Atypical presentation of "takotsubo cardiomyopathy" without ST segment elevation: a case report
© Liu et al; licensee BioMed Central Ltd. 2008
Received: 21 May 2008
Accepted: 14 November 2008
Published: 14 November 2008
"Takotsubo cardiomyopathy" is characterized by transient LV dysfunction and mimicking acute myocardial infarction.
We reported a case with atypical presentation of "takotsubo cardiomyopathy" without ST segment elevation, but with severe transient left ventricular dysfunction.
Diagnosis of "takotsubo cardiomyopathy" should be based on typical left ventricular dysfunction without coronary artery disease.
"Takotsubo cardiomyopathy", firstly described by Satoh et al. and Dote et al. in 1990, is named due to reversible abnormal wall motion of the left ventricle (LV) with a systolic shape on left ventriculography similar to the shape of a Japanese fishing pot for octopus, a unique shape with a round bottom and narrow neck. The wall motion abnormalities in this disease exhibit both apical akinesis and basal hyperkinesis in the acute phase. Yet the abnormality is not related to the coronary blood distribution. The LV dysfunction will be normalized within a few weeks, and without any sequalae. This is quite different from the wall motion abnormalities seen in acute myocardial infarction or acute myocarditis. Besides, diffuse T wave inversion, a prolonged QT interval, and greater ST-segment elevation in leads V4–6 without reciprocal changes occurred in most patients.[3, 4]
Notably, the onset is usually preceded by emotional stress or prior aggravation of an underlying disorder (for example, cerebrovascular accident, epilepsy, or acute abdomen etc.). It suggests that emotional or physical stress may play an important role in takostubo cardiomyopathy. In the case we reported, the patient exhibited a typical LV abnormality seen in takotsubo cardiomyopathy, but without any preceding emotional or physical stress and typical electrocardiogram (ECG) change.
There are several reports of patients with profound, reversible LV dysfunction, especially after sudden emotional stress. [5–7] This pattern of LV dysfunction has been referred to as "takotsubo cardiomyopathy". It is characterized by transient LV dysfunction with chest pain, electrocardiographic changes, especially ST segment elevation, and minimal release of myocardial enzymes mimicking acute myocardial infarction. Left ventriculograms showed apical ballooning with basal hyperkinesias.[4, 7] Diagnosis of "takotsubo cardiomyopathy" is based on the following characteristics: 1) onset symptoms resembling those of acute myocardial infarction, 2) apical akinesis and basal hyperkinesis, 3) ST segment elevation with T wave inversion and QT prolongation, 4) minimal myocardial enzymes release, 5) no angiographical stenosis in the coronary arteries, and 6) reversible left ventricular function.[1, 8] The following features of the patient we reported are consistent with typical "takotsubo cardiomyopathy": 1) minimal myocardial enzymes release; 2) an absence of organic coronary artery lesions; 3) a typical "round bottom and narrow neck" shape shown in left ventriculogram; and 4) reversible LV dysfunction, which was recovered in a few weeks and demonstrated by the follow-up echocardiography.
There were features different from the typical presentation of takotsubo cardiomyopathy. First, the degree of ST elevation was not eminent (0.05 mv) and not in the standard leads — the precordial leads, especially V4–6. Tsuchihashi et al. reported that ST-segment elevation was observed in 90% of patients with takotsubo cardiomyopathy. The higher ratio of ST-segment elevation in leads V4–6/V1–3 with the absence of reciprocal changes, and the prolonged QTc interval show a high sensitivity and specificity for diagnosing takotsubo cardiomyopathy.Second, we could not recognize any triggering conditions; the patients denied any emotional or physical stress and there was no abnormal physical examination in the first contact of the patient in the emergent department. However, most of the patients (around 75%) have the triggering conditions, including exposure to internal (emotional) and external stress (physical, exacerbated disorders, procedural and perioperative). As a result, although the patient we reported did not have the typical ECG ST-segment change and the precipitating factor, but had transient LV dysfunction with typical round bottom and narrow neck shape shown in left ventriculogram and normal coronary angiogram, yet takotsubo cardiomyopathy with a rare and atypical presentation was diagnosed.
The precise pathophysiology is still not identified. The mechanism has been reported as stunned myocardium, a prolonged postischemic left ventricular dysfunction after brief myocardial ischemia, coronary vasospasm, microvascular disturbance in the myocardium, acute myocarditis and so on. But in our case, we could not clarify the etiology of transient LV dysfunction. Although the pathophysiology is not clear, yet the mortality of takotsubo cardiomyopathy is very low and the recurrence is rare. However, Sharkey et al. once reported that in acute stage, almost 40% of the patients required aggressive treatment, including hemodynamic stabilization with vasopressor agents and intra-aortic balloon counterpulsation. Unfortunately, there is no independent predictor of deteriorated cardiac failure. Hence, prompt and aggressive pharmacological and hemodynamic support in hemodynamic unstable patients play an important role in the reversal of LV function and survival without sequalae.
We reported an atypical case of takotsubo cardiomyopathy without typical ECG ST-segment elevation and triggering events. Our case indicated that diagnosis of "takotsubo cardiomyopathy" should be based on typical left ventricular dysfunction without coronary artery disease. The etiology of "takotsubo cardiomyopathy" is probably not solely due to acute stress. Other pathophysiology should be considered.
Written informed consent was obtained from the publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Dote K, Sato H, Ishihara M: Myocardial stunning due to simultaneous multivessel coronary spasm: a review of 5 cases. J Cardiol. 1991, 21: 203-214.PubMedGoogle Scholar
- Akashi YJ, Nakazawa K, Sakakibara M, et al: The clinical features of takotsubo cardiomyopathy. Q J Med. 2003, 96: 563-573.View ArticleGoogle Scholar
- Wittstein IS, Thimann DR, Baughman KL: Neurohumoral features of myocardial stunning due to sudden emotional stress. N Engl J Med. 2005, 352: 539-548. 10.1056/NEJMoa043046.View ArticlePubMedGoogle Scholar
- Riyo O, Yoshikazu H, Takefumi T, Koji Y: Specific findings of the standard of 12-lead ECG in patients with "takotsubo" cardiomyopathy. Circ J. 2003, 67: 687-690. 10.1253/circj.67.687.View ArticleGoogle Scholar
- Pavin D, Le Breton H, Daubet C: Human stress cardiomyopathy mimicking acute myocardial syndrome. Heart. 1997, 78: 509-511.PubMed CentralView ArticlePubMedGoogle Scholar
- Brandspiegel HZ, Marinchak RA, Kowey PR: A broken heart. Circulation. 1998, 98: 1349.View ArticlePubMedGoogle Scholar
- Tsuchihashi K, Ueshima K, Uchida T: Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction. J Am Coll Cardiol. 2001, 38: 11-18. 10.1016/S0735-1097(01)01316-X.View ArticlePubMedGoogle Scholar
- Moriya M, Mori H, Yano K: Six-month follow-up of takotsubo cardiomyopathy with I-123-meta-iodobenzyl-guanidine myocardial scintigraphy. Inten Med. 2002, 41: 829-833. 10.2169/internalmedicine.41.829.Google Scholar
- Braunwald E, Kloner RA: The stunned myocardium: prolonged postischemic ventricular dysfunction. Circulation. 1982, 66: 1146-1149.View ArticlePubMedGoogle Scholar
- Kurisu S, Inoue I, Kawagoe T: Myocardial perfusion and fatty acid metabolism in patients with takotsubo-like left ventricular dysfunction. J Am Coll Cardiol. 2003, 41: 743-748. 10.1016/S0735-1097(02)02924-8.View ArticlePubMedGoogle Scholar
- Mitsuya N, Akashi Y, Ozawa Y: A case of transient left ventricular dysfunction so called takotsubo-like wall motion abnormality that appeared to be caused by acute myocarditis. Ther Res. 1999, 20: 80-85.Google Scholar
- Sharkey SW, Lesser JR, Zenovich AG, et al: Acute and reversible cardiomyopathy provoked by stress in women from the United Sates. Circulation. 2005, 111: 472-479. 10.1161/01.CIR.0000153801.51470.EB.View ArticlePubMedGoogle Scholar
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