The relationship between takotsubo syndrome, left ventricular hypertrabeculation/noncompaction, neurologic and neuromuscular disorders.

Abstract

Takotsubo syndrome (TTS) and left ventricular hypertrabeculation/noncompaction (LVHT) have in common that they are only diagnosed since 1990. Diagnostic criteria and prognosis of affected patients are still debated and the knowledge about etiology and pathogenesis of both disorders is limited. Both abnormalities are associated with neurologic and neuromuscular disorders (NMD). We summarize the data about the relationship between TTS, LVHT and NMD. We identified 8 case reports about the co-incidence of TTS and LVHT (6 females, 2 males, age 0-76 years). In 2/8 cases recurrent TTS occurred after 8 and 10 months. In most of the patients, LVHT was diagnosed together with TTS, thus, it cannot be assessed if LVHT was present since birth or developed during life-time. In one case, LVHT was absent in a previous echocardiogram, developed de novo during TTS, and disappeared after 3 months. In 4/8 patients follow-up were reported, and in all of them, regression or disappearance of LVHT was observed. NMD or psychiatric disorders were reported in 7/8 patients. We conclude -limited by the small number of cases-that patients with LVHT and TTS seem to be frequently associated with NMD and have a high risk of recurrence. LVHT seems to disappear after TTS, but it remains unclear whether trabeculations in fact regress or are still present, but not more visible because of a decrease in left ventricular size resulting in smaller spaces between the trabeculations. Patients with LVHT and TTS require long-term follow-up to assess any changes of these abnormalities over time.