左心疾病相关性肺动脉高压

在疾病发展过程中,多种左心疾病可引起肺静脉高压,从而继发肺动脉高压。相关的左心疾病主要为①心力衰竭（心衰）,包括收缩和（或）舒张功能衰竭;②瓣膜病变,包括左房室瓣狭窄和关闭不全、主动脉瓣狭窄和关闭不全;③三房心。肺静脉高压和肺动脉高压是这些疾病发展过程中的现象之一。     

原发性高血压伴冠心病患者大动脉弹性的研究

目的:研究原发性高血压(EH)伴冠心病患者大动脉弹性功能的改变情况。方法:将患者分为EH组与对照组,前者根据有无冠心病进一步分为高血压伴冠心病及不伴冠心病组。应用脉搏波速度(pulse wavevelocity,PWV)测定系统测量颈动脉-股动脉脉搏波速度(C-F PWV)作为评价大动脉弹性功能的指标。结果:EH伴冠心病患者的C-F PWV较不伴冠心病患者明显增快。年龄、脉压是影响C-F PWV的独立因素。结论:EH伴冠心病患者大动脉弹性减退,与年龄和脉压有关。     

Congenital heart disease associated pulmonary arterial hypertension

Pulmonary arterial hypertension associated with congenital heart disease, although common (15%-30%) in all-comers with congenital heart disease, is variable in terms of clinical manifestations, severity of associated pulmonary arterial hypertension, and response to therapy and outcomes (depending on lesion anatomy, pulmonary circulation flows and pressures, and presence and timings of surgeries). Evaluation includes imaging and catheterization. Surgical or another anatomic correction may be desirable after rigorous preinterventional assessment. Patients who are not surgical candidates or who fail to improve early or late after surgery may have the potential to respond to idiopathic pulmonary arterial hypertension therapies. Lung or heart/lung transplantation remains an option for selected recalcitrant patients.     

先天性心脏病相关肺动脉高压患者的管理

先天性心脏病相关肺动脉高压的发病机制复杂,本病对患者的生活质量有相当大的影响。虽然目前对这类疾病尚无有效的治疗措施,但通过合理的治疗干预可以延长患者的生存时间,改善其临床症状和生活质量。因此,有必要通过多学科小组的交流对这类患者进行适当的管理,以改善患者的生活质量和预后。     

���������ಡ��طζ�����ѹ�������Ʒ���

??Abstract??Pulmonary arterial hypertension (PAH) commonly arises in patients with congenital heart disease (CHD).Management of PAH-CHD can involve surgical correction of the cardiac defect and/or treatment of the PAH.Prostacyclin analogues??phosphodiesterase type-5 inhibitors and bosentan may have benefits in advanced pulmonary vascular disease.Transplantation surgery can be curative but is not without limitations.The timing of intervention in patients with PAH-CHD is important.     

冠心病合并高血压患者血压控制程度对动脉弹性功能的影响

目的 探讨冠心病合并高血压患者血压控制程度对动脉弹性功能的影响.方法 选择经过冠状动脉造影证实的冠心病合并高血压患者46例,全部患者均经过降压药物治疗.根据血压水平将患者分为2组,其中23例为血压控制不良组(收缩压≥140 mm Hg或舒张压≥90mmHg),另23例为血压控制良好组(收缩压＜ 140 mm Hg且舒张压＜90 mm Hg)作为配对(1∶1).对2组患者血压、血管弹性功能[颈动脉-股动脉脉搏波速度(cfPWV),系统血管顺应性(SVC)、肱动脉扩张性(BAD)、系统血管阻力(SVR)]进行测定和分析.结果 (1)在血压控制良好组cfPWV(m/s)明显低于控制不良组(10.38±1.73比12.83±3.13,P＜0.01).(2)血压控制良好组的SVC(ml/mm Hg)、BAD(％/mm Hg)与血压控制不良组相比增高(1.16±0.23比0.94±0.17,6.01±0.98比4.63±0.71,均为P＜0.01).(3)cfPWV与年龄、收缩压、脉压正相关,相关系数r分别为0.374、0.416、0.507.多元线性逐步回归分析显示,cfPWV=4.796+0.099×脉压,即cfPWV与脉压成线性关系,提示收缩压及脉压增大加重患者的大动脉功能减退.结论 冠心病合并高血压患者存在血管弹性功能异常,并与血压控制不良直接相关.血压控制良好的患者SVC、BAD、cfPWV均有改善,冠心病合并高血压患者需要良好的血压控制.     

Regional arterial stiffness associated with ischemic heart disease in type 2 diabetes mellitus

Arterial stiffness is increased in type 2 diabetes mellitus, and diabetes preferentially affects arterial stiffness of the central (elastic, capacitive) over peripheral (muscular, conduit) arteries. We hypothesized that arterial stiffness of the central artery may be more closely associated with ischemic heart disease (IHD) than stiffness of peripheral arteries in type 2 diabetes mellitus. The subjects were 595 type 2 diabetes patients including 70 with IHD. Arterial stiffness was measured as pulse wave velocity (PWV) in the heart-carotid, heart-femoral, heart-brachial, and femoral-ankle regions. The PWV values of the four segments correlated with each other in patients without IHD. However, the correlations were less impressive in those with IHD, suggesting unequal stiffening of regional arteries in IHD. As compared with patients without IHD, the IHD group showed significantly higher PWV values of the four arterial segments, particularly of the heart-femoral region. The presence of IHD was significantly associated with higher heart-femoral PWV, and this association remained significant and independent of other factors in a multiple logistic regression analysis. Pulse pressure was more strongly correlated with PWV of the heart-femoral than other arterial regions. Thus, diabetic patients with IHD have increased stiffness of arteries, particularly of the aorta, supporting the concept that central arterial stiffness plays an important role in the development of IHD.     

Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease

Background

The objective of this prospective study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension (CHD–APAH).

Methods

Patients with invasively confirmed CHD–APAH received in-hospital exercise training for 3 weeks and continued at home. Efficacy parameters were evaluated at baseline, after 3 and 15 weeks. Medical treatment remained unchanged. Worsening events and survival rate were assessed in a follow-up period of 21 ± 14 months.

Results

Twenty consecutive CHD–APAH patients (16 female, 4 male, mean pulmonary arterial pressure 60 ± 23 mm Hg) were included. Patients significantly improved the mean distance walked in 6 min compared to baseline by 63 ± 47 m after 3 weeks (p < 0.001) and by 67 ± 59 m after 15 weeks (p = 0.001). Quality of life-score (p = 0.05), peak oxygen consumption (p = 0.002) and maximal workload (p = 0.003) improved significantly by exercise training after 15 weeks. The 1- and 2‐year survival rates were 100%, the transplantation-free survival rate was 100% after 1 year and 93% after 2 years.

Conclusion

Exercise training as add-on to medical therapy may be effective in patients with CHD–APAH and improved work capacity, quality of life and further prognostic relevant parameters. It was associated with an excellent long-term survival. Further randomized controlled studies are needed to confirm these results.     

Risk of premature atherosclerosis and ischemic heart disease associated with HIV infection and antiretroviral therapy

The use of new potent protease inhibitor-based antiretroviral therapies in patients with human immunodeficiency virus (HIV) infection has been increasingly associated with cardiovascular risk factors, including hyperlipidaemia, fat redistribution syndrome, insulin resistance, and diabetes mellitus. The introduction of highly active antiretroviral therapy (HAART) in clinical practice has remarkably changed the natural history of HIV disease, leading to a notable extension of life expectancy, and prolonged lipid and glucose metabolism abnormalities are expected to lead to significant effects on the long-term prognosis and outcome of HIV-infected patients. Prediction modeling, surrogate markers and hard cardiovascular endpoints suggest an increased incidence of cardiovascular diseases in HIV-infected subjects receiving HAART, even though the absolute risk of cardiovascular complications remains still low, and must be balanced against the evident virological, immunological, and clinical benefits descending from combination antiretroviral therapy. Nevertheless, the assessment of cardiovascular risk should be performed on regular basis in HIV-positive individuals, especially after initiation or change of antiretroviral treatment. Appropriate lifestyle measures (including smoking cessation, dietary changes, and aerobic physical activity) are critical points, and switching HAART may be considered, although maintaining viremic control should be the main goal of therapy. Pharmacological treatment of dyslipidaemia (usually with statins and fibrates), and hyperglycaemia (with insulin-sensitizing agents and thiazolidinediones), becomes suitable when lifestyle modifications and switching therapy are ineffective or not applicable.     

先天性心脏病继发性肺动脉高压患者血浆中miRNA的研究

目的 应用微小RNA （microRNA,miRNA）芯片技术研究先天性心脏病合并重度肺动脉高压（pulmonary arterial hypertension,PAH）患者和不合并PAH患者血浆中miRNA表达谱的差异,并初步预测差异表达的miRNA调控的靶基因.方法 收集室间隔缺损（ventricular septal defect,VSD）合并重度PAH（PAH组）和不合并PAH患者（对照组）的血浆.分别提取总RNA,然后采用miRNA芯片进行miRNA表达谱差异分析,并对结果进行实时定量聚合酶链反应（polymerase chain reaction,PCR）验证.运用Targetscan、Pictar、Miranda软件预测可能调控的靶基因.结果 miRNA芯片结果提示：与对照组相比,左向右分流先天性心脏病继发重度PAH患者血浆中表达上调的miRNA有50个,表达下调的miRNA有36个.实时定量PCR验证miR-98与芯片结果一致,表达上调.靶基因预测软件显示内皮素（ET-1）为hsa-miR-98的重要靶基因.结论 miRNA在先天性心脏病继发重度PAH患者血浆中存在差异性表达,miRNA可能与PAH的发生、发展密切相关,血浆miR-98有可能成为先天性心脏病合并重度PAH的新的分子生物学标志物.     

Pulmonary arterial hypertension associated with congenital heart disease: Recent advances and future directions

Congenital heart disease (CHD), the most common inborn defect, affects approximately 1% of all newborns worldwide. Advances in its diagnosis and treatment have led to a dramatic improvement in patients' quality of life and long-term survival prospects. However, recently it has been realised that many of these patients are affected by ongoing and life-long cardiac issues, namely residual and progressive haemodynamic lesions, arrhythmia and sudden cardiac death, as well as the development of chronic heart failure and pulmonary arterial hypertension (PAH) — all of which merit tertiary care. Unfortunately, many patients with CHD are lost to follow-up, due to the assumption that their initial response to surgical and or catheter intervention in childhood led to cure. Furthermore, there are many patients with undiagnosed or unoperated CHD in the developing world coming to medical attention during adulthood. Our article focuses on advances in the management of PAH associated with CHD, a common association with an adverse impact on quality of life and survival prospects that affects approximately 10% of patients with CHD. Much of the recent progress in PAH–CHD has focused on the extreme end of the disease spectrum, namely on Eisenmenger syndrome. Herein we discuss this progress and future directions for this emerging cardiovascular field.     

Elevated endothelin-1 levels are associated with decreased arterial elasticity in hypertensive patients

Endothelin-1 stimulates collagen synthesis and is increased in hypertension, but its effect on collagen degradation remains unknown. The current study tested the hypothesis that elevated endothelin-1 levels are associated with decreased collagenase activity, markers of collagen degradation, and arterial compliance in hypertensive patients. Normotensive (n = 10) and hypertensive (n = 13) patients who were not on any antihypertensive medication were recruited, and small and large artery elasticity index, systemic vascular resistance, pulse pressure, and blood pressure were determined using blood pressure waveform analysis. Large artery elasticity index and collagen degradation products were decreased whereas endothelin-1, systemic vascular resistance, and pulse pressure were elevated in hypertensive patients. Plasma endothelin-1 was negatively correlated with a cross-linked C-terminal telopeptide of collagen type I, a collagen degradation marker (r = -0.43; p = 0.04), collagenase matrix metalloproteinase-1 (r = -0.48; p = 0.02), and large artery elasticity (r = -0.45; p = 0.03) and positively correlated with pulse pressure (r = 0.68; p = 0.0005). These results suggest that endothelin-1 contributes to decreased arterial compliance in hypertension via inhibition of collagen degradation.     

冠心病患者血管内皮功能障碍与动脉弹性关系的研究

目的　探讨冠心病患者血管内皮功能障碍与动脉弹性的关系。方法　采用高分辨率血管超声法检测 30例冠心病患者与 30例正常对照组肱动脉血流介导的内皮依赖性血管舒张功能(FMD);应用动脉弹性功能检测仪测定受试者的大动脉弹性指数 (C1 )和小动脉弹性指数 (C2 )。结果　冠心病组血流介导的肱动脉舒张反应明显低于对照组[ (5 17±2 13)% 与 (11 10±4 36)%,P<0 05];冠心病组与正常对照组的C1 差异无统计学意义 [ ( 11 59±4 56 )ml/mmHg( 1mmHg=0 133kPa) ×10与 (12 11±3 82)ml/mmHg×10, P>0 05],但冠心病组的C2 明显低于正常对照组[ (4 20±1 80)ml/mmHg×100与 (6 26±2 36)ml/mmHg×100, P<0 05],冠心病组血流介导的肱动脉舒张反应与C2 呈正相关(r=0 53, P<0 05)。结论　冠心病患者肱动脉内皮依赖血管舒张功能受损和C2 降低,且两者之间呈正相关,提示C2 可作为一种评价血管内皮功能的新指标。