【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.
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。
ObjectiveTo summarize the clinical characteristics and the long-term results of pulmonary thromboendarterectomy (PTE) in the chronic thromboembolic pulmonary hypertension (CTEPH) patients with unilateral main pulmonary artery occlusion.MethodsWe retrospectively analyzed the clinical data of 15 CTEPH patients with unilateral main pulmonary artery occlusion in Fuwai Hospital between 2004 and 2018. There were 11 males and 4 females aged 34.1±12.0 years at operation.ResultsThe mean circulatory arrest was 31.1±12.1 minutes. The ICU stay was 5 (2-29) d. The hospital stay was 15 (8-29) d. There was no hospital death. There was a decline in systolic pulmonary artery pressures (sPAP, 69.9±27.9 mm Hg to 35.1±9.7 mm Hg, P=0.020) after surgery. On postoperative V/Q scan, only 6 patients (40.0%) had significant improvement in reperfusion (≥75% estimated) of the occluded lung. There was no death during the median observation period of 49 months follow-up, while 2 patients had recurrence of pulmonary embolism.ConclusionCTEPH patients with unilateral main pulmonary artery occlusion represent a challenging cohort. PTE is a curative resolution in both early- and long- term results, although there is a high requirement of perioperative management and a high risk of postoperative complications and rethrombosis.
Medical nitric oxide (NO) flow control system plays an important role in lowering pulmonary hypertension. The design requirements, overall scheme, delivery system and hardware circuits of a medical NO flow control system were introduced in this paper. Particularly, we proposed the design of NO delivery system and hardware circuits in detail. To deliver nitric oxide of a variable concentration, the designed system needs to work with a ventilator. The system can adjust and monitor the inhaled nitric oxide concentrations and send out sound and light alarms when the inhaled nitric oxide concentrations are out of the set range. To validate reliability and efficacy, we measured specifications such as linearity, stability and response time of the proposed NO flow control system by continuously administering nitric oxide into inspiratory circuit to deliver nitric oxide of variable concentrations to a test lung. The experiments showed that these specifications can meet the desired requirements.
ObjectiveTo explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease.MethodsFrom May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed.ResultsPreoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥1.2 m/s at discharge was an independent risk factor for mortality and restenosis.ConclusionThe prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.
Pulmonary arterial hypertension (PAH) is a fatal and complex disease characterized by multifactorial involvement in pulmonary vascular remodeling, leading to heart failure. It is difficult to treat and has a poor long-term prognosis. Recent studies highlight the significant role of epigenetic modulation in the pathophysiological progression of PAH, offering new therapeutic approaches to improve clinical outcomes. This article summarizes the role of epigenetic modulation in the development and progression of PAH, focusing on deoxyribonucleic acid methylation, ribonucleic acid methylation, histone modifications, and non-coding ribonucleic acid, in order to understand the role of epigenetic modulation in PAH and identifying new evaluation indexes and therapeutic targets, thereby improving the prognosis of PAH.