Novel insight into the natural history of short QT syndrome.

ABSTRACT:

Objectives

This study intends to gain further insights into the natural history, the yield of familial and genetic screening, and the arrhythmogenic mechanisms in the largest cohort of short QT syndrome (SQTS) patients described so far.

Background

SQTS is a rare genetic disorder associated with life-threatening arrhythmias, and its natural history is incompletely ascertained.

Methods

Seventy-three SQTS patients (84% male; age, 26 ± 15 years; corrected QT interval, 329 ± 22 ms) were studied, and 62 were followed for 60 ± 41 months (median, 56 months).

Results

Cardiac arrest (CA) was the most frequent presenting symptom (40% of probands; range, <1 month to 41 years). The rate of CA was 4% in the first year of life and 1.3% per year between 20 and 40 years; the pr

SUBMITTER: Mazzanti A 

PROVIDER: S-EPMC3988978 | biostudies-literature | 2014 Apr

REPOSITORIES: biostudies-literature