Dilated Cardiomyopathy Revealing Cushing Disease

Abstract

Cardiovascular impairments are frequent in Cushing's syndrome and the hypercortisolism can result in cardiac structural and functional changes that lead in rare cases to dilated cardiomyopathy (DCM). Such cardiac impairment may be reversible in response to a eucortisolaemic state.A 43-year-old man with a medical past of hypertension and history of smoking presented to the emergency department with global heart failure. Coronary angiography showed a significant stenosis of a marginal branch and cardiac MRI revealed a nonischemic DCM. The left ventricular ejection fraction (LVEF) was estimated as 28% to 30%. Clinicobiological features and pituitary imaging pointed toward Cushing's disease and administration of adrenolytic drugs (metyrapone and ketoconazole) was initiated. Despite the normalization of cortisol which had been achieved 2 months later, the patient presented an acute heart failure. A massive mitral regurgitation secondary to posterior papillary muscle rupture was diagnosed as a complication of the occlusion of the marginal branch. After 6 months of optimal pharmacological treatment for systolic heart failure, as well as treatment with inhibitors of steroidogenesis, there was no improvement of LVEF. The percutaneous mitral valve was therefore repaired and a defibrillator implanted. The severity of heart failure contraindicated pituitary surgery and the patient was instead treated by stereotaxic radiotherapy.This is the first case reporting a Cushing's syndrome DCM without improvement of LVEF despite normalization of serum cortisol levels.

Successful Management of Acute Congestive Heart Failure by Emergent Caesarean Section Followed by Adrenalectomy in a Pregnant Woman with Cushing's Syndrome-induced Cardiomyopathy

“…An association between CS and DCM has been reported in several cases (3,14,17). Although the underlying mechanism remains unclear, some studies have suggested that a direct effect of cortisol on the myocardium through glucocorticoid receptors is involved in the cardiac remodeling of CS, independent of hypertension (20,21).…”

“…CS is associated with increased mortality and morbidity, including cardiovascular disorders (2). Several cases of dilated cardiomyopathy (DCM) with or without congestive heart failure (CHF) have been reported (3). However, the management of pregnancy in patients with CS is accompanied by several issues.…”

Cushing syndrome cardiomyopathy: an unusual manifestation of small‐cell lung cancer

“…In summary, this case is an interesting presentation of CHF with refractory hypokalaemia, which prompted further evaluation and ultimately led to the diagnosis of ectopic ACTH production by SCLC. Most of the previously described cases of CS cardiomyopathy have been associated with pituitary and adrenal tumours 15 . CS cardiomyopathy is classically characterized by eccentric or concentric LV hypertrophy 3,4 .…”

“…To date, little information is available on the development of CS cardiomyopathy and the risk of developing this syndrome in the context of SCLC. Much of the existing literature is a sampling of case reports describing probable downstream effects of cortisol on the myocardium 15 . In particular, most of these case reports of CS cardiomyopathy involve either pituitary adenomas or adrenal tumours 15 .…”

“…Much of the existing literature is a sampling of case reports describing probable downstream effects of cortisol on the myocardium 15 . In particular, most of these case reports of CS cardiomyopathy involve either pituitary adenomas or adrenal tumours 15 . This is a rare case to describe CS cardiomyopathy associated with a paraneoplastic SCLC.…”