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Recurrent takotsubo cardiomyopathy in the setting of transient neurological symptoms: a case report
© Sardar et al; licensee BioMed Central Ltd. 2011
Received: 24 January 2011
Accepted: 24 August 2011
Published: 24 August 2011
First described in Japan, takotsubo cardiomyopathy is increasingly becoming recognized worldwide as a cause of sudden and reversible diminished left ventricular function characterized by left apical ballooning and hyperkinesis of the basal segments, often with symptoms mimicking a myocardial infarction. Associated with physical or emotional stress, its exact pathogenesis has not been established, though evidence supports a neurohumoral etiology. Additionally, recurrence of this condition is rare. In this report, we present a rare case of recurrent takotsubo cardiomyopathy in a post-menopausal woman who presented with transient neurological complaints on both occasions.
We present a rare case of a 76-year-old Caucasian woman with no history of congestive heart failure who presented to our emergency department twice with transient neurological complaints. On the first occasion, she was found to have transient aphasia which resolved within 24 hours, yet during that period she also developed symptoms of congestive heart failure and was noted to have a new, significantly depressed ejection fraction with apical akinesis and possible apical thrombus. One month after her presentation a repeat echocardiogram revealed complete resolution of all wall motion abnormalities and a return to baseline status. Seven months later she presented with ataxia, was diagnosed with vertebrobasilar insufficiency, and again developed symptoms and echocardiography findings similar to those of her first presentation. Once again, at her one-month follow-up examination, all wall motion abnormalities had completely resolved and her ejection fraction had returned to normal.
Though the exact etiology of takotsubo cardiomyopathy is unclear, a neurohumoral mechanism has been proposed. Recurrence of this disorder is rare, though it has been reported in patients with structural brain abnormalities. This report is the first to describe recurrent takotsubo cardiomyopathy in a patient with transient neurological symptoms. In our patient, as expected in patients with this condition, complete resolution of all left ventricular abnormalities occurred within a short period of time. It is important for clinicians to be aware of this increasingly recognized syndrome, including its association with recurrence, especially in the clinical setting of neurologic dysfunction.
Left apical ballooning syndrome, also known as takotsubo cardiomyopathy (TTC), is a clinical syndrome of transient diminished left ventricular (LV) apical wall motion with relative preservation of the basal heart segment in the presence of normal coronary arteries. It was first described in Japan in the early 1990s  and is named for ventricles which seem similar in appearance to a Japanese octopus trap on ventriculography scans. This syndrome has been linked to emotional or physical stress, with a high incidence among post-menopausal women . However, the etiology of TTC is not completely understood. Several possible mechanisms, including microvascular dysfunction, coronary artery vasospasm, aborted myocardial infarction, and excess catecholamine stimulation, have been proposed [2, 3]. Typically, LV function returns to normal within six to eight weeks. Recurrence, which is increasingly being reported in the literature [2, 4, 5], can be related to neurological pathology [6, 7]. We present a case of a woman with recurrent TTC whose presenting symptoms on both occasions were of neurological origin.
A 76-year-old Caucasian woman presented to our tertiary care hospital on two separate occasions. She had a medical history significant for hypertension, diabetes mellitus, dyslipidemia, hypothyroidism, no congestive heart failure (CHF), no evidence of coronary artery disease visualized by catheterization two years prior to her initial presentation, and a normal baseline left ventricular ejection fraction (LVEF) of 55% to 60%. In each episode, she presented without evidence of existing physical or emotional distress.
At her first presentation, she arrived at the emergency department with the onset of aphasia in the setting of hypertensive emergency (blood pressure (BP) 226/100 mmHg). Her aphasia was isolated and transient, and her physical examination was otherwise unremarkable. Because we were concerned about an acute intracerebral event in the setting of hypertensive emergency, computed tomography and diffusion-weighted MRI/magnetic resonance angiography (MRI/MRA) of the brain were performed, both of which showed no evidence of acute ischemia, hemorrhage, or cerebral edema. She was admitted for work-up of acute stroke and transient ischemic attack and treatment of her hypertensive emergency. Her aphasia resolved within 24 hours after onset, and a modest reduction in BP was achieved. Shortly after admission, however, she developed acute onset of dyspnea, and rales were heard bilaterally during her physical examination.
Given the clinical, laboratory, and echocardiography data, the patient was started on anti-platelet and anti-coagulation therapy and optimized on heart failure treatment for presumed CHF secondary to ischemic cardiomyopathy. Her symptoms improved, and she was discharged from the hospital with follow-up planned in the out-patient clinic. At one month post-presentation, an echocardiogram revealed complete improvement of LV function back to baseline level (LVEF of 55%) and no evidence of wall motion abnormalities or thrombus (Figure 1B).
Our patient presented twice with various neurological complaints, and on each occasion she was found to have evidence of acute onset severe apical LV dysfunction that completely resolved within one month of onset. After her first presentation, we suspected that this patient had developed TTC. This condition is a syndrome of transient diminished LV apical wall motion with relative preservation of the basal heart segment in the presence of normal coronary arteries . First described in Japan , TTC has since been identified worldwide . Named for the ventricle's similar appearance on ventriculograms to a takotsubo, or Japanese octopus trap, this syndrome, also known as left apical ballooning syndrome or "broken heart syndrome," has been linked to emotional or physical stress, with a high incidence among post-menopausal women. As the syndrome is transient, LV function typically normalizes within six to eight weeks of onset .
Our patient is unique in that she had a recurrence of this syndrome within seven months of her initial presentation, and both episodes were preceded by transient neurological complaints. Recurrence of TTC, once thought to be rare, has been increasingly recognized in the literature, with a recurrence rate of 5% to 10% worldwide [2, 4, 5]. Additionally, the development of TTC in the setting of fleeting neurological symptoms such as aphasia and ataxia without structural brain disease has never been reported. Histological and nuclear imaging data in humans have shown regional differences in efferent sympathetic innervation where the basal ventricular wall possesses a greater mean density of nerve endings and local catecholamine concentrations compared to the apex, while the apex possesses higher concentrations of adrenoreceptors. This differential distribution is proposed to induce the LV dysfunction found in patients with TTC [2, 9, 10].
Similarly, neurogenic myocardial stunning has also been proposed as a cause of the findings in patients with TTC [2, 11]. LV dysfunction with a takotsubo-like state is often seen with intracranial pathology, including subarachnoid hemorrhage and congenital brain abnormalities in children. In the setting of intracranial injury or dysfunction, such as the catecholamine toxicity described in our patient, a surge in sympathetic release will cause a relative hyperdynamic contraction of the LV basal segments with a relative stunning, or ballooning, of the LV apical portion due to saturation of the adrenoreceptors in that distribution .
Therefore, there appears to be a correlation between intracranial and LV dysfunction. In fact, recurrence of TTC in association with underlying chronic neurological pathology, mainly status epilepticus, has previously been reported [6, 7]. Our present report, however, is the first to describe a patient with recurrent TTC in the setting of transient neurological symptoms without structural evidence of neurologic dysfunction.
Although no clearly defined etiology for TTC exists, clinicians should be aware of the possibility of TTC in patients whose presentation mimics acute myocardial infarction, especially in the setting of emotional, physical, and specifically neurological stress. Additionally, it has been reported that patients with one episode of TTC are at increased risk for recurrence . As our patient's two presentations suggest, the development and recurrence of TTC likely involve a neurocardiogenic mechanism. Though this condition rarely leads to death, it is imperative that the clinician be aware of this syndrome to ensure the prompt initiation of appropriate supportive care so that a return of normal LV function can be achieved 
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
We thank Dr Adnan Siddiqui, Dr Brian Triola, and Barbara Schiavi for obtaining the echocardiographic images and Dr Jerome Santoro and Dr Cynthia Smith of the Department of Medicine, Lankenau Hospital, Wynnewood, PA, USA.
- Dote K, Sato H, Tateishi H, Uchida T, Ishihara M: [Myocardial stunning due to simultaneous multivessel coronary spasms: a review of 5 cases] [in Japanese]. J Cardiol. 1991, 21: 203-214.PubMedGoogle Scholar
- Akashi YJ, Goldstein DS, Barbaro G, Ueyama T: Takotsubo cardiomyopathy: a new form of acute, reversible heart failure. Circulation. 2008, 118: 2754-2762. 10.1161/CIRCULATIONAHA.108.767012.View ArticlePubMedPubMed CentralGoogle Scholar
- Lindsay J, Paixao A, Chao T, Pichard AD: Pathogenesis of the takotsubo syndrome: a unifying hypothesis. Am J Cardiol. 2010, 106: 1360-1363. 10.1016/j.amjcard.2010.06.068.View ArticlePubMedGoogle Scholar
- Yamamoto Y, Watari Y, Kobayashi K, Tanaka K: Recurrent takotsubo cardiomyopathy can appear as transient midventricular ballooning syndrome. J Cardiol Cases. 2009, 1: e141-e143.View ArticleGoogle Scholar
- Sharkey SW, Windenburg DC, Lesser JR, Maron MS, Hauser RG, Lesser JN, Haas TS, Hodges JS, Maron BJ: Natural history and expansive clinical profile of stress (tako-tsubo) cardiomyopathy. J Am Coll Cardiol. 2010, 55: 333-341. 10.1016/j.jacc.2009.08.057.View ArticlePubMedGoogle Scholar
- Legriel S, Bruneel F, Dalle L, Appere-de-Vecchi C, Georges JL, Abbosh N, Henry-Lagarrigue M, Revault D'Allonnes L, Ben Mokhtar H, Audibert J, Guezennec P, Troche G, Bedos JP: Recurrent takotsubo cardiomyopathy triggered by convulsive status epilepticus. Neurocrit Care. 2008, 9: 118-121. 10.1007/s12028-008-9107-6.View ArticlePubMedGoogle Scholar
- Lemke DM, Hussain SI, Wolfe TJ, Torbey MA, Lynch JR, Carlin A, Fitzsimmons BF, Zaidat OO: Takotsubo cardiomyopathy associated with seizures. Neurocrit Care. 2008, 9: 112-117. 10.1007/s12028-008-9075-x.View ArticlePubMedGoogle Scholar
- Sharkey SW, Lesser JR, Zenovich AG, Maron MS, Lindberg J, Longe TF, Maron BJ: Acute and reversible cardiomyopathy provoked by stress in women from the United States. Circulation. 2005, 111: 472-479. 10.1161/01.CIR.0000153801.51470.EB.View ArticlePubMedGoogle Scholar
- Tsuchihashi K, Uesima K, Uchida T, Oh-mura N, Kimura K, Owa M, Yoshiyama M, Miyazaki S, Haze K, Ogawa H, Honda T, Hase M, Kai R, Morii I, Angina Pectoris-Myocardial Infarction Investigations in Japan: Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction: Angina Pectoris-Myocardial Infarction Investigations in Japan. J Am Coll Cardiol. 2001, 38: 11-18. 10.1016/S0735-1097(01)01316-X.View ArticlePubMedGoogle Scholar
- Kawano H, Okada R, Yano K: Histological study on the distribution of autonomic nerves in the human heart. Heart Vessels. 2003, 18: 32-39. 10.1007/s003800300005.View ArticlePubMedGoogle Scholar
- Wittstein IS, Thiemann DR, Lima JA, Baughman KL, Schulman SP, Gerstenblith G, Wu KC, Rade JJ, Bivalacqua TJ, Champion HC: Neurohumoral features of myocardial stunning due to sudden emotional stress. N Engl J Med. 2005, 352: 539-548. 10.1056/NEJMoa043046.View ArticlePubMedGoogle Scholar
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