|
 |
| CASE REPORT |
|
| Year : 2013 | Volume
: 14
| Issue : 4 | Page : 185-189 |
|
|
Recurrent transient apical cardiomyopathy (Tako-Tsubo-like left ventricular dysfunction) in a postmenopausal female with diffuse esophageal spasms
Jorge A Brenes Salazar
Department of Medicine, Division of Cardiovascular Diseases, Mayo Clinic, MN, USA
| Date of Web Publication | 12-Feb-2014 |
Correspondence Address:
Jorge A Brenes Salazar
Mayo Clinic, 200 First St. SW, Rochester, MN 55905
USA
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/1995-705X.126888
 Abstract | | |
Transient apical cardiomyopathy, also known as Takot-tsubo-like left ventricular dysfunction, is a clinical syndrome characterized by reversible left ventricular dysfunction at the apex with preserved basal contractility, in the setting of new ST and T wave changes suggestive of ischemia but no evidence of obstructive coronary artery disease on angiography. The main mechanism appears to be intense neuroadrenergic myocardial stimulation with endothelial dysfunction of the coronary vasculature. It has been noted that patients with esophageal spasms also have a tendency for coronary spasms. We present the case of a postmenopausal female with documented severe esophageal spasms who presented with atypical angina and recurrent Tako-tsubo cardiomyopathy. Keywords: Esophageal spasms, Tako-tsubo, transient apical cardiomyopathy
How to cite this article:
Brenes Salazar JA. Recurrent transient apical cardiomyopathy (Tako-Tsubo-like left ventricular dysfunction) in a postmenopausal female with diffuse esophageal spasms. Heart Views 2013;14:185-9 |
How to cite this URL:
Brenes Salazar JA. Recurrent transient apical cardiomyopathy (Tako-Tsubo-like left ventricular dysfunction) in a postmenopausal female with diffuse esophageal spasms. Heart Views [serial online] 2013 [cited 2023 Nov 29];14:185-9. Available from: https://www.heartviews.org/text.asp?2013/14/4/185/126888 |
| Introduction | |  |
Since its first description in 1990, transient apical cardiomyopathy (also known as Takot-subo-like left ventricular dysfunction) has been recognized more frequently with the widespread use of echocardiography. It represents a unique model of stress-related, acute reversible ventricular dysfunction that mimics an acute coronary syndrome in the very early stages. Despite multiple mechanisms suggested to explain its pathophysiology, there is increasing evidence that supports direct neuroadrenergic myocardial stimulation with concomitant transient, reversible, coronary vasospasm as the inciting event, either at the epicardial or arteriolar level.
Recurrence is rare, and estimated to be less than 3.5% of all documented cases. [1] These patients tend to have a particular predisposition for smooth muscle spasm. Intense, reciprocal input between the esophagus and coronary arteries has been well documented in both animal and human models. [2] Hence, we present the challenging case of a postmenopausal female with severe esophageal spasms, who presented with recurrent transient apical cardiomyopathy (TAC) in the setting of emotional distress and probable coronary vasospasm. The pathophysiologic, diagnostic and therapeutic aspects of TAC are briefly discussed.
| Case Report | | |
A 66-year-old woman with a history of AV nodal ablation for atrial fibrillation and severe, diffuse esophageal spasms which required multiple dilations and surgical myectomy had been hospitalized multiple times at a nearby institution with shortness of breath and chest pain. Most of her symptoms had been attributed to esophageal spasms confirmed by manometry. During her first admission, an echocardiogram demonstrated an ejection fraction of 25-30% with apical anterior, inferior and lateral wall akinesis, compatible with Tako-tsubo-like cardiomyopathy. She underwent a coronary angiogram 48 h later with no evidence of obstructive disease and normalization of left ventricular function. Coronary vasospasm was suspected but no provocative testing was performed.
She presented six months later to our hospital complaining of severe chest pressure that started abruptly after she became furious at her driver for being late. Her ECG on admission [Figure 1]a displayed a ventricular paced rhythm, with hyperacute T waves and new ST segment elevation in anterior precordial leads; she was given aspirin and started on heparin infusion. Once intravenous infusion of nitroglycerin was initiated, she had resolution of her index symptoms, as well as most of her ECG changes [Figure 1]b. She had a minor elevation in her troponin levels. Echocardiogram performed the next morning [Figure 2] revealed an ejection fraction of 15% with apical ballooning, consistent with Tako-tsubo-like cardiomyopathy. She received supportive management and was started on long acting nitrates and calcium channel blockers with no recurrence of symptoms. On admission day # 5, her left ventricular function was reassessed by echocardiogram and noted to be within normal limits.  | Figure 1: (a) 12-lead ECG on presentation while the patient was experiencing angina , reveals a ventricular-paced rhythm with hyperacute T-waves in anterior precordial leads (arrows), along with ST segment elevation of more than 1 mm in those leads. (b) 12- lead ECG after initiation of nitroglycerin infusion; the patient had resolution of angina symptoms. Notice the reversal of acute changes in precordial leads as compared to Fig 1a
Click here to view |
 | Figure 2: Transthoracic echocardiogram with contrast that shows apical akinesis with a hyperkinetic base, consistent with transient apical (Tako-tsubo-like) cardiomyopathy. Ejection fraction was estimated at 15%
Click here to view |
| Discussion | | |
In clinical practice, the diagnosis of transient apical cardiomyopathy with chest pain is usually achieved after exclusion of a typical obstructive acute coronary syndrome, since both entities share common elements, particularly during the early stages of presentation. Defining features of TAC include the sudden onset of new ST and T wave abnormalities (usually ST elevation, T wave inversion or both) in the setting of a stressor, mild elevation in cardiac enzymes, transient, disproportionate left ventricular dysfunction that does not follow a typical coronary distribution, and finally, absence of angiographic obstructive coronary disease that can explain the ventricular dysfunction. Data from the CASPAR trial [3] showed that as many as 25 % of patients who present with chest pain at rest suspicious for a coronary event have no evident culprit lesion; furthermore, in this select group of patients, intracoronary challenge with acetylcholine (Ach) is able to induce spasm in almost half of them. This technique is considered the gold standard for the diagnosis of coronary vasospasm. It has been noted that individuals with a persistently positive Ach response have higher tendency for recurrent left ventricular dysfunction, [4] and therefore use of Ach becomes both a diagnostic and prognostic tool.
Unfortunately, our patient was not challenged during her first presentation when an angiogram was performed, which might have yielded a definitive, specific diagnosis early on. The combination of angiography with provocative testing, along with perfusion echocardiography has been proposed as a comprehensive strategy to characterize this subset of patients with non-obstructed coronaries [Figure 3]. The lack of consistent results in the literature regarding Ach testing after TAC could be due to variable timing of testing or dosing; [5] furthermore, the difference in angiographic response might be related to the location of susceptible coronary arteries (epicardial arteries versus arterioles). | Figure 3: Suggested algorithm for the evaluation of patients undergoing angiography for suspected acute coronary syndrome. The combination of provocative testing with simultaneous echocardiography could detect a subset of patients with vasospastic features and "normal" coronaries
Click here to view |
The pathophysiology of TAC is likely multifactorial, and there is growing evidence [1],[4],[5] to suggest that complex neuro-hormonal stimulation and endothelial dysfunction is responsible; the initial mechanism appears to be neuroadrenergic stimulation of the myocytes under physical or emotional stress, resulting in myocardial stunning. Vascular sympathetic stimulation at the epicardial and/or arteriolar levels can lead to severe, diffuse, and transient coronary vasospasm, which in turn aggravates left ventricular dysfunction and associated metabolic abnormalities [Figure 4].  | Figure 4: Diagram that summarizes the pathophysiology of left ventricular dysfunction seen in transient apical cardiomyopathy as a consequence of neuro-hormonal mediated myocardial stimulation
Click here to view |
Galiuto and colleagues [1] conducted an insightful protocol to assess the role of vasospasm in TAC. They assessed a group of patients with evidence of TAC by commonly accepted criteria (n = 15), including absence of epicardial obstructive disease, with a similar control group of patients (n = 15) with an anterior STEMI. Both groups were studied using myocardial contrast echocardiography within 3 ± 2 days of the onset of symptoms, at the time that an intravenous infusion of adenosine (140 ug/kg/min) was administered. All myocardial functional variables (wall motion score index, contrast score index, myocardial perfusion defect length) significantly improved in the TAC group, promptly returning to resting conditions after discontinuation of adenosine infusion. There was no change in the same variables in the STEMI group. At one month follow-up, myocardial perfusion significantly improved when compared to baseline in TAC subjects, but not in patients who sustained an anterior STEMI. Furthermore, the investigators were able to find a match between the extent of perfusion defects by echocardiography and the size of regional dysfunctional areas, both improved with vasodilation of probably non-obstructed or minimally-obstructive coronary arteries, responsive to adenosine. It is important to emphasize that during adenosine infusion, myocardial functional parameters did not completely normalize, suggesting that restoration of blood flow can partially improve contractile reserve of a metabolically stunned but viable myocardium.
Fatty acid metabolism also appears significantly impaired in TAC, particularly during the early phase. [6] TIMI frame count, an index of coronary flow velocity, has been noted to be significantly higher (representing slower blood flow) in all coronary arteries in patients with TAC compared with controls, even after resolution of ventricular dysfunction. [7] These clinical experiments strongly support the concept that irrespective of the original insult, early reversible vascular dysfunction is a common element in patients with TAC.
A notorious feedback loop has been documented between the coronary arteries and the esophageal system. Cardio-esophageal neural reflex arcs have been described in humans. [8] Coronary vasospasm can be elicited by stimulation of alpha-adrenergic, histaminic, serotoninergic and muscarinic receptors; similarly alpha-adrenergic, histaminic and muscarinic receptors mediate esophageal contraction. [9] Patients with variant angina have been compared to symptomatic patients with obstructive CAD and nocturnal angina, with the concurrent use of esophageal manometry and continuous ECG monitoring. [2]
In individuals with variant angina, episodes of esophageal spasms are clearly time related to periods of ECG-recorded ischemia; bidirectional analysis of causal effects demonstrates that the influences are mutual and reciprocal. Abnormal sympathetic stimulation could be a common trigger, which would be consistent with the neuroadrenergic hypothesis of myocardial stunning in TAC. In clinical practice, this poses significant diagnostic challenges, as demonstrated in our case, since these patients with "linked angina" can present with atypical chest pain, at times attributable to the esophageal system, and others to transient coronary ischemia. Thus, in a patient with documented esophageal spasms, a "normal" coronary angiogram cannot be held to exclude transient myocardial ischemia. Conversely, patients with coronary spasms could have underlying esophageal motility disorders. It would be interesting to speculate if such reciprocal stimulation pathways exist between the coronaries and the bronchial tree in humans, since COPD and asthma have been reported in a high percentage of patients with TAC. [10]
There are no specific guidelines for the management of TAC presenting with chest pain; initial approach usually overlaps with that of an obstructive acute coronary syndrome, due to the inability to distinguish these entities on clinical or electrocardiographic grounds alone; urgent angiogram is key to make such distinction in the acute setting. Given current insight into the pathophysiology of TAC, it appears reasonable that drugs that can alleviate vasospasm or blunt adrenergic response such as alpha-beta blockers, nitrates and calcium-channel blockers could prove beneficial in the process of supporting patients with this condition; prospective comparative trials are needed to address this hypothesis. It is important to emphasize that despite its reversible nature, TAC is not always a benign condition, particularly in the acute phase, when pulmonary edema, ventricular dysrhythmias and cardiogenic shock can significantly compromise patients. Fatality rates can range from 0 to 4%. [7]
| Conclusions | | |
Transient apical cardiomyopathy that presents with chest pain continues to be a challenging entity, given its similarities to an acute atherosclerotic coronary syndrome in its early phase. Regardless of the initial insult, intense, neuroadrenergic myocardial stimulation with vasogenic and metabolic changes appears as the unifying mechanism that explains transient ventricular dysfunction. Due to the reciprocal feedback between coronary and esophageal spasms, some patients might be predisposed to a form of "linked angina", manifesting as a smooth muscle hyper-contractility syndrome. Prospective trials are needed to determine the optimal pharmacologic management for these patients.
| References | | |
| 1. | Galiuto L, Ranieri De Caterina A, Porfidia A, Paraggio L, Barchetta S, Locorotondo G, et al. Reversible coronary microvascular dysfunction: a common pathogenetic mechanism in Apical Ballooning or Tako-Tsubo Syndrome. Eur Heart J 2010;31:1319-27. 
|
| 2. | Manfrini O, Bazzocchi G, Luati A, Borghi A, Monari P, Bugiardi R. Coronary spasm reflects inputs from adjacent esophageal system. Am J Physiol Heart Circ Physiol 2006;290:H2085-91.
|
| 3. | Ong P, Athanasiadis A, Hill S, Vogelsberg H, Voehringer M and Sechtem U. Coronary artery spasm as a frequent cause of acute coronary syndrome. J Am Coll Cardiol 2008;52:523-7.
|
| 4. | Angelini, P. Takotsubo cardiomyopathy: what's behind the octopus trap? Tex Heart Inst J 2010;37:85-7.
|
| 5. | Angelini P. Transient left ventricular apical ballooning: a unifying pathophysiologic theory at the edge of Prinzmetal angina. Cath Cardiovasc Interv 2008;71:342-52.
|
| 6. | Kurisu S, Inoue I, Kagawoe T, Ishihara M, Shimatani Y, Nishioka K, et al. Myocardial perfusion and fatty acid metabolism in patients with tako-tsubo-like left ventricular dysfunction. J Am Coll Cardiol 2003;41:743-8.
|
| 7. | Buchholz S, Redan G. Tako-tsubo syndrome on the rise: a review of the current literature. Postgrad Med J 2006;83:261-4.
|
| 8. | Makk LJ, Leesar M, Joseph A, Prince CP, Wright RA. Cardioesophageal reflexes: an invasive human study. Dig Dis Sci 2000;45:2451-4.
[PUBMED] |
| 9. | Rasmussen K, Ravnsbaek J, Funch-Jensen P, Bagger JP. Esophageal spams in patients with coronary artery spasm. Lancet 1986;1:174-6.
[PUBMED] |
| 10. | Hertting K, Krause K, Harle T, Boczor S, Reimers J, Kuck KH. Transient left ventricular apical ballooning in a community hospital in Germany. Int J Cardiol 2006;112:282-8.
|
[Figure 1], [Figure 2], [Figure 3], [Figure 4]
|
