Takotsubo cardiomyopathy with late gadolinium enhancement

A 68-year-old woman with previous history of hypercholesterolemia and anxiety came to our ER with sudden chest pain radiating to the jaw, as well as palpitations. ECG showed inferior ST elevation and cardiac enzymes were slightly elevated. Coronary angiography results came back as normal.

Baseline echocardiography showed akinesia of middle and apical myocardial segments, as well as severe heart dysfunction (LVEF 30-35%).

Cardiac MR images were obtained a week after the acute episode: 

CINE sequences show slightly decreased contractility in mid-cavity and apical segments, with relative basal hypercontractility, giving the heart a ‘balloon’ appearance (Fig 1). Left ventricular ejection fraction was 70%, meaning it had already recovered by week 1.

On T2 weighted STIR images, a hyperintense apical region can be seen, as well as high T1 and T2 map values (1424 and 54 ms respectively) in the involved regions – all three findings in keeping with mid-cavity and apical myocardial oedema (Fig 2).

Perfusion sequence did not have any alterations.

Late gadolinium enhancement (LGE) sequences showed faint transmural LGE affecting the aforementioned mid-cavity and apical areas beyond vascular territories (Fig 3). The subendocardium is relatively spared.

Takotsubo cardiomyopathy (TC, also known as stress cardiomyopathy) is a reversible cardiomyopathy noted for its clinical resemblance to an acute coronary syndrome (ACS) and for its distinctive triggering circumstances (famously, emotional or physical stress are linked to 65-80% of cases) [1]. It typically presents in females aged 50-70. Incidence has been rising incessantly, and TC is currently believed to account for around 2% of patients initially classified as ACS [2]. 

Pathophysiologycally, it is believed that a rise in catecholamine levels results in a “stunned myocardium” and altered contractility. On biopsy, inflammatory cells, fibrosis and necrosed myocytes may be seen.

Clinically TC is almost akin to an ACS. Dyspnea and chest pain are the most common symptoms, with precordial ST elevation happening in 50% of cases along with a slight elevation of cardiac enzymes [3]. Patients are often taken into the catheterization lab, only to find out the absence of any significant coronary artery lesions.

Echocardiography may show systolic “ballooning” the LV apex, due to the lack of contraction of mid-cavity and apical regions and the hypercontractility of the basal region, as well as a LVEF decrease. 

Often, MRI will be required in order to differentiate Takotsubo from other causes of acute LV dysfunction such as myocarditis and ischemia. MRI features:

• CINE sequences: apical ballooning and LVEF reduction. 
• T2WI STIR, T1 and T2 maps: T2 hyperintensity and high map values are seen in the affected regions, in relation with myocardial oedema.
• Perfusion is usually normal. 
• Late gadolinium enhancement has classically said to be absent, but recent studies have shown LGE in up to 40% of cases. LGE in TC is faint, transmural (unlike myocarditis, which is epicardial or mid-wall) and mostly in mid-cavity and apical segments (unlike ischemia, which follows a vascular as well as subendocardial distribution) [4,5].

Imaging changes and LVEF impairment usually resolve within 4 weeks, which marks an overall good prognosis. Complications are rare, most commonly heart failure in the acute setting, which has recently been linked with the presence of LGE. [5] Treatment usually involves supportive care and ordinary HF protocols until the LV recovers its normal function.

Take-Home Messages:

- TC is a reversible cardiomyopathy believed to account for 2% of clinically suspected ACS.

- Its clinical resemblance to myocardial infarction makes it an important differential diagnosis, in which Cardiac MR is taking a central role.  Contrary to early information, recent studies show LGE can occasionally be seen in TC too. Nevertheless, LGE in TC is subtle, diffuse and transmural (unlike MI, which shows marked vascular and subendocardial distribution).

Written informed patient consent for publication has been obtained.