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Dilated Cardiomyopathy Revealing Cushing Disease: A Case Report and Literature Review
Lucien Marchand1, Bérénice Segrestin, Marion Lapoirie
1From the Department of Endocrinology, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France (LM, BS, ML, GR); Department of Radiotherapy, Centre Hospitalier de Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France (VF); Department of Cardiology, Tonkin clinic, Villeurbanne, France (JD); Department of Neurosurgery, Groupement Hospitalier Est, Hospices Civils de Lyon, Bron, France (EJ); and INSERM U1028, CNRS UMR5292, Lyon Neuroscience Research Center, Neuro-Oncology and Neuro-Inflammation Team, Lyon, France (EJ, GR).
Insights
Cushing's syndrome can cause heart failure, leading to dilated cardiomyopathy (DCM). This case shows DCM in Cushing's disease did not improve even after normalizing cortisol levels.
Area of Science:
- Cardiology
- Endocrinology
- Cardiovascular Medicine
Background:
- Cushing's syndrome, characterized by hypercortisolism, frequently causes cardiovascular impairments.
- These can manifest as structural and functional cardiac changes, rarely progressing to dilated cardiomyopathy (DCM).
- Cardiac dysfunction in Cushing's syndrome is often considered reversible upon achieving a eucortisolaemic state.
Observation:
- A 43-year-old man with hypertension and smoking history presented with global heart failure.
- Cardiac MRI revealed nonischemic DCM with reduced left ventricular ejection fraction (LVEF) of 28-30%.
- Investigations confirmed Cushing's disease, and adrenolytic drugs were initiated.
Findings:
- Despite cortisol normalization within 2 months, the patient experienced acute heart failure.
- Massive mitral regurgitation due to posterior papillary muscle rupture, a complication of marginal branch occlusion, was diagnosed.
- There was no improvement in LVEF after 6 months of optimal medical therapy and steroidogenesis inhibitors.
Implications:
- This is the first reported case of Cushing's syndrome-associated DCM where LVEF did not improve despite normalized cortisol levels.
- The patient underwent percutaneous mitral valve repair and defibrillator implantation due to severe heart failure.
- Pituitary surgery was contraindicated; stereotactic radiotherapy was used as an alternative treatment for Cushing's 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.
