A rare case of Cushing’s syndrome presenting with dilated cardiomyopathy

Abstract

Abstract Introduction: Cushing's syndrome (CS) is characterized by excessive cortisol production. Notably, 50% of deaths were due to cardiovascular disease. CS may associate with cardiomyopathies (CMPs), whose pathophysiology is poorly understood. We describe a patient presenting with acute heart failure and recently diagnosed with CMP who was found to have hypercortisolism secondary to an adrenal adenoma. Case: A 31-year-old male patient was admitted to the cardiology department due to dyspnea. He had a weight gain of 25 in the last year. His blood pressure was 135/85 mmHg, and his pulse rate was 102bpm. He had central obesity (body mass index: 31 kg/m2), buffalo hump, and abdominal purple-blue wide striae. Transthoracic echocardiography revealed global hypokinesia [left ventricle end-diastolic diameter of 6 cm/m2 (normal range < 3.2 cm/m2) on M-mode], and 20%of LVEF (standard: 50–70%). Laboratory evaluation revealed increased plasma cortisol levels with an absence of circadian rhythm. Serum cortisol levels were not suppressed after the overnight 1 mg and two-day low-dose dexamethasone suppression test (LDDST). The hormonal evaluation indicated ACTH-independent CS (Table-1). Magnetic-resonance imaging of the adrenal gland revealed a 37x28mm adenoma in the lateral limb of the left adrenal gland. The patient was diagnosed with CS. Metyrapone was used to reduce cortisol levels before the surgery. The plasma cortisol level decreased to 14.8 µg/dL on the 20th day of metyrapone treatment. Although the ejection fraction of the left ventricle was still 40% on echocardiography (Table-2), improvement of heart failure symptoms was achieved (New York Heart Association (NYHA) class II). After reducing plasma cortisol levels and improving the EF, he underwent a successful left adrenalectomy procedure. Clinical and laboratory findings improved significantly soon after surgery. The pathology result was reported as diffuse nodular hyperplasia. The patient's LVEF was 50% at the 6th month follow-up and > 60% at the 1st year follow-up. Conclusion: CS CMP is a reversible entity induced by hypercortisolism. It is characterized by cell hypertrophy, myofibrillolysis, and myocardial fibrosis that revert after adrenalectomy. In addition, preoperative anesthesia risk may increase due to low LVEF because of the CMP. Therefore, patients may need to be treated preoperatively with rapid-acting adrenolytic drugs.