肺动脉高压患者血浆肾上腺髓质素水平与肺动脉压力的关系

目的：探讨肺动脉高压（pulmonary hypertension,PH)患者血浆肾上腺髓质素（a-drenomedullin,AM)的浓度与肺动脉压力的关系。方法：选择正常肺动脉压力患者和轻度、中度、重度肺动脉高压患者各10例，在术中分别从肺动脉和肺静脉中抽血用放射免疫法测定AM含量。结果：肺动脉血浆AM浓度与肺动脉压力呈显著正相关；肺静脉血浆AM浓度低于肺动脉AM浓度，且其浓度差值与肺动脉压力呈显著正相关。结论：AM参与了肺动脉高压的病理生理过程，并在肺内部分代谢，对肺血管张力的调节起重要作用。     

Tratamiento médico y quirúrgico de la hipertensión pulmonar tromboembólica crónica: experiencia en un único centro

IntroductionPulmonary endarterectomy (PE) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to analyze our experience in the medical and surgical management of CTEPH.MethodsWe included 80 patients diagnosed with CTEPH between January 2000 and July 2012. Thirty two patients underwent PE and 48 received medical treatment (MT). We analyzed functional class (FC), six-minute walking distance (6MWD) and pulmonary hemodynamics. Mortality in both groups and periods were analyzed.ResultsPatients who underwent PE were younger, mostly men, and had longer 6MWD. No differences were observed in pulmonary hemodynamics or FC at diagnosis. One year after treatment, all PE patients versus 41% in MT group were at FC I-II. At follow-up, the PE group showed greater increase in 6MWD, and greater reduction in mean pulmonary arterial pressure and pulmonary vascular resistance than the MT group (P < .05). Overall survival in the MT group at 1 and 5 years was 83% and 69%, respectively. Conditional survival in patients alive 100 days post-PE at 1 and 5 years was 95% and 88%, respectively. Surgical mortality in operated patients in the first period (2000-2006) was 31,3%, and 6,3% in the second (2007-2012).ConclusionsPE provides good clinical results, and improves pulmonary hemodynamics in patients who successfully overcome the immediate postoperative period. After a learning period, the current operatory mortality in our center is similar to international standards.     

Update on chronic thromboembolic pulmonary hypertension

Chronic, unresolved thromboemboli are an important cause of pulmonary hypertension (PH) with specific treatment strategies differing from other types of PH. Chronic thromboembolic pulmonary hypertension (CTEPH) is classified as group 4 PH by the World Health Organization. It is a rare, but underdiagnosed, complication of acute pulmonary embolism that does not resolve and results in occlusion of large pulmonary arteries with a fibro-thrombotic material. The etiology of CTEPH remains uncertain, and it is unknown why certain patients with acute pulmonary embolism develop this disorder. The evaluation for CTEPH is an important part of the evaluation for PH in general, and it is crucial not to overlook this diagnosis, as it is the only form of PH that is potentially curable. Patients diagnosed with CTEPH should be referred to an expert center for consideration of pulmonary endarterectomy, and surgical removal of the chronic thromboembolic material. Not all patients with CTEPH are surgical candidates, however, and there are emerging treatments—medical therapy and balloon pulmonary angioplasty—that have shown benefit in this patient population. Without treatment, CTEPH can lead to progressive pulmonary vascular obstruction, right heart failure, and death. Thus, it is important for clinicians to recognize this subtype of PH. In this review, we provide an overview of current understanding of the pathogenesis of CTEPH and highlight recommendations and recent advances in the evaluation and treatment of CTEPH.     

Pulmonary hypertension in systemic lupus erythematosus

Systemic lupus erythematosus is associated with several forms of pulmonary hypertension. It can cause pulmonary hypertension through pulmonary thromboembolic disease, left heart disease and lung disease as well as causing an isolated pulmonary vasculopathy called pulmonary arterial hypertension. The true prevalence of pulmonary arterial hypertension in patients with lupus is not known but probably is no more than 1%. Currently, treatment for lupus-associated pulmonary arterial hypertension is with pulmonary vasodilators including phosphodiesterase-5 inhibitors, endothelin receptor antagonists and prostacyclin analogues, as it is for other causes of pulmonary arterial hypertension. Case series suggest there may be a special role for immunosuppression in this rare group of patients. We present two brief case histories and summarise our experience over 15 years. Prognosis is better in lupus-associated pulmonary arterial hypertension than in systemic sclerosis-associated pulmonary arterial hypertension, but unfortunately it remains a fatal condition in most patients.     

提高对肺动脉高压的诊断意识

肺动脉高压（PAH）是一种预后极差的进展性疾病，由于病因多而复杂，症状体征不典型，常规检查敏感性低，PAH常常被延误诊断，而且当患者确立PAH诊断时，多数已经处于NYHA功能分级Ⅲ-Ⅳ级。最重要提高临床诊断水平的手段是提高对PAH的认识和诊断意识。临床医生只有全面理解WHO肺高压分类标准，掌握并合理使用各种诊断方法和措施，仔细筛查，确诊PAH并明确病因，评估病情，才能尽早采取具有循证医学证据的规范化治疗，提高患者的生活质量和预后。     

Tromboendarterectomía pulmonar en 106 pacientes con hipertensión pulmonar tromboembólica crónica

IntroductionPulmonary thromboendarterectomy is the treatment of choice in chronic thromboembolic pulmonary hypertension. We report our experience with this technique.MethodsBetween February 1996 and June 2014, we performed 106 pulmonary thromboendarterectomies. Patient population, morbidity and mortality and the long-term results of this technique (survival, functional improvement and resolution of pulmonary hypertension) are described.ResultsSubjects’ mean age was 53 ± 14 years. A total of 89% were WHO functional class III-IV, presurgery mean pulmonary pressure was 49 ± 13 mmHg and mean pulmonary vascular resistance was 831 ± 364 dynes.s.cm⁻⁵. In-hospital mortality was 6.6%. The most important post-operative morbidity was reperfusion pulmonary injury, in 20% of patients; this was an independent risk factor (p = 0.015) for hospital mortality. With a 31-month median follow-up (interquartile range: 50), 3- and 5-year survival was 90 and 84%. At 1 year, 91% were WHO functional class I-II; mean pulmonary pressure (27 ± 11 mmHg) and pulmonary vascular resistance (275 ± 218 dynes.s.cm⁻⁵) were significantly lower (p < 0.05) than before the intervention. Although residual pulmonary hypertension was detected in 14 patients, their survival at 3 and 5 years was 91 and 73%, respectively.ConclusionsPulmonary thromboendarterectomy offers excellent results in chronic thromboembolic pulmonary hypertension. Long-term survival is good, functional capacity improves, and pulmonary hypertension is resolved in most patients.     

吡那地尔防治大鼠低氧性肺动脉高压肺血管重建的实验研究

目的研究钾通道开放剂吡那地尔对低氧性肺动脉高压（ＨＰＨ）及其肺血管重建的影响。方法Ｗｉｓｔｅｒ大鼠４６只,随机分为３组：对照组１５只;低氧组１６只;治疗组（低氧＋吡那地尔）１５只。低氧组及治疗组建立低氧性肺动脉高压动物模型,治疗组于每天缺氧前腹腔注射吡那地尔３ｍｇ／ｋｇ。 ４周后测定各组平均肺动脉压（ｍＰＡＰ）、右心室（ＲＶ）／左心室＋室间隔（ＬＶ＋ Ｓ）比值和肺小动脉病理及其形态计量学。结果（１）低氧组ｍＰＡＰ、ＲＶ／（ＬＶ＋Ｓ）分别为（２８．４ ± ２．８）ｍｍＨｇ和（０．３０±０．０３）,明显高于对照组（１６．２±１．８）ｍｍ Ｈｇ和（０．２２±０．０３）（Ｐ＜０．０１）,管壁厚度与血管外径比值（ＭＴ％）、管壁面积与血管总面积比值（ＭＡ％）分别为（２５．７±２．６）％和（７５．３±５．６）％,亦明显高于对照组（１８．５±２．９）％和（５９．９±６．６）％（Ｐ＜０．０１）,管腔面积与血管总面积比值（ＶＡ％）为（２４．３±５．６）％,明显低于对照组（４０．７±８．１）％（Ｐ＜０．０１）。提示慢性缺氧导致大鼠发生明显肺动脉高压及右心室肥厚和肺小动脉管壁增厚、管腔狭窄等肺血管重建等改变。（２）治疗组ｍＰＡＰ、ＲＶ／     

Pulmonary ossification syndrome in a patient with chronic thromboembolic pulmonary hypertension

First described more than 150 years ago, diffuse pulmonary ossification is a rare disease that can be idiopathic or secondary to an underlying chronic disorder. Commonly under-recognized and challenging to diagnose, clinical, radiographic and functional tests are often necessary. Given the increasing prevalence of chronic lung disease in the aging population, it is likely the pulmonary ossification will be encountered in clinics more often. This article describes the diagnosis of the condition in a 51-year-old man.Pulmonary ossification syndrome is a rare disease characterized by bone tissue formation in the lung parenchyma with or without bone marrow elements. This disorder, often under-recognized, can be idiopathic or secondary to an underlying chronic disorder such as chronic thromboembolic pulmonary hypertension. The pathogenesis involves tissue injury and an alkaline environment in which the precipitation of calcium salts enables alkaline phosphatase activity. This activates profibrogenic cytokines that convert fibroblasts into osteoblasts. Diagnosis can prove challenging and is based on clinical, radiographic and functional tests, and by tissue biopsy. Achieving an accurate and timely diagnosis is essential to avoid erroneous treatments due to misdiagnosis and to expand knowledge of its progression, prognosis and treatment. As the population and prevalence of chronic lung disease increases, it is likely that physicians will encounter more cases of pulmonary ossification.     

合并/不合并肺动脉高压的肺纤维化合并肺气肿临床特点分析

目的:探讨合并/不合并肺动脉高压(PH)的肺纤维化合并肺气肿(CPFE)临床特点,寻找易合并PH的CPFE危险因素。方法:本研究为病例对照研究,采用非随机抽样的方法收集2020年1月至2021年12月河北医科大学第二医院呼吸与危重症医学科收治的肺纤维化合并肺气肿(CPFE)患者129例。依据是否合并PH分为合并PH的C...     

维生素D与动脉性肺动脉高压

动脉性肺动脉高压(PAH)是一种以肺动脉压升高为特征且预后极差的疾病。目前具有适应证的药物可改善肺动脉高压患者预后,但无法治愈,探讨其发病机制、寻找新的治疗靶点是当前PAH研究热点。维生素D是一种类固醇激素,在体内活性形式为1,25-二羟维生素D3。近年来维生素D缺乏已被证明与多种自身免疫病的发病有关。维生素D缺乏可影...     

肺动脉血栓内膜剥脱术治疗慢性栓塞性肺动脉高压

目的报告肺动脉血栓内膜剥脱术治疗慢性栓塞性肺动脉高压的经验。方法回顾性总结了连续8例肺动脉血栓内膜剥脱术的手术要点、围术期处理以及近中期结果。结果8例均存活。肺动脉收缩压由术前的(101±24)mmHg降至术后的(39±15)mmHg、动脉血氧分压由(59±11)mmHg升至(92±7)mmHg。动脉血氧饱和度由(0.89±0.06)升至(0.98±0.01),均有显著改善。随访显示患者心功能为纽约心功能协会分级标准(NYHA)Ⅰ级(3例)或NYHAⅡ级(5例),生活质量明显改善。结论肺动脉血栓内膜剥脱术是治疗慢性栓塞性肺动脉高压的有效手段。     

肺静脉闭塞病的研究进展

肺动脉高压（pulmonary arterial hypertension,PAH）是由不同病因导致的、以肺动脉压力和肺血管阻力升高为特点的一组综合征。肺静脉闭塞病（Dulmonary venoocclusive disease,PVOD）是公认的PAH比较少见原因之一。