Reverse takotsubo cardiomyopathy

Clinical History

60-year-old male patient recently retired, presented with retrosternal chest pain, progressive dyspnoea and mild elevated troponin.

Imaging Findings

SSFP ECG-gated cardiac MRI revealed middle left ventricle annular circumferential segment of akinesia associated with dyskinetic aneurysmally ballooned base level, notably septal with sparing of the apical segments and demonstrating relatively normal contractility (Fig. 1, 2) Subsequently, the left ventricle EF was impaired. Adenosine stress perfusion scan showed no evidence of myocardial perfusion defects denoting no evidence of myocardial ischaemia (Fig. 3, 4). The T2 myocardial map showed no evidence of myocardial wall oedema. Delayed enhancement MRI showed no abnormal enhancement denoting no evidence of scarring (Fig. 4).

Discussion

Takotsubo cardiomyopathy is a rare syndrome also known as broken heart syndrome or apical ballooning syndrome typically associated with emotional or physical stress and is thought to be caused by a catecholamine-mediated injury [1, 2]. The pathologic role of catecholamines was suggested due to evidence of the occurrence of this syndrome in association with pheochromocytoma or iatrogenic catecholamine administration [1, 3]. Coronary micro-vascular spasm or direct toxicity are the possible mechanisms of catecholamine-induced myocardial damage.

Takotsubo cardiomyopathy is most commonly seen in women older than 60 years of age. It is characterized by a transient systolic dysfunction of the apical segments of the left ventricle and hyperkinetic base mimicking myocardial infarction in the absence of obstructive coronary artery disease (Fig. 6) [8]. It accounts for approximately 1.2% of all troponin-proven acute coronary syndrome events [1, 2.] The belief is that catecholamines act on adrenoreceptors that have their highest density within the apex of the heart in postmenopausal women, which explains the occurrence of the common apical variant in older women [4]. In classic takotsubo cardiomyopathy, left ventricular outflow tract obstruction from left ventricular basal hyperkinesis can contribute to shock or mitral regurgitation [7].

Reverse takotsubo, a rare variant form of takotsubo cardiomyopathy in which the basal and midventricular segments of the left ventricle are akinetic with normal apex, occurs in a minority of patients [1, 2]. Compared with patients who have apical ballooning, patients with reverse takotsubo cardiomyopathy present at a younger age, with a mean age of 36, and often they have an emotional or physical stress trigger [1, 5, 6]. The presentation of inverted takotsubo cardiomyopathy in younger patients may be due to the abundance of adrenoreceptors at the base of the heart, compared with in the apex in older patients.

Patients with reverse takotsubo cardiomyopathy may present with less pulmonary oedema, dyspnoea and cardiogenic shock than patients with classic takotsubo cardiomyopathy due to absence of hyperkinetic base seen in the apical form [5].

Patients with reverse takotsubo cardiomyopathy have been reported to have significantly higher levels of cardiac markers than patients with apical takotsubo cardiomyopathy [6]. This could be explained by the extent of myocardium involved in each form, with more myocardial tissue being affected in reverse rather than classic takotsubo cardiomyopathy [6].
The prognosis is similar in takotsubo and reverse takotsubo, most patients will recover within few weeks, yet recent studies showed that some cases may have residual myocardial delayed enhancement denoting permanent myocardial damage.

Differential Diagnosis List

Final Diagnosis

Reverse takotsubo cardiomyopathy

URL:	https://www.eurorad.org/case/14616
DOI:	10.1594/EURORAD/CASE.14616
ISSN:	1563-4086