved basal LV segment’s function manifested classically as apical ballooning, resembling the takotsubo pot, a pot historically used to catch the octopuses in Japan. ABS has been reported following major surgical procedures that pose significant stress on the heart [ 6 - 7 ]. It may present with chest pain, dyspnea, palpitations, and peripheral edema and might be severe enough to result in cardiogenic shock.…”

Reverse Takotsubo Cardiomyopathy in a Critically Ill Patient in the ICU: A Case Report With Literature Review

“…Dysregulated corticosteroid hormonal balance leads to a maladaptive catecholaminergic response in cardiac tissue and is yet another important determinant of TTC. Hence, both excess and deficiency of corticosteroids can lead to TTC and rTTC [ 13 ].…”