MON-404 Reversible Dilated Cardiomyopathy in a Severe Case of Cushing’s Disease

Abstract

Introduction: Patients with Cushing’s syndrome (CS) present a higher mortality compared with the general population mainly due to cardiovascular and infectious diseases. Major forms of cardiac involvement include left ventricular hypertrophy, myocardial ischemia, and, more rarely, reversible dilated cardiomyopathy. This last condition is not fully understood, but it seems to be related to the direct action of cortisol, promoting increase of myocardial responsiveness to several factors involved in myocardial remodeling. Case report: A 18 yrs-old, male, was admitted with a four years clinical picture of progressive weight gain, moon face with plethora, proximal weakness with muscular atrophy, diffuse cutaneous violaceous striae, depressive symptoms and fragility fractures. Arterial hypertension started three years after. Two months before admission, he presented dyspnea, orthopnea and anasarca. At admission, tachycardia, tachypnea, arterial pressure of 170X100 mmHg, oxygen saturation of 83%, bilateral thoracic crackling, diffuse edema reaching inguinal region and BMI of 38.3 kg/m2. Hormonal analyses confirmed severe ACTH-dependent CS and pituitary MRI disclosed an expansible lesion of 1.6 cm suggestive of macroadenoma. Initial transthoracic echocardiography showed dilated left cavities, normal myocardial thickness but with moderate systolic dysfunction due to diffuse hypokinesia with ejection fraction (EF) of 45%. Patient was submitted to pharmacological approach to treat heart failure (HF) with furosemide, carvedilol, enalapril and spironolactone. Other etiologies for myocardiopathy such as infectious, autoimmune and metabolic diseases were excluded. Successful pituitary surgery was done in November 2016. The patient presented progressive improve of cardiac symptoms after all the treatments but mainly after pituitary surgery. Subsequent echocardiography 15 months after showed normal cardiac cavities, normal left ventricle and myocardial thickness and preserved global and segmental systolic function, EF of 56%. In the last evaluation (two years after surgery), the patient was using oral hydrocortisone twice/day and losartan Conclusion: Dilated cardiomyopathy is an uncommon clinical morbidity of CS. However, the immediate recognition of this condition and its effective treatment including control of hypercortisolism may reverse this life-threatening clinical condition