The Role of Echocardiography in the Evaluation of Pulmonary Arterial Hypertension

The evaluation of pulmonary arterial hypertension (PAH) requires a multimodality approach that combines invasive and noninvasive imaging studies to ensure accurate diagnosis and classification. Given the complexity of the hemodynamic relationships between the left heart, pulmonary circulation, and right heart, the diagnosis of PAH is often a challenging task. Right heart catheterization is the gold standard for diagnosis, providing the hemodynamic information that defines the disease. Nonetheless, echocardiography continues to be a valuable tool in the approach to the patient with suspected PAH. Echocardiographic assessment generates a wealth of information about the response of the right heart to elevated pulmonary pressures and provides essential diagnostic and prognostic data to the clinician. Numerous measurements can be used to identify alterations in right heart morphology, pressure, and function; although each variable in isolation may have little utility, meaningful information is revealed when multiple parameters are considered together. In this article, we will review the echocardiographic measurements employed in assessment of the right heart and seek to clarify the role of echocardiography in the diagnostic workup of PAH.     

Novel Treatment Pathways in Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a severe and progressive vascular disease characterized by pulmonary vascular remodeling, proliferation, and inflammation. Despite the availability of effective treatments, PAH may culminate in right ventricular failure and death. Currently approved medications act through three well-characterized pathways: the nitric oxide, endothelin, and prostacyclin pathways. Ongoing research efforts continue to expand our understanding of the molecular pathogenesis of this complex and multifactorial disease. Based on recent discoveries in the pathobiology of PAH, several new treatments are being developed and tested with the goal of modifying the disease process and ultimately improving the long-term prognosis.     

肺动脉高压的治疗进展

肺动脉高压是以肺小动脉的血管痉挛、内膜增生和重构为主要特征的一种疾病。它是一进展性疾病,生存期为2.8年,早期诊断和治疗干预是改善患者预后的重要措施,现将新近的治疗进展综述。     

肺动脉高压患者的预后评估

肺动脉高压是发病率较低、预后较差的肺血管疾病.近10余年来,随着靶向治疗药物的应用,肺动脉高压的生存率较前有明显的改善;多个登记注册研究和一些临床研究结果的发布,促使对肺动脉高压预后相关的因素有了更加全面的认识.现简要综述各预测因素在肺动脉高压预后评估中的作用,以及肺动脉高压预后模型的建立.     

肺动脉高压联合药物治疗的研究进展

肺动脉高压是一类以肺血管阻力进行性增高为主要特征,最终导致右心衰竭、功能严重受限、死亡的疾病。近年来肺动脉高压在单一药物治疗上取得一定疗效,而联合应用不同的药物取得最佳临床疗效是治疗肺动脉高压的新观点,可以发挥药物间的协同效应,降低单个药物的不良反应。现综述联合药物治疗肺动脉高压的临床应用进展。     

超声心动图评价肺动脉高压患者右室功能的研究进展

肺动脉高压的发展会导致患者右室功能减低,对肺动脉高压患者右室功能的评价对其预后具有十分重要的临床意义。随着超声技术的不断发展,特别是新型超声技术的出现能敏感、准确地定量反映肺动脉高压患者的右室功能。现就目前超声心动图技术在评价肺动脉高压患者右室功能中的应用做一综述。     

肺动脉高压的诊断和治疗进展

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。     

肺动脉高压患者右室功能的多普勒超声评价与进展

肺动脉高压会导致患者右室功能减低,早诊断、早治疗肺动脉高压能明显改善患者的预后。近年来应用多普勒超声技术对肺动脉高压患者右室功能研究的新进展层出不穷,如双多普勒同步技术、Tei指数及三尖瓣环收缩期位移等,均为临床提供了全新的评价肺动脉高压及右室功能的新手段及新参数。     

Idiopathic Pulmonary Arterial Hypertension Misdiagnosed as Asthma

Idiopathic pulmonary arterial hypertension (IPAH) is a rare disorder that is progressive and often leads to right heart failure if left untreated. Because of the vague nature of symptoms at presentation, IPAH may take several months to diagnose. The most common presenting complaint in patients with IPAH is dyspnea with exertion, which is also commonly seen with asthma. This report describes an adult female with refractory exertional dyspnea who was diagnosed with asthma 2 years earlier but was ultimately diagnosed with IPAH. Exclusion of other etiologies is a necessity for refractory dyspnea in the setting of asthma.     

Pericardial fat mimicking pericardial effusion on two-dimensional echocardiography

A 78-year-old overweight woman with diabetes mellitus, bronchial asthma, and Sheehan's syndrome on chronic steroid therapy presented with mild short-lived hematemesis, significant hypotension disproportionate to the degree of bleeding and radiographic evidence of cardiomegaly. Endoscopy showed duodenal ulcer. During evaluation of the unexplained brief hypotension and cardiomegaly, 2D-echocardiogram demonstrated anterior and posterior echo-free spaces consistent with large pericardial effusion (PE). However, subsequent elective surgical pericardiotomy unexpectedly revealed large amounts of pericardial fat. Pericardial fat was also noted on magnetic resonance imaging of the chest. Our case illustrates a potential pitfall of 2D-echocardiography in the diagnosis of PE.     

肺动脉高压的药物治疗进展

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。     

Evaluation and Management of Pulmonary Arterial Hypertension in Congenital Heart Disease

Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect. Pulmonary arterial hypertension (PAH) has a multifactorial etiology depending on the size and nature of the cardiac defect as well as environmental factors. Although progress has been made in disease-targeting therapy using pulmonary vasodilators to treat Eisenmenger syndrome, important gaps still exist in the evaluation and management of adult patients with CHD-associated PAH (PAH-CHD) who have systemic-to-pulmonary shunts. The choice of interventional, medical, or both types of therapy is an ongoing dilemma that requires further data. This review focuses on the evaluation and management of PAH-CHD in the contemporary era.     

环磷酰胺在野百合碱诱导大鼠肺动脉高压模型中的作用

目的 应用环磷酰胺(CTX)对野百合碱(MCT)诱导的大鼠肺动脉高压(PAH)模型进行干预,观察CTX在该模型中的疗效,并对其机制进行初步探讨.方法 建立MCT诱导的PAH模型,用CTX对模型进行预防干预和治疗干预,分别在建模2、4周,通过右心导管技术测量大鼠肺动脉平均压( mPAP),与模型组比较,判断干预是否有效;...     

进行心包穿刺和引流的心包积液患者的临床特点分析

目的：探讨需要进行心包穿刺和引流的心包积液患者的临床特征,以指导该类患者的诊断和治疗。方法：总结并分析202例需要进行心包穿刺和引流的心包积液患者的临床特征、病因及心包积液性质。结果：除11例急性心肌梗死患者来不及进行心包穿刺外,191例均成功进行心包穿刺和引流,漏出液12例,渗出液179例。渗出液中,肿瘤性77例,结核性62例,医源性18例,急性心肌梗死15例,结缔组织疾病10例。77例肿瘤性心包积液中,肺癌42例,乳腺癌7例,原发灶暂时不明确的转移性腺癌6例,淋巴瘤7例,肝癌4例,纵隔瘤3例,胃癌、膀胱癌、直肠癌、心包肉瘤、横纹肌肉瘤、黑色素瘤、恶性胸腺瘤和心脏血管瘤各1例。18例医源性心包积液中,心律失常导管消融者10例,经皮冠状动脉介入治疗者6例。心律失常导管消融并发医源性心包积液的比例女性高于男性、房颤患者高于非房颤患者。9例导管消融者行心包穿刺和引流即可,余9例医源性心包积液还需外科修补。15例急性心肌梗死者在住院期间均死亡。结论：需要进行心包穿刺和引流的心包积液多为渗出液及血性,肿瘤和结核为主要原因,注意识别和鉴别医源性心包积液和急性心肌梗死,积极心包穿刺和引流是重要的治疗手段。     

New Insights in the Treatment Strategy for Pulmonary Arterial Hypertension

Introduction Recent advances in our understanding of the pathophysiological and molecular mechanisms involved in pulmonary arterial hypertension have led to the development of novel and rational pharmacological therapies. In addition to conventional therapy (i.e., supplemental oxygen and calcium channel blockers), prostacyclin or endothelin receptor antagonists have been recommended as a first-line therapy for pulmonary arterial hypertension. However, these treatments have potential limitations with regard to their long-term efficacy and improvement in survival. Furthermore, intravenous prostacyclin (epoprostenol) therapy, which is recommended by most experts for patients with New York Heart Association (NYHA) functional class IV, is complicated, uncomfortable for patients, and expensive because of the cumbersome administration system. Considering these circumstances, it is necessary to develop additional novel therapeutic approaches that target the various components of this multifactorial disease. Case report In this short review, we present an overview of the current treatment options for pulmonary arterial hypertension and describe a case report with primary pulmonary hypertension. A male patient with NYHA functional class IV and showing no response to calcium channel blockers and prostacyclin exhibited significantly improved exercise tolerance and hemodynamics and long-term survival for more than 2.5 years after receiving an oral combination therapy of a phosphodiesterase type 5 inhibitor (sildenafil), phosphodiesterase type 3 inhibitor (pimobendan), and nicorandil. Future perspective We also discuss the background and plausible potential mechanisms involved in this case, as well as future perspectives in the treatment of pulmonary arterial hypertension.