核素肺显像在慢性血栓栓塞性肺动脉高压诊断和定量评价的应用

目的评价放射性核素肺灌注／通气（Q／V）显像在慢性血栓栓塞性肺动脉高压（CTEPH）诊断和定量评价中的价值。方法78例肺动脉高压住院患者，经超声心动图排除了瓣膜性心脏病和先天性心脏病引起的肺动脉高压，且既往无急性肺栓塞病史。所有患者均接受Q／V显像，并以肺动脉造影确诊，分别计算Q／V显像对CTEPH的诊断敏感性、特异性和准确性。对肺动脉造影确诊为CTEPH患者的肺灌注显像进行定量分析，计算全肺灌注缺损百分数（PPDs％），并对PPDs％分别与平均肺动脉压（mPAP）、肺动脉收缩压（SPAP）进行相关性分析，并进行统计学检验。结果以高度可能分级作为CTEPH的诊断标准，Q／V显像的诊断敏感性、特异性和准确率分别为96．0％，81．1％和86．9％；而如果将高度和中度可能分级均作为CTEPH的判断依据，则分别为100％，69．8％和79．5％。PPDs％与mPAP和SPAP均具有直线相关性，PPDs％与mPAP，r=0．538，P〈0．01；PPDs％与SPAP，r=0．456，P〈0．05。结论Q／V显像是CTEPH诊断和定量评价的有价值的无创性影像学方法。     

慢性血栓栓塞性肺动脉高压的误诊分析

慢性血栓栓塞性肺动脉高压(CTEPH)是指肺动脉及其主要分支由于血栓栓塞导致的血流受阻,长期不能缓解或进行性加重,导致肺动脉高压。CTEPH的误诊率很高,笔者对近3年所收治的7例CHEPH患者进行误诊分析,以提高对CTEPH的认识。1对象与方法近3年我院心内科确诊的CTEPH患者共7例,男2例,女5例。年龄40~66(52±9.52)岁。均经螺旋CT肺血管造影(CTPA)和肺通气灌注扫描证实的至少有1个肺段以上的肺栓塞。患者均经过超声多普勒检测肺动脉收缩压>30mmHg(1mmHg=0·133kPa)以上,均排除其他非血栓栓塞性肺动脉高压。2结果症状体征:主要症状有…     

慢性血栓栓塞性肺动脉高压

慢性血栓栓塞性肺动脉高压（chronic thromboembolic pul-monary hypertension, CTEPH ）是急性肺栓塞或肺动脉原位血栓形成的长期后果,由于种种原因血栓未溶解而持续存在,通过机化、纤维化形成^[1]。国外文献报道急性肺栓塞发作后存活的患者中0．1％～0．5％发展为CTEPH,该疾病发生率虽然很低,但预后差,若无有效的干预,大多数CTEPH患者多逐渐死于难治性右心衰竭。     

慢性血栓栓塞性肺动脉高压发病机制新进展:从基础到临床

慢性血栓栓塞性肺动脉高压(CTEPH)属于肺动脉高压分型的第四大类,是一种自然预后极差的慢性进展性疾病.CTEPH通常被认为是急性肺血栓栓塞症(APTE)的一种长期并发症,但研究表明部分CTEPH患者无明确的APTE既往史.因此,阐明CTEPH的发病机制,对于认识CTEPH的发生发展过程、指导疾病诊治具有重要意义.现围...     

慢性血栓栓塞性肺动脉高压诊疗进展

慢性血栓栓塞性肺动脉高压（chronic thromboembolic pulmonary hypertension,CTEPH）由肺动脉血栓栓塞后不完全再通和血管重构而形成,在未接受治疗情况下中位生存期仅不到3年,预后差且极易漏诊或误诊。本文就CTEPH的最新诊疗进展作以综述。     

130例闭塞性肺动脉高压患者自然病程的分析

目的：分析闭塞性肺动脉高压患者的自然病程。方法：对130例闭塞性肺动脉高压患者以通讯或门诊方式进行随访,将生存结果与临床心电图,X线胸片,超声心动图,血液动力学资料进行相关分析。不能解释的肺动脉高压组（UPH组）与慢性栓塞性肺动脉高压组（CTEPH组）两组对照分析。结果：本组闭塞性肺动脉高压患者平均随访时间5．86±4．63年,其生存函数曲线显示2年、3年、5年生存率分别为79.0％,73.0％,50.0％。UPH组与CTEPH组生存率比较,后者较好。影响生存率的因素有心功能,肺动脉压力,心输出量,心脏指数,心胸比率,动脉血氧分压等。结论：闭塞性肺动脉高压的预后不乐观,尤以不能解释的肺动脉高压更严重。降低肺血管压力,改善心功能是提高闭塞性肺动脉高压生存率的重要环节。     

肺阻抗血流图对慢性肺原性心脏病的诊断价值

目的：探讨肺阻抗血流图对慢性肺原性心脏病的诊断价值。方法：18例肺心病患者及48例临床疑诊肺心病患者在临床缓解期应用ZK－Ⅵ多功能阻抗测试仪检测。检测结果应用武汉同济医科大学附属同济医院鲁昌珍教授诊断系统进行分析。结果：18例肺心病患者的肺阻抗血流图提示肺心病16例（88．9％），提示肺动脉高压的为18例（100％）；48例疑诊肺心病患者的肺阻抗血流图提示肺心病的为27例（56．3％），提示肺动脉高压的为29例（64．6％），提示负荷后肺动脉高压的为42例（87．5％）。结论：肺阻抗血流图对代偿期肺心病的诊断符合率及失代偿期肺心病的诊断符合率均与文献报道的50％－70％和83％－93．3％相近似。它不但能早期诊断肺心病，也可早期发现肺动脉高压及隐性肺动脉高压。     

慢性肺血栓栓塞症与慢性血栓栓塞性肺动脉高压

慢性肺血栓栓塞症（简称慢性肺栓塞）与慢性血栓栓塞性肺动脉高压（CTEPH）被认为是急性肺栓塞的远期并 发症。近年来，随着对慢性血栓认识的深入以及介入、手术等治疗技术的发展，慢性肺栓塞和CTEPH患者的诊治进 展迅速，患者预后显著改善。但另一方面，慢性肺栓塞和CTEPH的诊治相对复杂，而国内医师对其认识相对不足，临 床不规范诊治的情况并不少见。文章重点阐述慢性肺栓塞和CTEPH的基本概念及诊治思路。     

内皮素通路在靶向治疗慢性血栓栓塞性肺动脉高压中的作用及机制

正慢性血栓栓塞性肺动脉高压(chronic thrombembolic pulmonary hypertension,CTEPH)是肺动脉高压的一种疾病亚型,是指由来自静脉系统或右心的血栓,引起一次或反复发生的肺血栓栓塞导致血栓机化、肺动脉管腔狭窄甚至闭塞、肺血管重塑,从而引起肺动脉压力进行性升高,进一步发展可出现呼吸功能不全和右心衰竭并最终死亡的疾病。2015年欧洲心脏病学会/欧洲呼吸病学会联合颁布的《肺动脉高压诊断和     

慢性血栓栓塞性肺高压的治疗现状

慢性血栓栓塞性肺高压（chronic thromboembolic puhnonary hypertension, CTEPH）是指由于机化血栓阻塞近端或远端肺动脉后，造成以肺动脉压力增高为特点的一类疾病。过去曾认为CTEPH是非常罕见的疾病，目前国际上虽仍缺乏确切可靠的流行病学资料，但实际上其发病率被远远低估。CTEPH已成为引起严重肺高压的重要病因之一。     

Descriptive patterns of severe chronic pulmonary hypertension by chest radiography

BACKGROUND: To find chest roentgenographic (CXR) features to help differentiate two representative diseases with severe chronic pulmonary hypertension (PH). STUDY SUBJECTS: Thirty-six consecutive patients with chronic thromboembolic PH (CTEPH), 38 with primary PH (PPH), and 37 with left heart disease and PH. METHODS: CXRs were reviewed about 6 features (left 2nd arc protrusion, right descending pulmonary artery diameter (rPAD), cardiothoracic ratio (CTR), right 2nd arc width, avascular area and pleural abnormality). Hemodynamic data and the degree of tricuspid regurgitation (TR) on echocardiography were compared with CXR findings. RESULTS: The diagnostic pattern of CTEPH was the presence of one of two findings, an avascular area or marked rPAD (>20mm) together with pleuritic change. The diagnostic pattern of PPH was one of the two features; without pleuritic abnormality, marked left 2nd arc protrusion (>10mm) or moderate left 2nd arc protrusion (5-10mm) with marked rPAD (<20mm). The sensitivity for the diagnosis of CTEPH among the three diseases was 78% and specificity was 92%. The sensitivity for the diagnosis of PPH was 45% and specificity was 88%. CTR and right 2nd arc width were related to the degree of TR in CTEPH and PPH. CONCLUSIONS: Characteristic roentgenographic findings can help differentiate two frequent diseases associated with chronic pulmonary hypertension and reflect the severity of disease.     

Pulmonary hemodynamic and gas exchange effects of various oxygen concentrations in patients with severe pulmonary hypertension primarily affecting the pulmonary vasculature]

The aim of this study was to evaluate pulmonary hemodynamic and gas exchange response to oxygen inhalation in patients with severe pulmonary hypertension primarily affecting the pulmonary vasculature. This study included 7 patients with primary pulmonary hypertension (PPH), 11 with pulmonary hypertension related to collagen vascular diseases (CoPH), and 18 with chronic thromboembolic pulmonary hypertension (CTEPH). All patients had mean pulmonary arterial pressure (PPAm) of greater than 25 mm Hg. We divided the patients into two groups: a PPH + CoPH group comprising the 7 PPH and 11 CoPH patients, and the CTEPH group. We measured cardiopulmonary variables after 10 min inhalation of various oxygen concentrations (FiO2 0.24, 0.28, 0.4, 1.0). In the PPH + CoPH group, PPAm significantly decreased after the inhalation of oxygen concentrations of 40% or more. This was associated with a significant reduction in pulmonary arteriolar resistance (PAR), and suggested active pulmonary vasodilation was caused by oxygen inhalation. In the CTEPH group, on the other hand, PPAm significantly decreased after the inhalation of oxygen concentrations of 28% or more, apparently in association with a significant fall of cardiac output. However, PAR was unchanged regardless of the inspired oxygen concentration, indicating an absence of pulmonary vasodilation in the CTEPH group. When breathing room air, 7 patients in the PPH + CoPH group (38.9%) and 10 in the CTEPH group (55.6%) demonstrated mixed venous oxygen tension (PvO2) values of less than 35 Torr. Extra attention should be paid to PvO2 when administering oxygen therapy to patients with severe pulmonary hypertension.     

Surgical Treatment for Chronic Thromboembolic Pulmonary Hypertension Under Profound Hypothermia and Circulatory Arrest in 24 Patients

Abstract Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious disease that induces hypoxemia and pulmonary hypertension, eventually leading to respiratory failure and right heart failure. We evaluated the results of surgical treatment in patients undergoing circulatory arrest under profound hypothermia. Methods: Between February 1995 and June 1999, 24 cases of CTEPH were surgically treated. The age of patients (11 males and 13 females) ranged from 21 to 71 years (mean 49 ± 15 years). Because of hypoxemia, severe pulmonary hypertension (mean pulmonary artery pressure 45 & 7 mmHg 1, and low cardiac output, the functional class of these patients was New York Heart Association (NYHA) 111 or IV. Following a median sternotomy, profound hypothermia was induced using cardiopulmonary bypass, and pulmonary thromboendarterectomy in the bilateral pulmonary arteries was performed under intermittent circulatory arrest. Surgery was performed emergently in four patients. Results: Of these 24 patients, 2 of 20 patients who underwent elective surgery and 3 of 4 patients who underwent emergent surgery died in the hospital. Symptoms of CTEPH markedly improved in 18 patients who survived the surgery. Pulmonary arterial pressure was decreased to 16 ± 6 mmHg, and cardiac output was increased. Conclusions: When CTEPH is resistant to medical treatment, surgical treatment is useful. When surgical indications are carefully selected, pulmonary thromboendarterectomy using intermittent circulatory arrest under profound hypothermia is quite effective for treating CTEPH.     

Acute effects of the combination of sildenafil and inhaled treprostinil on haemodynamics and gas exchange in pulmonary hypertension

BACKGROUND: Inhaled treprostinil was recently developed for the treatment of pulmonary arterial hypertension (PAH). We investigated the safety and acute haemodynamic effects of the combination oral sildenafil and inhaled treprostinil in an open label study in patients with precapillary pulmonary hypertension. METHODS AND PATIENTS: Inhaled nitric oxide (20ppm; n=50), sildenafil (50mg; n=50) and inhaled treprostinil (15mug; n=25 or 30mug; n=25) were applied in subsequent order during right heart catheter investigation to consecutive patients with pulmonary arterial hypertension (PAH; n=28), non-operable chronic thromboembolic pulmonary hypertension (CTEPH; n=17) and pulmonary fibrosis associated pulmonary hypertension (n=5). RESULTS: Inhaled nitric oxide reduced pulmonary vascular resistance (PVR) to 87.3+/-5.1% of baseline values, reduced mean pulmonary arterial pressure (PAP) to 89.7+/-3.5% and increased cardiac output (CO) to 102.4+/-2.9%. Sildenafil reduced PVR to 80.1+/-5.0%, mPAP to 86.5+/-2.9% and increased CO to 103.8+/-3.2%. Treprostinil, inhaled 1h after sildenafil, reduced PVR to 66.3+/-3.8%, mPAP to 77.8+/-3.3%, and increased CO to 107.1+/-3.3% (mean+/-95% confidence interval). Subgroup analysis showed similar acute haemodynamic effects in PAH and CTEPH patients. Ventilation/perfusion distribution measurement in six patients with pre-existing gas exchange limitations was not changed by sildenafil and treprostinil. Relevant side effects were not observed. CONCLUSION: The combination of sildenafil and inhaled treprostinil was well tolerated and induced additive, pulmonary selective vasodilatation in pulmonary hypertension patients. This could be of relevance also for long-term treatment of PAH and CTEPH patients.     

Prostacyclin therapy before pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension

OBJECTIVES: The continuous IV administration of prostacyclin improves pulmonary hemodynamics and prognosis in patients with primary pulmonary hypertension. We investigated whether the administration of prostacyclin therapy to patients before they undergo pulmonary thromboendarterectomy ameliorates pulmonary hypertension in patients with the most severe form of chronic thromboembolic pulmonary hypertension (CTEPH). METHODS: Of the 33 patients with CTEPH who were candidates for pulmonary thromboendarterectomy, 12 patients with severe pulmonary hypertension (pulmonary vascular resistance, > 1,200 dyne. s. cm(-5)) received IV prostacyclin prior to undergoing pulmonary thromboendarterectomy. Right heart catheterization and plasma brain natriuretic peptide (BNP) measurements were repeated at baseline, immediately before surgery, and 1 month after surgery. RESULTS: During a mean (+/- SEM) follow-up period of 46 +/- 12 days, the IV administration of prostacyclin resulted in a 28% decrease in pulmonary vascular resistance (1,510 +/- 53 to 1,088 +/- 58 dyne. s. cm(-5); p < 0.001) before surgery. Prostacyclin therapy markedly decreased plasma BNP level (547 +/- 112 to 188 +/- 30 pg/mL; p < 0.01), suggesting improvement in right heart failure. Pulmonary thromboendarterectomy caused a further reduction of pulmonary vascular resistance (302 +/- 47 dyne. s. cm(-5)) and plasma BNP levels (60 +/- 11 pg/mL) compared to each preoperative value (p < 0.05). Operative mortality rates were relatively low (8.3%) in patients with the most severe form of CTEPH. CONCLUSION: The IV administration of prostacyclin caused beneficial hemodynamic effects in patients with severe CTEPH and may serve as pretreatment for patients undergoing pulmonary thromboendarterectomy.     

Occlusion pressure analysis role in partitioning of pulmonary vascular resistance in CTEPH

Flow-directed pulmonary artery occlusion is posited to enable partitioning of vascular resistance into small and large vessels. As such it may have a role in assessment for pulmonary endarterectomy. To test if the occlusion technique distinguished small from large vessel disease we studied 59 subjects with chronic thromboembolic pulmonary hypertension (CTEPH), idiopathic pulmonary arterial hypertension (IPAH), and connective tissue disease (CTD)-associated PAH. At right heart catheterisation, occlusion pressures were recorded. With fitting of the pressure decay curve, pulmonary vascular resistance was partitioned into downstream (small vessels) and upstream (large vessels, Rup). 47 patients completed the study; 14 operable CTEPH, 15 inoperable CTEPH, 13 idiopathic or CTD-PAH and five post-operative CTEPH. There was a significant difference in mean Rup in the proximal operable CTEPH group 87.3 (95% CI 84.1-90.5); inoperable CTEPH mean 75.8 (95% CI 66.76-84.73), p=0.048; and IPAH/CTD, mean 77.1 (95% CI 71.86-82.33), p=0.003. Receiver operating characteristic curves to distinguish operable from inoperable CTEPH demonstrated an area under the curve of 0.75, p=0.0001. A cut-off of 79.3 gave 100% sensitivity (95% CI 73.5-100%) but 57.1% specificity (95% CI 28.9-82.3%). In a subgroup analysis of multiple lobar sampling there was demonstrable heterogeneity. Rup is significantly increased in operable proximal CTEPH compared with non-operable distal CTEPH and IPAH/CTD-PAH. Rup variability in patients with CTEPH and PAH is suggestive of pathophysiological heterogeneity.     

Survival in pulmonary hypertension in Spain: insights from the Spanish registry

A pulmonary hypertension (PH) registry (Spanish Registry of Pulmonary Arterial Hypertension) was undertaken to analyse prevalence, incidence and survival of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) in Spain, and to assess the applicability of recent survival prediction equations. Voluntary reporting of previously diagnosed and incident PAH or CTEPH cases (July 2007-June 2008) was performed. Demographic, functional and haemodynamic variables were evaluated. 866 patients with PAH and 162 with CTEPH were included. PAH associated with toxic oil syndrome and pulmonary veno-occlusive disease were reported for the first time in a PAH registry. Estimated prevalences were as follows: PAH, 16 and CTEPH, 3.2 cases per million adult inhabitants (MAI). Estimated incidences were as follows: PAH, 3.7 and CTEPH, 0.9 cases per MAI per yr. 1-, 3- and 5-yr survival was 87%, 75% and 65%, respectively, with no differences between PAH and CTEPH. Male sex, right atrial pressure and cardiac index were independent predictors of death. Matching between observed survival and that predicted by different equations was closer when the characteristics of the cohorts were similar. Epidemiology and survival of PAH patients in the Spanish registry are similar to recent registries. Characteristics of the population from which survival prediction equations are derived influence their applicability to a different cohort. CTEPH is much less prevalent than PAH, although has a similar survival rate.     

Evaluation of the Incidence of Chronic Thromboembolic Pulmonary Hypertension 1 Year After First Episode of Acute Pulmonary Embolism: A Cohort Study

Purpose

Chronic thromboembolic pulmonary hypertension (CTEPH) is an important complication after acute pulmonary embolism (PE) with considerable morbidity and mortality. The aim of this study was to estimate the CTEPH incidence in a cohort after the first occurrence of PE.

Methods

We conducted a 1-year follow-up cohort study between 2015 and 2018 to assess the incidence of CTEPH in 474 patients with their first acute episode of PE. For the diagnosis of CTEPH, patients with unexplained persistent dyspnea during follow-up underwent transthoracic echocardiography, right heart catheterization, ventilation-perfusion lung scanning, and CT pulmonary angiography.

Results

Overall, 317 patients were included in the study. The mean age of the patients was 56.5 ± 16 years. One hundred and three patients (32%) had exertional dyspnea at the 1-year follow-up. Patients with evidence of pulmonary hypertension (PH) on echocardiography underwent right heart catheterization. Eleven patients (18%) had no PH (mPAP < 25 mmHg); 47 patients (81%) had mPAP > 25 mmHg. Fifteen patients had PAWP > 15 mmHg, including those with underlying left heart problems or valvular diseases. There were 32 patients with PAH (mPAP > 25 mmHg and PVR > 3 WU) undergoing CTEPH studies; 22 patients (6.9%) had multiple segmental defects suggesting CTEPH on a perfusion scan.

Conclusion

The incidence of CTEPH observed in this study 1 year after the first episode of acute PE was approximately 6.9%. This incidence seems to be high in our population, and diagnostic and therapeutic strategies for the early identification of CTEPH are needed.

     

Epidemiology of pulmonary hypertension: new data from the Swiss registry

BACKGROUND: since 1999 data from pulmonary hypertension (PH) patients from all PH centres in Switzerland were prospectively collected. We analyse the epidemiological aspects of these data. METHODS: PH was defined as a mean pulmonary artery pressure of >25 mm Hg at rest or >30 mm Hg during exercise. Patients with pulmonary arterial hypertension (PAH), PH associated with lung diseases, PH due to chronic thrombotic and/or embolic disease (CTEPH), or PH due to miscellaneous disorders were registered. Data from adult patients included between January 1999 and December 2004 were analysed. RESULTS: 250 patients were registered (age 58 +/- 16 years, 104 (41%) males). 152 patients (61%) had PAH, 73 (29%) had CTEPH and 18 (7%) had PH associated with lung disease. Patients <50 years (32%) were more likely to have PAH than patients >50 years (76% vs. 53%, p <0.005). Twenty-four patients (10%) were lost to followup, 58 patients (26%) died and 150 (66%) survived without transplantation or thrombendarterectomy. Survivors differed from patients who died in the baseline six-minute walking distance (400 m [300-459] vs. 273 m [174-415]), the functional impairment (NYHA class III/IV 86% vs. 98%), mixed venous saturation (63% [57-68] vs. 56% [50-61]) and right atrial pressure (7 mm Hg [4-11] vs. 11 mm Hg [4-18]). DISCUSSION: PH is a disease affecting adults of all ages. The management of these patients in specialised centres guarantees a high quality of care. Analysis of the registry data could be an instrument for quality control and might help identify weak points in assessment and treatment of these patients.     

Clinical characteristics, diagnosis and management of patients with pulmonary thromboembolism who are not diagnosed in the acute phase and not classified as chronic thromboembolic pulmonary hypertension.

BACKGROUND: There have been many cases of pulmonary thromboembolism (PTE) that were not diagnosed in the acute phase and not classified as chronic thromboembolic pulmonary hypertension (CTEPH). The aim of the present study was clarify the clinical characteristics of chronic PTE. METHODS AND RESULTS: The study subjects were 601 patients (chronic PTE = 92, acute PTE = 456, CTEPH = 53) who were clinically diagnosed before their death. Dyspnea and chest pain, which are frequently found in acute PTE, were found less frequently in chronic PTE. The diagnosis of chronic PTE is often delayed in cases of mild to moderate severity with atypical onset. Chronic heart failure and chronic respiratory failure were most frequent in chronic PTE, and cerebrovascular disease was present in approximately 15% of the cases of chronic PTE. Pulmonary angiography and ventilation lung scan were used least frequently in acute PTE. Heparin and thrombolysis were used most frequently in acute PTE. CONCLUSIONS: Besides the atypical onset and reduced severity, the presence of preexisting diseases may be one of the reasons why the diagnosis for chronic PTE is delayed. The diagnostic and management techniques differ according to the type of PTE.