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

<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>

Plasma levels of vWF and NO in patients with metabolic syndrome and their relationship with metabolic disorders / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>To measure the plasma levels of von Willebrand factor (vWF) and nitric oxide (NO) in patients with metabolic disorders and to study their relationships with the disease.</p><p><b>METHODS</b>The plasma levels of vWF and NO were determined in patients with metabolic syndrome (MS group, n=36), patients with 1 - 2 metabolic disorders (MD group, n=43) and normal subjects (control group, n=30).</p><p><b>RESULT</b>The plasma vWF level was higher in MS group than that in MD group (P <0.05) and in control group (P <0.001); the vWF level in MD group was higher than that in control group (P <0.05). The NO level was lower in MS group and in MD group than that in control group (both P<0.05), while the difference between MS and MD groups was not statistically significant (P >0.05). Multiple stepwise regression showed that vWF level was correlated with systolic BP (SBP), diastolic BP(DBP) and pulse BP; that NO level was correlated with BMI, SBP and TG.</p><p><b>CONCLUSION</b>Multiple metabolic disorders of metabolic syndrome may injure endothelial cells, and the degree of endothelial cell injury seems to be correlated with the BMI, SBP, DBP, pulse BP and TG.</p>