Clinical characteristics and treatment of a Chinese family with congenital short QT syndrome / 中华心血管病杂志

ABSTRACT

<p><b>OBJECTIVE</b>To observe the clinical characteristics and therapeutic efficacy of a Chinese family with congenital short QT Syndrome (SQTs).</p><p><b>METHODS</b>Fourteen family members including the proband were screened with routine clinical examination, serum electrolyte, serum myocardial enzymes, electrocardiography (ECG), Holter recording, treadmill exercise test, echocardiography and chest radiograph. High risk patient received intracardiac electrophysiological study (EPS).</p><p><b>RESULTS</b>Among the fourteen family members, 4 members died of sudden cardiac death at young ages, 4 members including the proband and his two daughters and one son were diagnosed as SQTs with structurally intact hearts. Initial ECG in these subjects revealed sinus rhythm with a high peaked T wave, QTc interval </= 320 ms and QT/QTp < 80%. The proband was a 45-year-old man presented with occasional dizziness and family history of sudden cardiac death. Polymorphic ventricular tachyarrhythmias and ventricular fibrillation as well as syncope were induced in this patient during programmed stimulation (S1 = 400 ms, S2 = 250 ms, S3 = 140 ms) from right ventricular outflow tract and a dual chamber Implantable Cardioverter Defibrillator (ICD) was implanted and the patient remained asymptomatic during the 6 months follow up.</p><p><b>CONCLUSIONS</b>SQTs could be presented as an inherited disorder with increased risk for arrhythmias and sudden cardiac death. ICD implantation is the first-choice therapy for high risk patients.</p>