Noncompaction and Takotsubo Syndrome in a Neuromuscular Disorder

Takotsubo syndrome (TTS) in patients with left ventricular hypertrabeculation/noncompaction (LVHT) has been reported in four patients, and a TTS plus LVHT plus a neuromuscular disorder (NMD) was only reported once so far. Here, we present the fifth patient with LVHT and TTS and the second patient with LVHT, TTS, and a NMD.

The patient is a 68 yo female hobby choir singer with a history of skin dermatofibroma, skin fibrokeratoma, arterial hypertension, hyperlipidemia, hypothyroidism, anemia, hyponatremia, diverticulosis, LVHT detected at age 60 y, five syncopes, a liver cyst, and carotid endarterectomy 2 months prior to admission because of sudden-onset chest pain. Workup revealed ST elevation, troponin elevation, and mild coronary artery sclerosis. Ventriculography and transthoracic echocardiography (TTE) showed the apical type of a TTS. ECG normalised within 10 w and TTE within 6 w under beta-blockers and ATII-blockers. The TTS was triggered by being offended of being unable to sing anymore after endarterectomy. Neurological workup suggested the presence of a NMD.

This case shows that LVHT occurs in NMD patients and that patients with LVHT and a NMD may develop a TTS. Whether patients with LVHT and a NMD are particularly prone to develop a TTS requires further confirmation. NMD patients with LVHT should avoid stress not to trigger a TTS.