Isolated ACTH deficiency presenting with severe myocardial dysfunction

Abstract

We present a case of isolated adrenocorticotropic hormone (ACTH) deficiency complicated by acute adrenal crisis and severe myocardial dysfunction. A 54-year-old woman developed consciousness disturbance, hypoglycemia, hyponatremia, and rhabdomyolysis. Initial echocardiographic examinations on the sixth hospital day revealed marked right-sided atrial and ventricular dilatation and severe tricuspid regurgitation. A computed tomography scan for pulmonary embolism was negative. On the 14th hospital day, she became dyspneic and hypotensive. Repeated echocardiographic examinations demonstrated diffuse and severe hypokinesis of the left ventricle. The previous right-sided chamber dilatation became less apparent. Congestive heart failure and severe hypotension were refractory to catecholamines, while she was eventually diagnosed as having acute adrenal crisis due to isolated ACTH deficiency. Hydrocortisone replacement therapy was started, and echocardiographic examinations revealed that the left ventricular dysfunction completely returned to normal in the following eight days. Severe myocardial dysfunction is an uncommon but serious complication of acute adrenal insufficiency. The present case was unique in that diffuse left ventricular dysfunction was preceded by right ventricular dysfunction.

Keywords: Adrenal crisis; Cardiomyopathy; Isolated adrenocorticotropin deficiency; Myocardial dysfunction; Neuroleptic malignant syndrome.

Figure 1

Initial echocardiographic examinations performed on the sixth hospital day demonstrate right-sided atrial and ventricular dilatation. The left ventricle is compressed at end-diastole by the dilated right ventricle. Severe tricuspid regurgitation is shown on the right of panel C. (A) Parasternal long-axis view. (B) Parasternal short-axis view. (C) Apical four chamber view. (Left: end-diastole, right: end-systole.)

Figure 2

Repeated echocardiographic examinations performed on the 14th hospital day demonstrate diffuse and severe hypokinesis of the left ventricle along with right ventricular dysfunction. Massive pleural effusion resulting from congestive heart failure is seen. (A) Parasternal long-axis view. (B) Parasternal short-axis view. (C) Apical four chamber view. (Left: end-diastole, right: end-systole.)