Ventricular Septal Perforation Associated with Takotsubo Syndrome and Its Pathogenic Mechanisms

Takotsubo syndrome (TTS), once considered a benign self-limiting disease, is now recognized to have substantial short-term morbidity and mortality. During the acute phase, it can result in severe heart failure, cardiogenic shock, arrhythmias, cardiac rupture, ventricular septal perforation (VSP), and thromboembolic events, with in-hospital mortality rates comparable to those observed in acute coronary syndrome. Although VSP is rare, it represents a potentially lethal complication of TTS. Herein, we present a case of VSP associated with TTS and discuss a mechanism for the development of VSP in TTS.

A 69-year-old female presented with dyspnea. The diagnosis of TTS was established based on the presence of apical ballooning of the left ventricle (LV), hyperkinesis of the LV basal walls, normal coronary arteries, and apical defect in myocardial scintigraphy. Additionally, a VSP was identified, and given the stabilization of hemodynamics with an intra-aortic balloon pump and medical treatment, it was deemed preferable to delay surgical treatment. Approximately 1 month later, after the LV wall motion abnormality had been normalized, the VSP was successfully closed. Histopathological examination of the ventricular septum revealed contraction band necrosis along with fibrosis and focal cardiomyocyte necrosis.

While VSP is a potentially lethal complication of TTS, recovery of LV function can be anticipated, suggesting that surgical treatment could be deferred, provided that hemodynamic stability is maintained. This case demonstrates that focal myocardial necrosis can occur in TTS. Furthermore, increased wall tension occurs in the ballooning segments during systole, as the LV radius increases and the ventricular wall fails to thicken, in accordance with Laplace's law. Both myocardial fragility resulting from focal myocardial necrosis and increased LV wall tension due to systolic ballooning are considered to be key mechanisms underlying the development of VSP in TTS.