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Acute pulmonary arterial hypertension (PAH), which may complicate the course of many complex disorders, is always underdiagnosed and its treatment frequently begins only after serious complications have developed. Acute PAH is distinctive because they differ in their clinical presentation, diagnostic findings, and response to treatment from chronic PAH. The acute PAH may take either the form of acute onset of chronic PAH or acute PAH or surgery‐related PAH. Significant pathophysiologic differences existed between acute and chronic PAH. Therapy of acute PAH should generally be aimed at acutely relieving right ventricular (RV) pressure overload and preventing RV dysfunction. There are three classes of drugs targeting the correction of abnormalities in endothelial dysfunction, which have been approved recently for the treatment of PAH: (1) prostanoids; (2) endothelin receptor antagonists; and (3) phosphodiesterase‐5 inhibitors. The efficacy and safety of these compounds have been confirmed in uncontrolled studies in patients with PAH. Intravenous epoprostenol is suggested to serve as the first‐line treatment for the most severe patients. In the other situations, the first‐line therapy may include bosentan, sildenafil, or a prostacyclin analogue. Recent advances in the management of PAH have markedly improved prognosis.  相似文献   

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Background

Diabetes mellitus is associated with left ventricular hypertrophy and dysfunction. Parallel studies have also reported associations between diabetes mellitus and right ventricular dysfunction and reduced survival in patients with pulmonary arterial hypertension. However, the impact of diabetes mellitus on the pulmonary vasculature has not been well characterized. We hypothesized that diabetes mellitus and hyperglycemia could specifically influence right ventricular afterload and remodeling in patients with Group I pulmonary arterial hypertension, providing a link to their known susceptibility to right ventricular dysfunction.

Methods

Using an adjusted model for age, sex, pulmonary vascular resistance, and medication use, associations of fasting blood glucose, glycated hemoglobin, and the presence of diabetes mellitus were evaluated with markers of disease severity in 162 patients with pulmonary arterial hypertension.

Results

A surrogate measure of increased pulmonary artery stiffness, elevated pulmonary arterial elastance (P = .012), along with reduced log(pulmonary artery capacitance) (P = .006) were significantly associated with the presence of diabetes mellitus in patients with pulmonary arterial hypertension in a fully adjusted model. Similar associations between pulmonary arterial elastance and capacitance were noted with both fasting blood glucose and glycated hemoglobin. Furthermore, right ventricular wall thickness on echocardiography was greater in pulmonary arterial hypertension patients with diabetes, supporting the link between right ventricular remodeling and diabetes.

Conclusion

Cumulatively, these data demonstrate that an increase in right ventricular afterload, beyond pulmonary vascular resistance alone, may influence right ventricular remodeling and provide a mechanistic link between the susceptibility to right ventricular dysfunction in patients with both diabetes mellitus and pulmonary arterial hypertension.  相似文献   

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肺动脉高压是以肺小动脉的血管痉挛、内膜增生、重构为主要特征的一种疾病。肺小动脉的血管增生、重构导致肺血管阻力进行性增加,最终导致右心衰竭和死亡。在过去的20多年,随着我们对肺动脉高压病理机制的认识增加,该病的治疗也获得了很大的进步。除了传统的吸氧、利尿、强心、钙离子拮抗剂、抗凝等治疗,靶向药物治疗的研发与推广使用使肺动脉高压患者的预后得到了明显的改善,目前主要的靶向药物治疗包括:前列环素类似物、内皮素受体拮抗剂、磷酸二酯酶-5抑制剂,且一些新型的药物及联合治疗方案也在研究中。现就肺动脉高压的药物治疗进展简要地做一综述。  相似文献   

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肺动脉高压是一种病死率很高的严重疾病,它以肺血管阻力不断升高为主要特征并最终引起右心衰竭和死亡。近年来,在肺动脉高压的诊断、治疗的研究中取得了长足的进步。规范的诊断和治疗将有助于改善肺动脉高压患者的生活质量和预后。  相似文献   

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肺动脉高压的药物治疗进展   总被引:2,自引:0,他引:2  
肺动脉高压是以小肺动脉的血管增生和重构为主要特征,导致肺血管阻力进行性增加,最终引起右心衰和死亡的一种疾病。以前治疗肺动脉高压的药物包括钙离子拮抗剂、抗凝剂和对严重患者持续静脉注射依前列醇。最近,前列环素类似物treprostinil、贝拉普罗、伊洛前列素和内皮素受体拮抗剂波生坦已用于临床试验。因此,在过去的20年间,对肺动脉高压治疗取得的进展有助于延长病人的生存时间和提高他们的生活质量。  相似文献   

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