INTRODUCTION: Adrenal crisis is a true medical emergency requiring prompt recognition and treatment. It is characterized by insufficient steroid hormone release to meet the body's physiological needs. However, cardiomyopathy in adrenal crisis is not well described.
CASE PRESENTATION: 35-years old Ecuadorian male presented to the ER with complaints of nausea, vomiting, diarrhea and vague epigastric discomfort for four days. He also had significant weight loss and lack of energy over the past 2 months. Past medical and family histories were unremarkable. On physical exam he was cachectic. Blood pressure 80/42mmHg; heart rate was 130 beats/min, respirations 26 breaths/min and oral temperature of 97.2oF. Cardiovascular and lung examination was normal. Abdomen was soft, mildly tender in the epigastrium. He was alert and orientated with no focal neurological deficits. EKG showed sinus tachycardia. Serum sodium-124mmol/L; potassium-5.9mmol/L; calcium-10.0mmol/L; BUN-44mg/ml: creatinine-l.9mg/dl, glucose-46mg/dl, Hb-14.3gm/dl, WBC-16000/mm3 with 49% neutrophils, and CK-116U/L with CKMB-1.6ng/mL and troponin I-0.0ng/ml. Despite aggressive IV fluid resuscitation, blood pressure continued to drop and norepinephrine was added. As Adrenal crisis was suspected, IV hydrocortisone was started. CT scan of the chest showed patchy areas of scaring and infiltrate in the lung apices. Upper abdominal cuts showed bilateral calcification of adrenal glands. On the second day he complained of chest pain. EKG was unremarkable. However, Troponin-I increased to 26.8ng/dl. Echocardiogram revealed an ejection fraction (EF) of 37%, with dilated left ventricle. Cardiac catheterization revealed normal coronary arteries, EF of 15% and pulmonary artery wedge pressure of 22mmHg. Patient's cortisol level came back as <0.5ug/dl. PPD was positive. Because of high clinical suspicion for tuberculosis, he was started on Rifampin, Isoniazid, Pyrazinamide, and Ethambutal. On subsequent days he showed significant clinical improvement. A repeat echocardiogram on day 10 revealed an EF of 50%, with improvement in Left ventricular diameter (LVD). Sputum cultures came back positive for Tuberculosis.
DISCUSSIONS: Cortisol is an important mediator in maintenance of homeostasis and an essential component to stress. Depressed levels are associated with collapse of vascular tone, derangements of vascular permeability and disruption of distribution of total body water. These abnormalities lead to a state of shock that is not responsive to fluids or pressors. Cardiomyopathy in adrenal insufficiency has previously been reported in adults (1,2). Cortisol has many well documented physiological effects. Seldom described is its effect on the myocyte. Dexamethasone has been shown to significantly enhance the contractile tension and increase the velocity of contraction and relaxation in cardiac muscles. Dexamethasone also increases Ca-ATPase activity and level of Ca2+/calmodulin-dependent protein kinase II in cardiac sarcoplasmic reticulum vesicles in adrenalectomized rats (3).
CONCLUSION: Depressed levels of cortisol can lead to the impairment of both contraction and relaxation of the cardiac myocytes, resulting in impaired cardiac function. Cardiomyopathy may be more prevalent in patients with adrenal insufficiency and it may play a larger role in the acute presentation of adrenal crisis. As it is resolved with administration of corticosteroids the cardiomyopathy may simply go unnoticed. This finding may also explain why patients in shock from adrenal crisis respond so well to corticosteroids.
(1) Afzal A, Reversible cardiomyopathy associated with Addison's disease. Can J Cardiol 2000; 16(3):377-379.2.
(2) Eto K, Adult reversible cardiomyopathy with pituitary adrenal insufficiency caused by empty sella". Angiology, 2000; 151,319-323.
(3) Rao MK, Glucocorticoid modulation of protein phosphorylation and sarcoplasmic reticulum function in rat myocardium. Am J Physiol Heart Circ Physiol 2001;281: H325-H333
DISCLOSURE: Rana Ali, None.
Rana Y. Ali MD * Basir Haque MD Kevin Irish S Hilwa Farhad Arjomand MD The Brooklyn Hospital Center, Brooklyn, NY
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