Feature

Takotsubo Cardiomyopathy, a.k.a., Transient Left Ventricular Apical Ballooning Syndrome: An Acute Coronary Syndrome Imposter

Brenda McCulloch, RN, MSN, CNS
Brenda McCulloch, RN, MSN, CNS

Case Study

A 66-year-old female presented to the ED complaining of 8/10 retrosternal chest pain and dyspnea. She was triaged quickly and her initial ECG, done within minutes of arrival, showed possible lateral infarct, T wave inversion in the precordial leads, and QT prolongation. She was given chewable aspirin 325mg and started on eptifibatide (Integrilin). Her first troponin was elevated at 3.35 ng/mL. She had no significant medical history. She was the primary caregiver for her husband with severe Alzheimer’s disease.
She was diagnosed with acute myocardial infarction and referred to cardiology for additional evaluation. She was taken to the cardiac cath lab for cardiac catheterization and possible coronary intervention. Interestingly, her coronary arteries were normal with no signs of atherosclerotic plaque (Figure 1, 2). Methergine testing was done and ruled out coronary artery spasm. The left ventriculogram was markedly abnormal, however, demonstrating significant anteroapical wall motion abnormalities. Marked systolic dyskinetic ballooning of the apex was noted (Figure 3). The LVEDP was 29 mmHg and the ejection fraction was estimated to be 40–45%.

Based on these findings, the patient’s diagnosis was revised to Takotsubo cardiomyopathy. The eptifibatide infusion was discontinued and she was transferred to the cath lab holding area. The 6 Fr femoral arterial sheath was removed and hemostasis was achieved. The patient was then transferred to the telemetry unit for continued nursing care. She remained stable and her remaining hospital course was uneventful. Her ECG on the day of discharge showed normal sinus rhythm with giant T wave inversion in leads V3-V6, and her QT continued to be prolonged at 605 ms (QTc 657 ms, Figure 4). She was discharged home on day 2 with the diagnosis of Takotsubo cardiomyopathy. Discharge medications included enteric coated aspirin 325 mg daily, Lisinopril 2.5 mg daily, Metoprolol 50 mg daily. She was instructed to see her primary care physician in one week and to follow up with cardiology in four weeks for trans-thoracic echocardiography.

Introduction
Takotsubo cardiomyopathy, also currently referred to in the medical literature as transient left ventricular apical ballooning, broken-heart cardiomyopathy, or stress-induced cardiomyopathy, is an interesting and infrequently seen phenomenon. It is thought to account for about 2% of all acute myocardial infarction presentations.1 It was originally described by the Japanese in the early 1990’s and named after the takotsubo pot, a narrow-necked, bulging pot used by Japanese fishermen to catch octopus,2 a shape they felt described the appearance of the ballooning left ventricle.
This syndrome has been reported in both genders, but it is much more common in post-menopausal women.1 Most of these women have experienced a significant psychological or physical stressor that precipitates the onset of their symptoms. The reason for this gender difference is not clear. Sex hormones may play a role in the propensity for ballooning of the left ventricle in females.

Patient Presentation

Presenting symptoms reported in Takotsubo cardiomyopathy include chest pain and/or dyspnea in most patients. Patients may also experience nausea, near-syncope, syncope or hypotension. Some patients have a more dramatic presentation, which may include left ventricular failure, pulmonary edema, lethal arrhythmias such as complete heart block and ventricular fibrillation, and cardiogenic shock.3
Electrocardiographic changes seen in Takotsubo cardiomyopathy mimic those of acute coronary syndrome. ST-segment elevation or depression, particularly in the precordial leads, is seen. T waves may be inverted, sometimes deeply. In many patients, the QT interval may be prolonged beyond 500 ms.4 Giant inverted T waves with QT prolongation may persist for 2 or more months in some patients.5 Cardiac biomarkers, including troponin and CPK, are elevated but do not reach the high levels typically seen in acute myocardial infarction.1
At cardiac catheterization, minimal or no coronary artery disease is seen. Left ventriculography demonstrates an akinetic or dyskinetic ventricle with systolic ballooning of the apex, while the base of the heart is preserved and often hypercontractile. The ejection fraction may be decreased and the left ventricular end diastolic pressure (LVEDP) may be elevated.6 Increasing reports are found in the literature of variations in the patterns of the apical ballooning of the left ventricle as well as involvement of the right ventricle.7,8
Some of the most commonly reported triggers include the unexpected death of a spouse or child, being in an accident or witnessing an accident, house fires, losing significant amounts of money, public speaking and intense quarreling. Hypothermia, earthquake and lightening strikes have been implicated. Physical stressors that have been reported to cause Takotsubo cardiomyopathy include acute respiratory distress, sepsis, non-cardiac surgery and cerebrovascular accident.9 Reported triggers can be seen in Table 1. Takotsubo cardiomyopathy, or transient left ventricular apical ballooning, has also been identified in the critically ill medical patient in the intensive care unit.10 Patients who have developed this sudden onset of apical ballooning, identified by echocardiography, were admitted to the unit because of hypoxia, respiratory failure, sepsis, hemoptysis and coma.

Criteria for Diagnosis
There are no widely accepted criteria for the diagnosis of Takotsubo cardiomyopathy at this time. Bybee et al6 from the Mayo Clinic have proposed criteria for the clinical diagnosis of this disorder (Table 2). Researchers from Brown University in Providence, RI, have developed a registry of patients in the United States with Takotsubo cardiomyopathy with the hopes of learning more about this interesting disorder.

Pathophysiology

The pathophysiology of Takotsubo cardiomyopathy is not clearly understood at present. Several theories of the pathophysiology have been postulated, including multi-vessel coronary vasospasm and myocarditis.4 Stress is thought to be a significant risk factor for its development and currently, the leading theory is that catecholamines trigger the left ventricular abnormalities. High levels of circulating catecholamines have been measured in patients with Takotsubo cardiomyopathy.11

Treatment
At this time, there are no published evidence-based guidelines for the treatment of Takotsubo cardiomyopathy.4 Most patients are managed empirically for acute coronary syndrome. The treatment is supportive and may include aspirin, beta blockers, ACE inhibitors, diuretics, inotropes, vasopressors, anti-coagulants and intra-aortic balloon counterpulsation.12

Complications
Reported complications include left heart failure, cardiogenic shock, acute mitral regurgitation, arrhythmias, left ventricular thrombus and left ventricular free wall rupture.6 The prognosis for Takotsubo cardiomyopathy is usually favorable, with an inpatient mortality rate of 1–2%. Though rare, recurrence has been reported in 5–10% of patients with Takotsubo cardiomyopathy.4,13,14

The author can be contacted at bmcculloch@jps.net

 

References

References

1. Wittstein IS. The broken heart syndrome. Cleveland Clinic Journal of Medicine 2007;74: s17–s22.
2. Abe Y, Kondo M, Matsuoka R, et al. Assessment of clinical features in transient left ventricular apical ballooning. JACC 2003;41(5): 737–742.
3. Seth PS, Aurigemma GP, Drasnow JM, et al. A Syndrome of Transient Left Ventricular Apical Wall Motion Abnormality in the Absence of Coronary Disease: A Perspective from the United States. Cardiology 2003;100: 61–66.
4. Prasad A. Apical ballooning syndrome: An important differential diagnosis of acute myocardial infarction. Circulation 2007;115: e56–e59.
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6. Bybee KA, Kara T, Prasad A, et al. Systematic review: Transient left ventricular apical ballooning: A syndrome that mimics ST-segment elevation myocardial infarction. Ann Intern Med 2004; 141:858–865.
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8. Reuss CS, Lester SJ, Hurst RT, et al. Isolated left ventricular basal ballooning phenotype of transient cardiomyopathy in young women. Am J Cardiol 2007;99:1451–1453.
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12. Sharkey SW, Lesser JR, Zenovich AG, et al. Acute and reversible cardiomyopathy provoked by stress in women from the United States. Circulation 2005;111:472–479.
13. Parodi G, Del Pace S, Carrabba N, et al. Incidence, clinical findings, and outcomes of women with left ventricular apical ballooning syndrome. Am J Cardiol 2007;99: 182–185.
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