Midventricular Takotsubo syndrome.

A 78-year-old woman with a background of type 2 diabetes mellitus, hyperlipidaemia and hypertension experienced sudden onset severe chest pain while in the emergency department, after a stressful emotional event. The pain lasted for 20–30 min and the patient developed widespread T-wave inversion on 12-lead ECG. Cardiac troponin was elevated, and a bedside transthoracic echocardiogram showed moderate left ventricular systolic dysfunction (LVSD) with no significant valvular abnormalities. Hence, she was treated as an acute coronary syndrome (ACS) and her symptoms subsequently improved.

A coronary angiogram performed via the left transradial approach, demonstrated unobstructed coronary arteries with no evidence of significant atherosclerotic disease (Figure 1A & B). The diagnosis of Takotsubo syndrome (TTS) was then formulated. Therefore, we proceeded to left ventriculography, which showed moderate LVSD with akinesia of mid-level LV myocardial walls and preserved systolic function of basal and apical segments (Figure 1C & D, Supplemental Video S1), compatible with mid-ventricular TTS. The LV end diastolic pressure was measured at 12 mmHg. She was discharged on a β-blocker and an ACE inhibitor, following an uncomplicated hospitalisation. A cardiac magnetic resonance (CMR) two months later confirmed complete LV systolic function recovery.

Figure 1.

Invasive coronary angiography.

(A): RAO caudal view showing unobstructed left coronary arteries; (B): LAO view showing unobstructed right coronary artery; (C): left ventriculography (RAO view) in diastole; and (D): left ventriculography (RAO view) in systole demonstrating akinesia of mid-level (red arrows) and preserved systolic function of basal and apical segments (white arrows). LAO: left anterior oblique; RAO: right anterior oblique.

Takotsubo is an acute reversible heart failure syndrome which was first described in 1990 in Japan. It is estimated that 1% to 2% of patients with suspected ACS are eventually diagnosed with TTS. Diagnostic criteria include the presence of transient LV regional wall motion abnormalities, which usually extend beyond a single epicardial vascular distribution, in the absence of culprit atherosclerotic coronary artery disease.[1] A recently published international expert consensus document added the following criteria: Neurologic disorders (e.g. subarachnoid haemorrhage) or pheochromocytoma may serve as triggers for TTS. Myocarditis should be excluded with a CMR for confirmation of the diagnosis.[2]

There are four major anatomical variants of TTS: apical ballooning (75%–80% of cases), midventricular (10%–20%), basal or inverted (5%) and focal (< 1%). Other very rare variants (e.g., biventricular, isolated right or global) have also been described.[2]

The pathophysiological mechanism of the syndrome is unclear. Nevertheless, it appears that sympathetic stimulation and catecholamine surge play a central role.[1]–[3] An emotional, physical, or combined trigger can precede, predominantly affecting post-menopausal women. Physical triggers, which are more common in male patients can be other medical, surgical or psychiatric conditions.[1],[2] Iatrogenic TTS (e.g., following stress echocardiography or an ablation procedure) has been described as well.[4],[5]

Investigations are notable for various electrocardiographic abnormalities encompassing ST-segment elevation, ST depression, LBBB, T-wave inversion, and/or QTc prolongation. Cardiac biomarkers can be raised with elevation of both serum natriuretic peptides and troponin during the acute phase.[1]–[3]

With regards to prognosis, TTS initially believed to represent a benign condition. However, growing evidence suggests that more than 50% of the patients can have complications such as ventricular arrhythmias, acute heart failure, LV outflow tract obstruction, significant mitral regurgitation, thrombus formation in the akinetic apex and cardiogenic shock.[1],[3] The LV systolic function can fully recover completely within three-to-six months.[1]

Our patient represents a case of mid-ventricular TTS who had uncomplicated recovery. Left ventriculography played a key role to final diagnosis demonstrating the characteristic mid-ventricular akinetic pattern which resembles to the ancient Greek vessel known as ‘amphora’.