The Pivotal Role of Echocardiography in the Diagnosis of Stress-Induced Cardiomyopathy Presenting with Atypical Pattern in Critically Ill Children. An Illustrative Case Report

Background: Takotsubo cardiomyopathy (TCM) has some distinctive features like greater proportion of reverse-TCM and central nervous system disease as a prevalent triggering cause. We expose the case of a child with cardiogenic shock presenting an atypical echocardiographic TCM pattern on an echocardiography, after an acute neurologic trigger. We also include a systematic literature review of previously described cases of atypical-TCM in children.

Case report: A previously healthy 9 year-old boy with status epilepticus presented abrupt cardiogenic shock. The EKG showed signs of myocardial ischemia, cardiac biomarkers NT-proBNP (2756 pg/mL ) and Troponin I (1707 pg/mL ) , and the echocardiography exposed a dilated LV with severely reduced systolic function (LVEF 28%) along with hypokinetic mid-basal segments (circumferential ballooning), and preserved hypercontractile apical segments, with the normal origin of both coronary arterial systems. A presumptive diagnosis of "reverse", "inverse" or atypical Takotsubo cardiomyopathy was built based on the echocardiographic findings, apart from the ACS-like EKG findings, the raised cardiac biomarkers, and the neurological trigger of the hyper catecholaminergic state. Despite cardiovascular improvement with supportive treatment, the patient eventually expired on day 2 after PICU admission due to neurological complications. As shown in our systematic review, only 19 similar cases have been reported to date.

Conclusion: With the report of this unusual case, we aim to point out the fundamental role of bedside echocardiography as a diagnostic test for critically ill children presenting with ACS-like in the context of neurosurgical emergencies, where bedside echocardiography itself can accurately establish a presumptive diagnosis of TCM.