Echocardiographic evaluation of left-ventricular diastolic function in patients with chronic pulmonary hypertension.

Different patterns of left-ventricular (LV) diastolic dysfunction were reported in patients with pulmonary hypertension (PHT). There are no data regarding the relationship between the severity of PHT and LV diastolic dysfunction. In order to determine the severity of PHT at which LV diastolic dysfunction occurs and to identify its pattern, we studied by Doppler echocardiography 120 patients with PHT (57 with severe PHT and 63 with mild or moderate PHT) and compared them with 75 normal controls. Systolic pulmonary artery pressure (SPAP) was measured by tricuspid regurgitant jet method and the usual transmitral LV diastolic indices were recorded. LV diastolic dysfunction of impaired relaxation type is most commonly seen in patients with severe PHT. No differences were observed between patients with mild and moderate PHT regarding LV diastolic function. A SPAP > or =60 mm Hg is needed to induce changes in the LV diastolic filling pattern.     

Nocturnal pulmonary hypertension in patients with chronic obstructive pulmonary disease.

Oxygen desaturation occurs during sleep in some patients with COPD. To investigate the effects of these hypoxemic episodes on the pulmonary vasculature, we studied four patients with our routine polysomnographic techniques and simultaneously recorded pulmonary artery pressure. In all four subjects, nocturnal episodes of desaturation were accompanied by elevations in the pulmonary artery pressure. Low flow oxygen abolished the drops in arterial oxygen saturation (but not the breathing abnormalities) and no elevations in the PA pressure were observed. We postulate that in some COPD patients these initially transient events may lead to sustained pulmonary hypertension and cor pulmonale. Nocturnal oxygen therapy may be indicated in more patients than previously suspected and may prevent the development of cor pulmonale.     

Bosentan for patients with chronic thromboembolic pulmonary hypertension

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease leading to worsening functional status and reduced survival for those patients who cannot undergo pulmonary endarterectomy (PEA). Pharmacotherapy with novel drugs for pulmonary hypertension may be useful in treating patients who are poor candidates for surgery, but there are still few clinical data on medical therapy for CTEPH.The aim of this prospective open-label, multicenter, study is to compare the efficacy of 1-year bosentan treatment to standard drugs in nonoperated patients with CTEPH.Patients and methodsThirty-four nonoperated patients with CTEPH were enrolled. Functional assessment included 6 minute walk test (6MWT), Borg index, WHO classification, arterial blood gases and echocardiography systolic pulmonary artery pressure (sPAP). Seventeen patients received bosentan (62.5 mg bid for 4 weeks and then 125 mg bid); 17 patients were treated with standard therapy alone.ResultsAt admission sPAP was 76.18 +/? 5.96 mmHg in bosentan group and 71.48 +/? 3.71 mmHg in controls, p_aO₂ 64.68 +/? 2.25 mmHg in bosentan group, and 59.52 +/? 2.05 mmHg in controls, 6MWT 297.53 +/? 34.25 mt in bosentan group, and 268.47 +/? 36.54 mt in controls. After 12 months there were significant differences between the groups in the 6MWT (+ 57.24 +/? 22.21 m vs ? 73.13 +/? 21.23 m, p < 0.001), dyspnoea index (Borg score 4.29 +/? 0.49 vs 7.06 +/? 0.32, p < 0.001) and oxygenation (p_aO₂ 65.93 +/? 3.76 mmHg vs 48.48 +/? 1.31 mmHg, p < 0.001). The sPAP was stable after 12 months of bosentan (76.18 +/? 5.96 mmHg vs 71.00 +/? 5.41 mmHg, p = 0.221) in contrast to controls (71.48 +/? 3.71 mmHg vs 80.44 +/? 4.70 mmHg, p = 0.029).ConclusionThe data of this open-label study in nonoperated CTEPH patients suggest an improvement in functional outcomes adding Bosentan to diuretics and oral anticoagulants. No improvement was observed using only standard drugs after 1-year.     

Haemodynamics in patients with chronic post-embolic pulmonary hypertension

Haemodynamic studies were undertaken in 30 patients with chronic post-embolic pulmonary hypertension (CPEPH), and the findings were compared with those found in acute thromboembolism of the pulmonary artery. The study showed that radiocardiographic examination is a useful supplementary method for diagnosing postembolic lesions of pulmonary arteries and for dynamic examination of patients after pulmonary embolism. The appearance of a "single-hump" curve on the radiocardiogram was an unfavourable prognostic sign and attested both to an increase of pulmonary hypertension or to a latent heart failure. The importance of radiocardiographic examination for determining the prognosis of the disease and for choosing the most suitable method of its treatment is analysed.     

Clinical characteristics of patients with chronic thromboembolic pulmonary hypertension

<正>Objective To investigate the clinical characteristics of patients with chronic thromboembolic pulmonary hypertension (CTEPH).Methods CTEPH cases consecutively admitted into China-Japan Friendship Hospital from September 2015 to June 2019 were enrolled with prospective data collection.The medical histories,clinical characteristics,laboratory tests,imaging manifestation and hemo-     

Differences in patients with chronic pulmonary embolism and primary pulmonary hypertension

Chronic pulmonary embolism is a rare disease which can occur at first with pulmonary hypertension. In these cases it may be difficult to distinguish between primary pulmonary hypertension. We examined nine patients with Chronic Pulmonary Embolism (CPE) (three females and six males, mean age 45 +/- 13 years, range 21-67 years) and ten patients with Primary Pulmonary Hypertension (PPH) (seven females and three males, mean age 35 +/- 13 years, range 10-56 years) who came to our attention during the years 1973-1986 (mean follow up 3 years). All patients had an electrocardiogram, chest x-ray, echocardiogram, cardiac catheterization with pulmonary angiography; seven patients with CPE and eight with PPH had perfusion lung scans. Progressive dyspnoea was the main feature in all the patients; four out of nine with CPE and none of the ones with PPH had a previous history of thrombophlebitis. In all the patients the electrocardiogram, chest x-ray and echocardiogram showed signs of pulmonary hypertension, so that a clear distinction between the two groups was not possible. Cardiac catheterization showed pulmonary pressure values higher in patients with PPH as compared to the ones with CPE (systolic pressure 96 mmHg vs 70 mmHg, diastolic pressure 49 mmHg vs 31 mmHg, mean pressure 65 mmHg vs 45 mmHg). Pulmonary angiography in more than half of the patients with CPE showed a "cut off" of two or more lobar branches of the pulmonary arteries. In the patients with PPH pulmonary angiography showed a dilatation of the main pulmonary artery and a diffuse bilateral hypoperfusion. Perfusion lung scan in all the cases of CPE showed zonal perfusion defects, while in all cases of PPH, with the exception of one, it was largely normal. Venograms in the districts of the inferior vena cava demonstrated thrombosis in two out of six patients with CPE. Negative venograms were found in the five patients with PPH who had this investigation performed. One patient with CPE had a surgical embolectomy, the other eight had anticoagulant oral treatment. During the follow-up period three patients with CPE and five with PPH died within five years and within fifteen months respectively, of the diagnosis.(ABSTRACT TRUNCATED AT 400 WORDS)     

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.     

Clinical evaluation of nebulized verapamil in out‐patients with pulmonary hypertension secondary to chronic obstructive pulmonary disease

ObjectiveChronic obstructive pulmonary disease (COPD) is associated with many health complications, including pulmonary hypertension (PH). Although oral calcium channel blockers have shown promising results in managing COPD‐induced PH, significant systemic side effects may limit their use in this population. Administering verapamil through nebulization can be an alternative approach. We aim to assess the possible therapeutic effects of verapamil inhalation in out‐patients with pulmonary hypertension (PH) secondary to COPD.MethodsA double‐blind, randomized placebo‐controlled clinical trial was conducted. Patients with PH were randomly assigned to two groups of 15 participants. The intervention group received a short‐term single dose of 10 mg nebulized verapamil (4 ampoules of 2.5 mg/ml verapamil solutions). The control group received nebulized distilled water as a placebo in addition to their standard treatment throughout the study.ResultsSystolic pulmonary artery pressure (sPAP) did not improve as a primary outcome significantly in patients receiving nebulized verapamil compared with those on placebo (p = 0.89). Spirometry results showed a significant improvement in FVC in the intervention group from 1.72 ± 0.63 to 1.85 ± 0.58 L (p = 0.00), and FEV1/FVC ratio decreased significantly after verapamil administration (p = 0.027).ConclusionVerapamil did not improve any of the pulmonary artery or RV parameters in patients with COPD‐associated, but it did improve SpO₂ and increase FVC, which revealed us possibility of verapamil in treating V/Q mismatch. The improved gas exchange may have been due to improvements in FVC as reflected in the improved spirometry. Higher doses of verapamil may be more efficacious and can be the subject of future trials.     

hs-CRP、Fib、NT-proBNP在COPD合并肺动脉高压中的研究及意义

目的观察C反应蛋白(hs-CRP)、纤维蛋白原(Fib)、N-端脑钠肽前体(NT-proBNP)在慢性阻塞性肺病(COPD)合并肺动脉高压患者血液中的变化,分析相关性及意义。方法选取我院COPD患者105例,分A组(单纯COPD组,55人)、B组(COPD合并肺动脉高压组,50人)。行肺功能、心脏彩超检查,检测hs-CRP、NT-proBNP、Fib及血气分析。结果 hs-CRP、Fib、NT-proBNP在B组均高于A组(均P0.05);PaCO2、hs-CRP、Fib、NT-proBNP水平与肺动脉压呈正相关;PaCO2、hs-CRP、Fib与NT-proBNP呈正相关。结论hs-CRP、Fib、NT-proBNP参与了COPD合并肺动脉高压的形成,全身炎症在COPD继发肺动脉高压的发病中发挥重要作用。     

Improved survival in patients with inoperable chronic thromboembolic pulmonary hypertension

Patients with inoperable chronic thromboembolic pulmonary hypertension (Inop-CTEPH) treated with conventional therapy have a poor survival. We compare the 3-year survival between those treated with conventional therapy and those treated with conventional therapy and a combination of novel drugs. We also evaluate the clinical course. A total of 34 Inop-CTEPH consecutive patients were evaluated from 1991 to 2009 including right heart catheterization (RHC) and perfusion lung scan (PLS): 7 underwent surgical treatment while 27 were confirmed inoperable. Of these 27 patients, 12 evaluated from 1991 to 2003 (Group 1) were treated with conventional therapy and 15 evaluated from 2004 to 2009 (Group 2) were treated with conventional and novel therapies. At baseline, no group difference emerged at RHC. Based on clinical course, novel drugs and oxygen supplementation were given to patients of Group 2. Seven of these who had worse clinical course repeated RHC and four of them also PLS during therapy. Those without repeat RHC had baseline pulmonary artery mean pressure and brain natriuretic peptide (NT-proBNP) lower and mixed venous saturation (SvO₂) and exercise test higher (p = 0.022, 0.015, 0.044 and 0.003, respectively). During therapy, those with repeat RHC had total pulmonary vascular resistance reduced (p = 0.012), base excess increased (p = 0.002) and significant redistribution of pulmonary blood flow at PLS. At the 3-year follow-up, survival was 86% in Group 2 and 31% in Group 1 (p = 0.031). In Inop-CTEPH patients, the clinical course may help to select drugs and the level of oxygen supply that can improve hemodynamics, gas exchange and long-term survival.     

Effects of diprophylline on pulmonary hypertension in patients with chronic pulmonary disease]

The present study was undertaken to evaluate the short-term effects of diprophylline on hemodynamic and gases exchange in 10 patients in stable condition with chronic pulmonary disease. Diprophylline decreased the pulmonary vascular resistance by markedly reducing the mean pulmonary arterial pressure but didn't change cardiac output significantly. Diprophylline also caused a significant decrease in arterial carbon dioxide tension, but there was no significant difference between the level of PaO2, SaO2 and A-aDo2 before and after the administration of diprophylline.     

Haemodynamic evaluation of pulmonary hypertension.

Pulmonary hypertension is characterised by the chronic elevation of pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) leading to right ventricular enlargement and hypertrophy. Pulmonary hypertension may result from respiratory and cardiac diseases, the most severe forms occurring in thromboembolic and primary pulmonary hypertension. Pulmonary hypertension is most often defined as a mean PAP >25 mmHg at rest or >30 mmHg during exercise, the pressure being measured invasively with a pulmonary artery catheter. Doppler echocardiography allows serial, noninvasive follow-up of PAPs and right heart function. When the adaptive mechanisms of right ventricular dilatation and hypertrophy cannot compensate for the haemodynamic burden, right heart failure occurs and is associated with poor prognosis. The haemodynamic profile is the major determinant of prognosis. In both primary and secondary pulmonary hypertension, special attention must be paid to the assessment of pulmonary vascular resistance index (PVRI), right heart function and pulmonary vasodilatory reserve. Recent studies have stressed the prognostic values of exercise capacity (6-min walk test), right atrial pressure, stroke index and vasodilator challenge responses, as well as an interest in new imaging techniques and natriuretic peptide determinations. Overall, careful haemodynamic evaluation may optimise new diagnostic and therapeutic strategies in pulmonary hypertension.     

阿托伐他汀对慢性阻塞性肺疾病合并肺动脉高压患者慢性炎症反应的影响

目的 探讨阿托伐他汀对慢性阻塞性肺疾病(COPD)合并肺动脉高压(PH)患者慢性炎症反应的影响及其安全性.方法 选择COPD合并PH患者78例,随机分为阿托伐他汀组和常规组,两组各39例.常规组采用常规治疗措施治疗,阿托伐他汀组在常规组基础上加用阿托伐他汀.比较两组患者治疗前、治疗后3个月时炎性因子、一氧化氮(NO)、内皮素-1(ET-1)、肺动脉平均压(PAPm)、肺动脉收缩压(PAPs)及肺功能指标变化情况.结果 两组患者治疗后白细胞介素(IL)-6、肿瘤坏死因子(TNF)-α、C反应蛋白(CRP)、ET-1、肺动脉平均压(PAPm)、肺动脉收缩压(PAPs)水平均较治疗前明显降低(P＜0.05),但阿托伐他汀组降低程度更明显(P ＜0.05);NO、FEV1％、FVC较治疗前明显升高(P＜0.05),而阿托伐他汀组升高程度更明显(P＜0.05).阿托伐他汀组服药期间无明显不良反应发生.结论 阿托伐他汀可降低COPD合并PH患者的各种细胞炎性因子,减轻炎症反应,调节NO与ET-1的平衡,降低PH,同时减少肺损伤,改善肺功能,疗效安全可靠,可作为治疗COPD合并PH的有效药物.     

Balloon pulmonary angioplasty attenuates sleep apnea in patients with chronic thromboembolic pulmonary hypertension

BackgroundBalloon pulmonary angioplasty (BPA) improves pulmonary hemodynamics in chronic thromboembolic pulmonary hypertension (CTEPH) patients. However, whether it affects the severity of sleep apnea (SA) remains unknown. We investigated the effect of BPA on the severity of SA in CTEPH patients.MethodsWe studied 13 patients with CTEPH who had an apnea hypopnea index (AHI) > 10 before BPA and underwent a second polygraph test 6 months after the last BPA session.ResultsBPA decreased pulmonary vascular resistance, mean pulmonary artery pressure (PAP), and plasma B-type natriuretic peptide levels, and increased the 6-minute walking distance. BPA decreased the AHI (from 20.9 [13.9–35.7] to 16.3 [7.7–21.8] times/hour, P = 0.023) and hypopnea index (from 13.2 [8.4–22.5] to 6.4 [3.8–10.9] times/hour, P = 0.013), but not the obstructive, central, or mixed apnea index. The change in AHI correlated with that in mean PAP, but not with the change in body mass index or other parameters of hemodynamics.ConclusionsBPA-induced improvement in hemodynamics was associated with the attenuation of SA in patients with CTEPH and SA. Therefore, close attention should be paid to SA in CTEPH patients, and SA should be re-evaluated after BPA to avoid overestimating its severity.     

Rheologic characteristics of blood in patients with chronic post-embolic pulmonary hypertension

Examination of blood rheologic properties in 38 patients with chronic ++post-embolic pulmonary hypertension revealed an elevated blood viscosity at all shift rates and a reduced erythrocyte deformability. A close positive correlation was found between blood viscosity and perfusion deficit. Hemorheologic disorders in patients with chronic post-embolic + pulmonary hypertension are caused by pachyhemia and blood acid-base imbalance due to respiratory failure. They deteriorate pulmonary and systemic hemodynamic disturbances and contribute to progression of blood flow derangement.     

Echocardiographic evaluation of right cardiac function in patients with chronic pulmonary diseases.

It is clinically important to evaluate the severity of right ventricular (RV) overload in patients with chronic pulmonary diseases (CPD). For such evaluation, echocardiography has been widely used because the procedure is noninvasive and can be performed repeatedly. We evaluated the severity of RV overload in CPD patients to assess the usefulness of pulsed Doppler echocardiography. The A/E ratio and deceleration time of early RV inflow velocity correlated significantly with the mean pulmonary artery pressure (MPAP) both in patients with and without CPD. The acceleration time/RV ejection time (AcT/RVET) was significantly lower in CPD patients than control subjects and correlated significantly with MPAP. Furthermore, AcT/RVET improved in patients with mild respiratory failure after oxygen therapy, along with a decrease in MPAP. We also compared the new index of myocardial performance (NI) in control subjects and patients with pulmonary tuberculosis sequelae (TB) undergoing home oxygen therapy. The NI was significantly higher in the TB group. Although these results were satisfactory, the pulsed Doppler echocardiography has certain disadvantages because monitoring is influenced by anatomical factors and it is difficult to perform in patients with atrial fibrillation or tachycardia. We conclude that echocardiography using a Doppler method is a useful noninvasive technique for assessment of the right heart system. The precision of this procedure can be improved by combination with other echocardiographic indices of RV overload.