QT-prolongation and noncompaction

Abstract

In a recent article, Szulik et al. reported about a 22 years old female with ventricular fibrillation, QT-prolongation, and left ventricular hypertrabeculation/noncompaction (LVHT) who died from hypoxic cerebral damage 5 days after admission [1]. We have the following comments and concerns. Patients with LVHT have a disposition for any type of cardiac arrhythmia [2,3]. This is why ventricular fibrillation not only could be due to hereditary long-QT syndrome but also due to LVHT. Ventricular fibrillation was either due to LVHT or a consequence of QT-prolongation. QT-prolongation is not unusual in LVHT and has been reported in several cases (table 1) [3-7]. LVHT has been also reported in association with long-QT-syndrome due to mutations in the KCNQ1 gene [8], in the KCNH2 gene [9], or due to an unidentified genetic defect (table 1) [10]. In a study of 105 patients with LVHT, the QT-interval increased during a mean follow up of 3.6y in 15 patients and normalized in 21 patients [11]. The increase was associated with the extension of LVHT and the presence of a neuromuscular disorder (NMD) [11].