Life-threatening Takotsubo Cardiomyopathy

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Abstract

Objective: To report a case of seizure-induced takotsubo cardiomyopathy with rare etiology and rarer complications. Methods: A 50-year-old woman had multiple epileptic seizures and later developed acute heart failure complicated by ventricular fibrillation and shock. A two-dimensional echocardiogram revealed apical ballooning of the left ventricle resembling a takotsubo (a Japanese fisherman’s pot used to trap octopi). The apex was also hypokinetic. Results: The hemodynamic abnormalities normalized with defibrillation, assisted ventilation, inotropic support, and pressor agents. More importantly, the apical ballooning deformity and systolic dysfunction reversed. The echocardiogram normalized three months later. A nuclear treadmill stress test was negative for ischemia. Conclusions: Apical ballooning of the left ventricle and hypokinesis are typical echocardiographic features in takotsubo cardiomyopathy, a stress-induced heart disease. It may follow severe emotional, physical, and neurologic stressors, in our rare case, grand mal seizures (0.2 % of all takotsubo disease patients). Also rare are life-threatening complications. Based on these observations, in a case with severe stress followed by acute heart failure, takotsubo cardiomyopathy should be a major diagnostic consideration. The dramatic initial triggering event, in our case an epileptic seizure, should not mask the possibility of coexisting takotsubo cardiomyopathy. Awareness of this disease, anticipation of complications, and two-dimensional echocardiography will help channel the management in the right direction.

Correspondence
M Shenoy, MBBS, FACC, Division of Cardiology, Queens Hospital Center (B 236), 82-68 164th Street, Jamaica, NY 11452. E: shenoym@nychhc.org
Citation
American Heart Hospital Journal 2011;9(2):119-21
DOI
https://doi.org/10.15420/ahhj.2011.9.2.119

In this case report, we present a patient with takotsubo cardiomyopathy (TC) that followed grand mal epileptic seizures. TC is a stress-induced, reversible heart disease with unique clinical, two-dimensional echocardiographic and prognostic features that differ from other cardiomyopathies.

Case Report

A 50-year-old woman with grand mal epilepsy was brought to the emergency department in a state of confusion, marked agitation, and incomprehensible speech. A friend had witnessed several grand mal seizures and called an ambulance. She had stopped antiepileptic medicines abruptly on her own accord and had recurrent seizures. In retrospect, it became apparent that her abnormal behavior was due to a seizure-related state. There was no history of cardiac disorder in the past. The vital signs were a temperature of 37 °C (99 °F), blood pressure of 160/100 mm Hg, a heart rate of 150 beats/min, and a respiratory rate of 20/min. Initially, there were no cardiovascular or pulmonary system abnormalities. Within the next hour, the patient was hyperventilating and a diagnosis of post-ictal phenomenon was considered. However, with increasing respiratory distress and lung crepitations, the correct diagnosis of left heart failure was made. Simultaneously, marked ST-segment elevation was witnessed in the precordial leads of an electrocardiogram. She was admitted to the medical intensive care unit where she had another grand mal seizure.

In view of the unusual combination of neurologic and cardiac disorders, a two-dimensional echocardiogram was obtained (Figure 1). It demonstrated ballooning and severe hypokinesis of the left ventricle (LV), extending from mid-ventricular level to the apex. In contrast, the basal segments contracted vigorously. The LV ejection fraction (LVEF) was reduced to 30 % (normal 50–75 %). Based on these features, particularly the apical ballooning of the LV, we diagnosed TC. Hypotension and soon after ventricular fibrillation then occurred. The ventricular fibrillation was terminated by electrical defibrillation. Hypotension responded to pressor agents. With assisted ventilation and treatment with inotropic agents, the heart failure resolved and the ST segments became isoelectric. The routine biochemical tests were normal. The urine and blood tests were reported as negative for toxic substances. We did not measure catecholamine levels because the confounding factors of seizure, agitation, and heart failure may increase them. The patient declined our advice to have coronary angiography.

A repeated two-dimensional echocardiography obtained three months later at a clinic visit showed a dramatic normalization of the LV (Figure 2). There was no apical ballooning. The LV contour, wall motions and global systolic function (LVEF 60 %) had become normal. We performed intravenous dipyridamole myocardial perfusion imaging, which revealed no ischemic defects.

Discussion

A takotsubo is a pot with a bulbous bottom and a narrow neck used by Japanese fishermen to trap octopi. TC derives its name from the altered LV shape, specifically the apical ballooning, resembling a takotsubo. The common clinical presentations are ST elevation (in 82 % of cases), acute LV failure (68 %), dyspnea (18 %), and cardiogenic shock (4 %). Life-threatening ventricular arrhythmias may occur. Although these features mimic an acute coronary event, the actual incidence of angiographically important coronary lesions is uniformly low.1 Coronary angiography was not performed in this case because the patient declined to have this test. However, we believe that significant coronary disease was excluded by the normal exercise myocardial perfusion test images.

The pathophysiology of TC is interesting. Serious emotional or physical stresses are suggested as the etiologies of TC. Therefore, descriptive terms like ‘broken-heart syndrome’, ‘stress-induced cardiomyopathy’, and ‘catecholamine-induced cardiomyopathy’ are in use. Common causal stressors include death of a loved one, financial loss, altercation, natural catastrophe, and similar intense emotional situations.2 Current thinking is that TC results from the ‘cardio-toxic’ effects of acutely elevated circulating epinephrine and norepinephrine levels (‘catecholamine storm’) following stress. As a result, there is myocardial stunning, cell injury, and myocytolysis.3 The selective apical involvement in TC is explained by greater concentration of adrenergic receptors in the LV apex than in the rest of the LV segments.4 As further evidence of catecholamine theory, the coincidence of TC and excessive catecholamine release in pheochromocytoma and cocaine use are described in the literature.5,6

The list of physical stresses, such as traumatic injury, surgical procedures, etc., resulting in TC, is extensive. In addition to physical and emotional stresses, a number of neuropsychiatric disorders can lead to TC. They include, to name a few, subarachnoid hemorrhage,7,8 addictive substances or their withdrawal (e.g. alcohol), head trauma, epileptic seizures,9 and psychiatric disorders.10 In the absence of any other stressors, we believe recurrent epileptic seizures cause TC.

Among the 974 cases reported in the medical literature, Stöllberger and coauthors found 36 cases of epilepsy-associated TC. Of these, only two had grand mal epilepsy.9 The occurrence of ventricular fibrillation is a noteworthy complication. Ventricular fibrillation, usually a fatal arrhythmia if untreated, is extremely rare in TC, with 1.8 % prevalence among all arrhythmias.11

Conclusions

TC occurs in a dramatic setting and is often overshadowed by the precipitating stressful event. For that reason, it may be missed or misdiagnosed. When faced with the combination of severe stress (neurologic, emotional, or physical) and acute heart failure, one needs to consider TC. TC is generally reported as a benign condition. Our case of shock and ventricular fibrillation indicates otherwise. Two-dimensional echocardiography is a simple and rapid initial non-invasive test. With an echocardiographic diagnosis of TC, one is prepared to anticipate acute heart failure and emergent ventricular arrhythmias, and to channel management appropriately.

References
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