A case report of a secondary Tako-Tsubo syndrome after Sudden Cardiac Arrest in a teenager with LQTS2

Abstract Background Takotsubo syndrome (TTS) is a condition first identified in the 1990s in the Japanese population. It is believed that 1-2% of acute coronary syndromes are due to TTS. The pathophysiological mechanism involves acute activation of the sympathetic nervous system with a cataclysmic release of catecholamines, causing acute myocardial dysfunction. Case Summary We report a case of an 18-year-old female patient who suffered an out-of-hospital cardiac arrest due to ventricular fibrillation of unknown etiology. Emergency cardiac catheterization revealed normal coronaries and hypokinesia of the apical segments. The patient was admitted to the ICU with refractory cardiogenic shock, necessitating extracorporeal life support measures. Serial echocardiograms showed rapid deterioration of LV-function. The diagnosis of TTS was confirmed with an early cardiovascular magnetic resonance (CMR) study. A QTc prolongation was observed intermittently. The patient showed an excellent neurological outcome and was discharged after ICD implantation for ambulatory care. Genetic testing revealed a previously unpublished KCNH2 gene mutation. Mutations in this gene are known to cause Long QT syndrome (LQTS). Discussion Our case presents a secondary TTS after survived sudden cardiac arrest in a teenager with so far unknown LGTS2 associated ventricular fibrillation. We emphasize the importance of repeated ECG recordings and serial echocardiography in unexplained cardiogenic shock. Our case highlights the importance of an early multimodal therapeutic approach in the management of TTS to improve patient outcomes in this complex clinical entity.