肺动脉高压( pulmonary artery hypertension, PAH) 是右心衰竭的首要原因, 而右心衰竭又是 PAH 患者的重要致命因素, 及时而准确地对肺动脉高压患者的右心室功能做出评价, 对其病情评估、治疗决策和预后判断有重要的意义。近年来新发展的超声心动图二维应变及应变率技术、实时三维成像技术、Tei 指数及三尖瓣环收缩期位移等新技术弥补了传统超声评价右心功能的不足。本文就超声新技术在评价PAH 患者右心功能的应用做一综述。
【摘要】 目的 探讨肺动脉高压患者药物靶向治疗的效果与耐受性。 方法 回顾分析2008年1月〖CD3/5〗2009年8月期间8例肺动脉高压患者分别接受波生坦及西地那非治疗的临床资料,评估其临床表现、WHO肺动脉高压功能分级、6 min步行距离及肺动脉收缩压在基线及治疗3个月后的变化。 结果 治疗后3个月,患者均能耐受药物治疗,无严重不良反应发生。WHO肺动脉高压功能分级在治疗前平均(31±04),治疗后为(23±09),明显得到改善(Plt;005)。肺动脉收缩压在治疗前平均(695±112 ) mm Hg(1 mm Hg=0133 kPa),治疗后为(483±124) mm Hg,明显降低(Plt;005)。6 min步行距离在治疗前平均(324±48) m,治疗后为(400±43) m,明显延长(Plt;005)。 结论 肺动脉高压患者药物靶向治疗的疗效显著,且耐受良好。【Abstract】 Objective To examine the effects of target medical therapy in patients with pulmonary arterial hypertension(PAH). Methods To determine the safety and efficacy of bosentan and sildenafil in eight patients with PAH.The patients’ clinical features, six minutes walking diastance, WHO functional class and systolic pulmonary arterial pressure (SPAP) were measured at baseline and at three months after initiating target medial treatment. Results At the three months followup assessments, WHO functional class was improved with 31±04 vs 23±09 (Plt;005); SPAP was significantly decreased with(695±112 ) mm Hg vs (483±124) mm Hg (Plt;005), the six minutes walking distance was significantly increased with(324±48) m vs(400±43) m (Plt;005). Target medical treatment was well tolerated. Conclusion Target medical treatment is well tolerated and has beneficial effects on PAH.
Pulmonary hypertension is a disease characterized by pulmonary artery pressure increased, with or without small artery pathological change, which ultimately leads to right heart failure or even death. Pulmonary hypertension seriously threatens to human health, however, the pathogenesis of pulmonary hypertension is unclear. Previous studies have found that bone morphogenetic protein (BMP) signaling system played an important role in the progress of pulmonary hypertension. In the current review, we describe the mechanism of BMP4 in the development of pulmonary hypertension.
ObjectiveTo explore whether nesiritide (recombinant human brain natriuretic peptide, rh-BNP) could be used to treat pulmonary artery hypertension. MethodsA 34-year-old female patient with severe symptomatic idiopathic pulmonary artery hypertension was reported, who was refractory to routine therapies, including prostacyclin. Therapy with continuous nesiritide infusion resulted in significant decrease in pulmonary vascular resistance and an improvement in dyspnea. The relevant literature was reviewed. ResultsThe clinical symptoms of this patient relieved significantly after nesiritide therapy. Literature review showed that nesiritide could increase the production of nitrogen oxides and cyclic guanosine monophosphate in the body, so as to dilate the vessels which were shrunk due to hypoxia and low down the pulmonary vascular resistance. ConclusionNesiritide is useful to treat severe pulmonary artery hypertension, and combination with phosphodiesterase type-5 inhibitors may be a brand new therapy of value.
Objective To explore the effect of pulmonary arterial hypertension on the children with functional single ventricle in the early period after Fontan operation. Methods Forty-three children with pulmonary arterial hypertension after Fontan operation were enrolled in our department between January 2015 and December 2016. There were 24 males and 19 females at a median age of 4.3 years ranging from 2.5 to 4.8 years. The pulmonary arterial pressure was evaluated by cardiac catheterization. There were 23 children diagnosed without pulmonary hypertension (a non-PAH group) including 16 males and 7 females, while 20 patients were diagnosed with pulmonary hypertension (a PAH group) including 8 males and 12 females. Postoperative parameters related to outcomes were compared between the two groups. Results There was no death in the non-PAH group, but the mortality of children in the PAH group was 20.0% (4/20, χ2=5.34, P=0.02). The central venous pressure (t=–2.50, P=0.02), N-terminal prohormone of brain natriuretic peptide (NT-proBNP, Z=–3.50, P<0.01), peritoneal dialysis rate (χ2=5.40, P=0.02), incidence of arrhythmia (χ2=4.40, P=0.03) in the PAH group were significantly higher than those of the non-PAH group. The early postoperative utilization rate of pulmonary vascular targeting agents in the PAH group was significantly higher than that in the non-PAH group (χ2=6.30, P=0.04). Conclusion Pulmonary arterial hypertension is one of the most important factors which influence the early postoperative prognosis of children with functional single ventricle after Fontan operation.
Objective To investigate the effects of simvastatin on monocrotaline-induced pulmonary hypertension in rats, and explore the potential mechanism of simvastatin by blocking heme oxygenase-1( HO-1) expression. Methods 52 male Sprague-Dawley rats were randomly divided into five groups, ie. a control group, a simvastatin control group, a pulmonary hypertension model group, a simvastatin treatment group, a ZnPP ( chemical inhibitor of HO) group. Mean pulmonary arterial pressure ( mPAP) and right ventricular systolic pressure ( RVSP) were detected by right heart catheter at 5th week. Right ventricular hypertrophy index ( RVHI) was calculated as the right ventricle to the left ventricle plus septum weight. Histopathology changes of small intrapulmonary arteries were evaluated via image analysis system.Immunohistochemical analysis was used to investigate the expression and location of HO-1. HO-1 protein level in lung tissue were determined by western blot. Results Compared with the model group, simvastatin treatment decreased mPAP and RVHI significantly [ ( 35. 63 ±5. 10) mm Hg vs. ( 65. 78 ±15. 51) mm Hg,0. 33 ±0. 05 vs. 0. 53 ±0. 06, both P lt; 0. 05 ] . Moreover, simvastatin treatment partially reversed the increase of arterial wall area and arterial wall diameter [ ( 50. 78 ±9. 03 ) % vs. ( 65. 92 ±7. 19) % ,( 43. 75 ±4. 23) % vs. ( 52. 00 ±5. 35) % , both P lt; 0. 01) . In the model group, HO-1 staining was primarily detected in alveolar macrophages. Simvastatin treatment increased HO-1 protein expression significantly, especially in the thickened smooth muscle layer and alveolar macrophages. Inhibiting HO-1 expression using ZnPP resulted in a loss of the effects of simvastatin. mPAP in the ZnPP group was ( 52. 88±17. 45) mm Hg, while arterial wall area and arterial wall diameter were ( 50. 78 ±9. 03) % and ( 52. 00 ±5. 35) % , respectively. Conclusions Simvastatin attenuates established pulmonary arterial hypertension andpulmonary artery remodeling in monocrotaline-induced pulmonary hypertension rats. The effect of simvastatin is associated with HO-1.
ObjectiveTo describe the effect of sequential pulmonary balloon angioplasty for patients with chronic thromboembolic pulmonary hypertension, who was accompanied with progressed pulmonary hypertension after pulmonary endarterectomy surgeries.MethodsFrom 2014 to December 2017, 7 patients were treated with a combination therapy of pulmonary endarterectomy and sequential pulmonary balloon angioplasty. There were 1 male and 6 females at age of 58 (43–59) years. A follow-up period of more than 1 year was accomplished. The result of right sided heart catheterization and ultrasonic cardiogram between and after the pulmonary endarterectomy or balloon angioplasty was collected.ResultsSeven patients were treated with a combination of pulmonary endarterectomy and sequential pulmonary balloon angioplasty, which included 1 patient of single pulmonary balloon angioplasty and 6 patients of multiple pulmonary balloon angioplasties. The balloon dilation times was 2 (2–6), and the number of segments during each single balloon dilatation was 3–5, compared with the first clinical results before the first balloon dilation, systolic pulmonary artery pressure [53 (47–75) mm Hg vs. 45 (40–54) mm Hg, P=0.042), mean pulmonaryartery pressure [38 (29–47) mm Hg vs. 29 (25–39) mm Hg, P=0.043], N terminal-B type natriuretic peptide [1 872 (1 598–2 898) pg/ml vs. 164 (72–334) pg/ml, P=0.018] improved significantly after the last balloon angioplasty. Heart function classification (NYHA) of all the 7 patients were recovered to Ⅰ-Ⅱclasses (P<0.05).ConclusionSequential pulmonary balloon angioplasty after pulmonary endarterectomy can further reduce the patient's right heart after load, improve the heart function for patients with progressed pulmonary hypertension after pulmonary endarterectomy surgeries.