目的 研究高血压病（EH）合并动脉粥样硬化患者大动脉缓冲功能的改变。方法 选择313例EH患者，其中男性230 例，女性83例，平均年龄（58.5±10.1）岁。应用脉搏波速度（pulsewave velocity， PWV）自动测量系统测定颈动脉-股动脉PWV作为反映大动脉节段扩张性的参数；B型超声对颈动脉进行扫查，动脉粥样硬化的定义为内膜-中层厚度≥1.3mm。颈动脉横断面顺应性和容积扩张性作为评价大动脉缓冲功能的指标。结果 313例EH患者合并颈动脉粥样斑块者120例。与未合并颈动脉粥样硬化患者相比，EH合并颈动脉粥样硬化患者收缩压[（149.76±20.20）mm Hg比（141.62±18.94）mm Hg] 、脉压[（61.72±18.94）mm Hg 比（52.84±14.58）mm Hg]和PWV[（12.91±2. 93）m/s比（10.78±2.02）m/s]显著升高（P均<0.001）；颈动脉横断面顺应性[（64.68±40.52）10-3-mm2/mm Hg 比（81.55±63.45）10-3-mm2/mm Hg]和容积扩张性[（1.71±1.40）10-3-mm Hg 比（2.39±2.01）10-3-mm Hg]显著降低（P均<0.05）。结论 EH合并颈动脉粥样硬化者大动脉弹性减退，缓冲功能显著降低。

The long QT syndrome (LQTS) is a cardiac disorder characterized by prolongation of the QT interval on electrocardiograms (ECGs), syncope and sudden death caused by a specific ventricular tachyarrhythmia known as torsade de pointes. LQTS is caused by mutations in ion channel genes including the cardiac sodium channel gene SCN5A, and potassium channel subunit genes KCNQ1, KCNH2, KCNE1, and KCNE2. Little information is available about LQTS mutations in the Chinese population. In this study, we characterized 42 Chinese LQTS families for mutations in the two most common LQTS genes, KCNQ1 and KCNH2. We report here the identification of four novel KCNQ1 mutations and three novel KCNH2 mutations. The KCNQ1 mutations include L191P in the S2-S3 cytoplasmic loop, F275S and S277L in the S5 transmembrane domain, and G306V in the channel pore. The KCNH2 mutations include L413P in transmembrane domain S1, E444D in the extracellular loop between S1 and S2, and L559H in domain S5. The location and character of these mutations expand the spectrum of KCNQ1 and KCNH2 mutations causing LQTS. Excitement, exercises, and stress appear to be the triggers for developing cardiac events (syncope, sudden death) for LQTS patients with KCNQ1 mutations F275S, S277L, and G306V, and all three KCNH2 mutations L413P, E444D and L559H. In contrast, cardiac events for an LQTS patient with KCNQ1 mutation L191P occurred during sleep or awakening from sleep. KCNH2 mutations L413P and L559H are associated with the bifid T waves on ECGs. Inderal or propanolol (a beta blocker) appears to be effective in preventing arrhythmias and syncope for an LQTS patient with the KCNQ1 L191P mutation.

我们对31例具有适宜于二尖瓣球囊扩张（PBMV）的瓣膜条件但又合并左心房血栓的患者进行了小剂量华法令（2mgöd）抗凝治疗观察。所有左心房血栓均由经食道超声心动图证实和随访，体积最大者3cm×4cm×6cm，最小者1cm×1cm×0.5cm，随访期间定期复查超声心动图、凝血酶原时间（PT）和凝血酶原活动度（PTA）。结果显示：除了3 例患者失访外，其余28例均随访到血栓消失，血栓消失的时间在2～12个月之间，8517%（24ö28）的患者血栓消失的时间是6个月以内；PT较服药前部分延长，PTA轻度下降，无出血并发症和过度抗凝征象；对这些患者在血栓消失后成功地进行了PBMV。结果表明对于二尖瓣狭窄合并左心房血栓患者，小剂量华法令的抗凝溶栓作用安全可靠，药物服用时间以6个月为宜。

To assess the clinical features and the management of congenital long QT syndrome (LQTS) in China, we collected the clinical data of 54 LQTS patients (40 females) from our newly established national registry. All patients were symptomatic, with syncope being the most common symptom. The average age of first symptoms was 17.9±15.6 (rang, 0.5-62) years; 55.6% of them had the first symptoms before the age of 20. The most common triggers of the symptoms were physical exercises or emotional stress. The average corrected QT interval was 0.55±0.08s. Using ECG criteria, there were 14 (25.9%) LQT1 patients, 28 (51.9%) LQT2 and 2 (3.7%) LQT3. Thirty (55.6%) patients were treated with β-blockers at the time of enrollment, with propranolol being the most commonly used drug on an average daily dose of 57.5+39.1 mg. Four patients underwent left cardiac sympathectomy. After an average of follow-up of 24.9+13.2 months, 3.1% (1/32) of patients with antiadrenergic therapy, and 9.1% (2/22) without antiadrengergic therapy, died of sudden cardiac death (p<0.05). We concluded that LQT2 might be the most common subtype inthese patients. Antiadrengergic treatment were underused, raising the urgent need for educating both physicians and patients on the nature of the disease and its optimal antiadrenergic therapy.

ensive subjects and in patients with end-stage renal disease, but there have been few studies on PWV in Chinese patients with essential hypertension. In this cross-sectional study, we investigated 3,156 consecutive patients (mean age: 53.7±11.58 years) of the Hypertension Division of Ruijin Hospital in Shanghai. Together with sphygmomanometric blood pressure measurements, aortic PWV was measured using a validated automatic device. PWV in patients with pulse pressure (PP) 60 mmHg was significantly greater than that in patients with PP＜60 mmHg ( p＜0.01). PP and PWV were positively related to age (PP: r＝0.396, p＝0.001; PWV: r＝0.531, p＝0.001). After adjustment by age and heart rate, PWV was still closely related to PP (r＝0.249, p＝0.001). At any given systolic blood pressure (SBP), PWV significantly decreased with the increase of diastolic blood pressure (DBP), whereas at any given DBP there was a significant increase of PWV with the increase of SBP. In conclusion, PWV was the major determinant of PP, and was highest in Chinese patients with isolated systolic hypertension, followed by those with systolic and diastolic hypertension, isolated diastolic hypertension, and normal blood pressure.