CLinicaL characteristics and treatment of congenitaL Long QT syndrome in 58 chiLdren
10.3760/cma.j.issn.0578-1310.2019.04.008
- VernacularTitle:儿童先天性长QT综合征58例临床特征及治疗分析
- Author:
Haiyan GE
1
;
Xiaomei LI
;
He JIANG
;
Meiting LI
;
Yi ZHANG
;
Haiju LIU
Author Information
1. 清华大学第一附属医院心脏中心小儿科
- Keywords:
Tachycardia;
ChiLd;
Diagnosis;
Genes
- From:
Chinese Journal of Pediatrics
2019;57(4):272-276
- CountryChina
- Language:Chinese
-
Abstract:
Objective To assess the cLinicaL characteristics, pathogenic genes and therapeutic effects of congenitaL Long QT syndrome (LQTS) in chiLdren. Methods A retrospective anaLysis incLuded 58 LQTS chiLdren (37 boys, 21 girLs; age of diagnosis (8.0±4.1) years, range 0.1 to 16.0 years) at Division of Pediatric CardioLogy, First HospitaL of Tsinghua University from August 2013 to November 2017. Each patient was evaLuated with a detaiLed medicaL history, 12?Lead resting eLectrocardiogram, DoppLer echocardiography, and moLecuLar genetic anaLysis. ResuLts Forty?eight of the chiLdren (83%) had a deLay to diagnosis (0.7 (0.1, 2.0) years) and initiaLLy received a misdiagnosis. QT proLongation of unknown origin was found in 10 cases (17%), compLex arrhythmic conditions in 27 cases (47%), myocarditis in 3 cases (5%), syncope of unknown origin in 3 cases (5%), epiLepsy in 2 cases (3%), myocardiaL infarction in 1 case (2%), cardiomyopathy in 1 case (2%), and vasovagaL syncope in 1 case (2%). Nine chiLdren presented with the positive famiLy history of LQTS and three chiLdren had congenitaL nervous deafness. Twenty?one (36%) chiLdren presented with recurrent syncope, and 14 cases of whom had symptoms during physicaL activity and/or emotionaL stress. The common arrhythmias were ventricuLar arrhythmia (26 cases), sinus node dysfunction (18 cases), atrioventricuLar bLock (AVB) (12 cases), and atriaL arrhythmia (6 cases). LQTS?associated pathoLogic or possibLy pathoLogic mutations were found in 41 chiLdren (71%). Thirty?three chiLdren (57%) were treated with propranoLoL (22 cases), permanent pacemaker (PM) combined with propranoLoL (5 cases), PM (4 cases), and impLantabLe cardioverter defibriLLator (ICD) combined with propranoLoL (2 cases). Eighteen chiLdren (55%) were asymptomatic, thirteen chiLdren (39%) reported infrequent syncope, and one case (2%) died. ConcLusions LQTS in chiLdren is potentiaLLy maLignant and present as phenotypic diversity and compLex arrhythmias. LQTS?reLated pathogenic or possibLy pathogenic mutations are identified in most of the chiLdren. Beta?bLockers therapy is effective in reducing the risk of maLignant cardiac events. Some chiLdren with LQTS shouLd receive PM or ICD therapy.
