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Takotsubo cardiomyopathy (TC) is a transient systolic dysfunction typically involving the apical segment of the left ventricle with no obstructive coronary artery disease. Its pathogenesis is debated and attributed to a coronary and peripheral vasospasm caused by an adrenergic overstimulation, metabolic alterations, or inflammatory mechanisms all triggered by emotional and physical stressors [1]. Myasthenia gravis (MG) has been associated with TC, especially when a myasthenic crisis (MC) occurs [2].
We report the case of a 57-year-old woman admitted to our clinic for worsening of her dysphagia, dysarthria, and dysphonia to undergo a course of plasma exchange. She had a 5-year history of MG (Ossermann IIB) with a low title of anti-acetylcholine receptor antibodies. In her medical history, she also had hypertension, type II diabetes mellitus, autoimmune thyroiditis with residual hypothyroidism, and allergic asthma. From 2018 to 2021, she was treated with prednisone (15 mg/die) and pyridostigmine (240 mg/die in four administrations) with a good control of symptoms. In 2021, after a bulbar relapse occurred 7 days after the second dose of COVID-19 vaccine (Pfizer-tozinameran) she started treatment with intravenous immunoglobulins (2 days/month). At the time of admission, 1 year after the vaccination, the patient showed pronounced dysarthria, dysphonia, and weakness of the cervical extensor muscles. She also complained orthopnea, but no other neurological signs were detected, and the arterial blood gas analysis was normal. Due to dysphagia for both solids and liquids, a nasogastric tube was proposed which the patient refused. Oral pyridostigmine was therefore switched to intramuscular neostigmine (1.5 mg/die in six administrations) and oral prednisone to intravenous methylprednisolone (40 mg/die). Therapeutic plasma exchange was started on day 2 from admission. On day 3, before starting the second session of plasma exchange, the patient had a transient loss of consciousness associated with dyspnea, tachycardia, hypersalivation, and cutaneous vasoconstriction. Blood pressure was normal; pupils were dilated and reactive to light; and no signs of infection were detected. Blood tests were normal, except for a mildly reduced arterial oxygen saturation (pO2 75 mmHg). An ECG showed an antero-lateral ST segment elevation with ST depression in the inferior leads (Fig. 1). Bedside echocardiography showed a mid-apical akinesia with ejection fraction of 35%, and a coronarography documented a mid-left ventricular akinesia with basal and apical hyperkinesia in the absence of significant coronary artery disease (Fig. 2). An atypical mid-ventricular Takotsubo cardiomyopathy was diagnosed. At the end of the procedure, the patient was admitted to the intensive care unit for acute respiratory failure, and the course of five plasma exchange was completed. A nasogastric tube was placed and pyridostigmine 60 mg × 6/die was administered instead of neostigmine.
Because of ventilatory weaning, tracheostomy was performed and then removed on day 21 of hospitalization. After 10 days, the patient was transferred to the rehabilitation unit and then discharged home with mild residual dysarthria on day 32 from admission. Echocardiography performed at that time showed normal left ventricular function.
Whether MC and TC can be considered causatively associated or concomitant events due to partial overlapping of common triggers is difficult to unveil [3], and the pathophysiology of TC during a MC is yet not fully understood [2]. The role of catecholamine surge triggered by emotional and physical stressors has been proposed, as well as a cardiac dysfunction secondary to MG-related autoimmune mechanisms, reversible vasospasm, microcirculatory dysfunction, and respiratory infections [4]. Therapy includes supportive and symptomatic treatment [1]. Beta-adrenergic and calcium channel antagonists should be handled carefully for the risk of triggering a MC [2].
Different variants of TC have been described, including a classic form with apical ballooning accounting for about the 80% of cases and atypical subtypes including basal, focal, or mid-ventricular TC [5] as in our case presentation. Atypical forms have different clinical characteristics, compared to the classic variant, such as a younger age of onset, a different ECG presentation, and a higher prevalence of association with central and peripheral neurologic disease [5]. Clinical outcomes after 1 year from the event are comparable across all the forms, although TC with neurological causes may have a higher mortality rate [5]. As previously shown, MC-associated TC may present as both classic and atypical forms but in a previous systematic review, the mid-ventricular subtype has been reported only in one case in association with a subsequent relapse of classic TC [2]. To the best of our knowledge, we described the first isolated case of MC-associated mid-ventricular TC.
As pointed out in a population-based study, patients with TC following MC experienced two times higher odds of all-cause in-hospital mortality, compared to TC following different triggers, including a higher rate of respiratory failure requiring mechanical ventilation and arrhythmia [4]. A significantly increased risk for respiratory complications in patients with MC is well known, and an overall worsening of outcomes and higher resource utilization due to TC-related complications in MC patients seems likely [4]. As a result, the need to be aware and prevent TC following MC in patients with MG appears to be a meaningful issue, and diagnostic and prognostic implications of different presentations of variants of TC in these patients are still unknown, and further studies are needed to unravel this issue.
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References
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Antenucci, P., Padroni, M., De Gennaro, R. et al. An atypical Takotsubo cardiomyopathy in a typical myasthenic crisis. Neurol Sci 44, 3747–3749 (2023). https://doi.org/10.1007/s10072-023-06844-3
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DOI: https://doi.org/10.1007/s10072-023-06844-3