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Mid-ventricular Takotsubo cardiomyopathy caused by myasthenic crisis

Session Clinical Case Corner 4 - The great fire: inflammation of the heart and beyond

Speaker Tjasa Furlan

Event : Heart Failure 2018

  • Topic : heart failure
  • Sub-topic : Clinical
  • Session type : Clinical Case Posters

Authors : T Furlan (Trbovlje,SI), S Poznic (Trbovlje,SI), D Kosuta (Trbovlje,SI), B Leskovar (Trbovlje,SI)

T Furlan1 , S Poznic1 , D Kosuta1 , B Leskovar1 , 1Trbovlje General Hospital, Department of Internal Medicine - Trbovlje - Slovenia ,

Acute Heart Failure - Clinical

Myasthenic crisis is an autoimmune condition, characterized by muscle weakness that leads to respiratory failure. Takotsubo cardiomyopathy is a syndrome, characterized by profound but reversible left ventricular dysfunction in the absence of coronary artery disease.
Case report
We describe a case report of a 66-year-old man with arterial hypertension, COPD and myasthenia gravis treated with pyridostigmine who developed Takotsubo cardiomyopathy and concomitant myasthenic crisis that were triggered by a physically and emotionally stressful event. On admission, he presented with mid-sternal chest pain and severe shortness of breath leading to respiratory failure requiring intubation. Electrocardiogram showed ST elevation in lateral leads and echocardiography showed diffuse hypokinesia. His troponin level was elevated, while cardiac catheterization revealed no significant coronary artery disease. Left ventriculogram showed mid-ventricular akinesia with reduced overall left ventricular systolic function suggestive of Takotsubo stress cardiomyopathy. He transiently required intra-aortic balloon pump, but his condition improved quickly and was extubated on the second day. Plasmapheresis or immunoglobulin therapy were not required. Control echocardiogram seven days after acute presentation showed complete resolution of cardiac function with normal left ventricular chamber size, normal ejection fraction and no regional wall motion abnormalities.
There are only a few case reports describing causative associations between myasthenic crisis and Takotsubo cardiomyopathy. In our case it seems that a severe physical and emotional stress triggered both myasthenic crisis and Takotsubo cardiomyopathy. Takotsubo cardiomyopathy is a clinical syndrome characterized by transient left ventricular systolic dysfunction, mimicking myocardial infarction. It is most commonly presented as apical ballooning on left ventriculogram, but our patient had a less common variant with ventricular hypokinesis restricted to the mid-ventricle and relative sparing of the apex (mid-ventricular type). It is generally a transient disorder that is managed with supportive therapy. Myasthenic crisis is a life-threatening condition with respiratory failure due to weakness of respiratory muscles. It can be precipitated by various factors, most often concurrent infection but physical and emotional stress have also been described as a trigger. General measures are supportive therapy and intravenous immunoglobulins and plasma exchange as primary pharmacologic therapies. In our case, we observed quick resolution of symptoms with supportive measures only.
Takotsubo cardiomyopathy can be a rare presentation of myasthenic crisis, both can be precipitated by physical or emotional stress and managed with supportive therapy only.

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