Congenital Long QT Syndrome: a Systematic Review
Congenital long QT syndrome (LQTS) is a disorder of myocardial repolarization defined by a prolonged QT interval on electrocardiogram (ECG) that can cause ventricular arrhythmias and lead to sudden cardiac death. LQTS was first described in 1957 and since then its genetic etiology has been resear...
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Sestre Milosrdnice University hospital, Institute of Clinical Medical Research
2021-01-01
|
| Series: | Acta Clinica Croatica |
| Subjects: | |
| Online Access: | https://hrcak.srce.hr/file/399212 |
| Summary: | Congenital long QT syndrome (LQTS) is a disorder of myocardial repolarization
defined by a prolonged QT interval on electrocardiogram (ECG) that can cause ventricular arrhythmias
and lead to sudden cardiac death. LQTS was first described in 1957 and since then its genetic
etiology has been researched in many studies, but it is still not fully understood. Depending on the
type of monogenic mutation, LQTS is currently divided into 17 subtypes, with LQT1, LQT2, and
LQT3 being the most common forms. Based on the results of a prospective study, it is suggested that
the real prevalence of congenital LQTS is around 1:2000. Clinical manifestations of congenital LQTS
include LQTS-attributable syncope, aborted cardiac arrest, and sudden cardiac death. Many patients
with congenital LQTS will remain asymptomatic for life. The initial diagnostic evaluation of congenital
LQTS includes obtaining detailed personal and multi-generation family history, physical examination,
series of 12-lead ECG recordings, and calculation of the LQTS diagnostic score, called
Schwartz score. Patients are also advised to undertake 24-hour ambulatory monitoring, treadmill/cycle
stress testing, and LQTS genetic testing for definitive confirmation of the diagnosis. Currently
available treatment options include lifestyle modifications, medication therapy with emphasis on betablockers,
device therapy and surgical therapy, with beta-blockers being the first-line treatment option,
both in symptomatic and asymptomatic patients. |
|---|---|
| ISSN: | 0353-9466 1333-9451 |