【摘要】 目的 探讨肺动脉高压患者药物靶向治疗的效果与耐受性。 方法 回顾分析2008年1月〖CD3/5〗2009年8月期间8例肺动脉高压患者分别接受波生坦及西地那非治疗的临床资料,评估其临床表现、WHO肺动脉高压功能分级、6 min步行距离及肺动脉收缩压在基线及治疗3个月后的变化。 结果 治疗后3个月,患者均能耐受药物治疗,无严重不良反应发生。WHO肺动脉高压功能分级在治疗前平均(31±04),治疗后为(23±09),明显得到改善(Plt;005)。肺动脉收缩压在治疗前平均(695±112 ) mm Hg(1 mm Hg=0133 kPa),治疗后为(483±124) mm Hg,明显降低(Plt;005)。6 min步行距离在治疗前平均(324±48) m,治疗后为(400±43) m,明显延长(Plt;005)。 结论 肺动脉高压患者药物靶向治疗的疗效显著,且耐受良好。【Abstract】 Objective To examine the effects of target medical therapy in patients with pulmonary arterial hypertension(PAH). Methods To determine the safety and efficacy of bosentan and sildenafil in eight patients with PAH.The patients’ clinical features, six minutes walking diastance, WHO functional class and systolic pulmonary arterial pressure (SPAP) were measured at baseline and at three months after initiating target medial treatment. Results At the three months followup assessments, WHO functional class was improved with 31±04 vs 23±09 (Plt;005); SPAP was significantly decreased with(695±112 ) mm Hg vs (483±124) mm Hg (Plt;005), the six minutes walking distance was significantly increased with(324±48) m vs(400±43) m (Plt;005). Target medical treatment was well tolerated. Conclusion Target medical treatment is well tolerated and has beneficial effects on PAH.
Objective To investigate the incidence and management of CTEPH in the Department of Pulmonary and Critical Care Medicine in Xijing Hospital to enrich the epidemiological data of chronic thromboembolic pulmonary hypertension (CTEPH) in China.Methods We conducted a retrospective study to investigate the incidence and management of CTEPH in the Department of Pulmonary and Critical Care Medicine in Xijing Hospital from 2008 to 2012. Results The incidence of CTEPH was 5.24% . About 62.90% of venous thromboembolism/pulmonary embolism (VTE/PE) patients were unprovoked, and about 53.85% of CTEPH patients was unprovoked. About 38% of CTEPH patients had no history of VTE, and 62% of CTEPH patients had no history of acute pulmonary embolism. None of the CTEPH patients was treated by pulmonary thromboendarterctom (PTE) , and about 53.85% of patients were only given anticoagulant monotherapy. Conclusions The incidence of CTEPH is higher in our hospital than reported. This phenomenon may be related to the lack of awareness of risk factors of CTEPH and the insufficient thrombolytic and anticoagulant therapy to acute pulmonary embolism. It’s very urgent to standardize the diagnosis and management of CTEPH in pulmonologists.
ObjectiveTo observe the pathological changes in heart and lung tissues in rats with pulmonary hypertension induced by monocrotaline. MethodsTwenty-four male Sprague-Dawley rats were randomly and equally divided into an experimental group and a control group. The rats in the experimental group were intraperitoneally injected with monocrotaline to induce pulmonary hypertension, and the rats in the control group were treated with saline. All rats were fed for 3 weeks, and the general situation were observed. Then the rats were sacrificed for measurement of mean pulmonary artery pressure (mPAP), right ventricular hypertrophy index [RV/(LV+S)], changes of myocardial cells and lung vascular, calculated density of middle membrane smooth muscle cells (SMC) in medium/small pulmonary arteries accompanied with bronchi and alveoli, media thickness of pulmonary artery (PAMT), the percentage of wall thickness with outer diameter (WT%), the percentage of wall area with total area (WA%), the average diameter of myocardial cells (AD), and myocardial nuclei density (MND). ResultsCompared with the control group, the condition of rats in the experimental group were getting worse obviously.mPAP and RV/(LV+S) were both increased (both P < 0.05). The observation by light microscope revealed that obvious myocardial hypertrophy and structure disturbances, severe luminal stenosis of medium/small pulmonary arteries, medial thickening, infiltration of inflammatory cell in tissue space, proliferation of unorganized collagen fibers in the experimental group. The observation by electronic microscope showed proliferation of endothelial cell with irregular nuclei, increased organelles and vacuoles in the experimental group. The differences in SMC, PAMT, WT%, WA%, AD, and MND were significant between two groups (all P < 0.05). ConclusionsThe monocrotaline can induced pulmonary hypertension and right ventricular hypertrophy. The mechanism may be related to severe stenosis or occlusion of the vessel lumen caused by plexiform proliferation of endothelial cells, proliferation of smooth muscle cells and collagen fibers, compensatory hypertrophy and hyperplasia of myocardial cells.
Abstract: Objective To retrospectively compare the difference of the effects of pulmonary thromboendarterectomy (PTE) between distal and proximal types of chronic thromboembolic pulmonary hypertension (CTEPH). Methods The data of 70 patients (including 44 male patients and 26 female patients, the average age was 46.2 years old, ranging from 17 to 72) with CTEPH having undergone PTE from March 2002 to March 2009 in Anzhen Hospital were retrospectively reviewed. We classified them into two different groups which were the proximal CTEPH group (n=51) and the distal CTEPH group (n=19) according to the pathological classification of the CTEPH. Clinical data, hemodynamics blood gas analysis and so on of both groups were compared. Results There was no perioperative deaths in both groups. Compared with the proximal group, cardiopulmonary bypass time [CM(159mm](189.5±41.5 min vs.155.5±39.5 min,P=0.003), aorta cross clamp time (91.3±27.8 min vs.67.2±27.8 min,P=0.002) and DHCA time (41.7±14.6 min vs.25.7±11.6 min,P=0.000) were significantly longer in the distal group. The incidence of residual pulmonary hypertension in the distal group was significantly higher than that in the proximal group (42.1% vs.13.7%,P=0.013), while the incidence of pulmonary reperfusion injury postoperatively in the proximal group was significantly higher than that in the distal group (41.2% vs.10.5%, P=0.021). SwanGanz catheterization and blood gas index were obviously improved in both groups. However, the pulmonary artery systolic pressure (PASP, 67.8±21.3 mm Hg vs.45.5±17.4 mm Hg,P=0.000) and the pulmonary vascular resistance ［PVR, 52.8±32.1 kPa/(L·s) vs.37.9±20.7 kPa/(L·s),P=0.024］ in the distal group were significantly higher than those in the proximal group and the partial pressure of oxygen in arterial blood of the distal group was significantly lower than that of the proximal group (76.7±8.7 mm Hg vs.88.8±9.3 mm Hg,P=0.000). After operation, 70 patients were followed up with no deaths during the followup period. The time of the followup ranged from 2 to 81 months (32.7±19.6 months) with a cumulative followup of 191.8 patientyears. Three months after operation, 47 patients were examined by pulmonary artery computer tomography angiogram (PACTA) and isotope perfusion/ventilation scan, which showed that the residual occlusive pulmonary artery segment in the proximal group was significantly fewer than that in the distal group (isotope perfusion/ventilation scan: 2.2±11 segments vs. 4.7±2.1 segments, P=0.000; PACTA: 3.5±1.4 segments vs. 4.9±2.0 segments,P=0.009). The New York Heart Association (NYHA) functional class and 6 minute walk distance (6MWD) in the proximal group were significantly better than those in the distal group (1.7±0.5 class vs 2.3±0.4 class; 479.2±51.2 m vs. 438.6±39.5 m, P=0.003). Venous thrombosis in double lower limbs reoccurred in two patients. According to KaplanMeier actuarial curve, the freedom from reembolism at 3 years was 96.7%±2.8%. Bleeding complications occurred in three patients. The linear Bleeding rate related to anticoagulation was 2.47% patientyears. Conclusion Although the early and midlong term survival rate of PTE procedure to treat both proximal and distal types of CTEPH is agreeable, the recovery of the PASP, PVR and 6MWD, and blood gases in patients with proximal type of CTEPH are significantly better than those in patients with distal type of CTEPH. On one hand, anticoagulation can singularly provide enough protection to patients with proximal type of CTEPH, but on the other hand, diuretics and pulmonary hypertension alleviation drug should be added to the treatment regimen for patients with distal type of CTEPH after the procedure of PTE.
ObjectiveTo investigate the clinical features and prognosis of fibrinous mediastinum and evaluate the value of different examinations in diagnosis and evaluation. MethodsTwenty-eight patients with mediastinal fibrosis diagnosed between January 2015 and September 2020 in China-Japan Friendship Hospital were studied retrospectively. The Clinical manifestations, radiological characteristics, endoscopic features, echocardiography, V/Q SPECT, cardiac catheterization, treatment and prognosis were analyzed.ResultsThe main clinical symptoms were cough (77.6%), expectoration (57.1%), wheezing or suffocating (42.9%), dyspnea (39.3%). There were 67.9% of the cases who were considered previous or present tuberculosis. Imaging findings showed that the fat density in the mediastinum disappeared, the irregular soft tissue of the mediastinum surrounded the airway and pulmonary vessels, and many lymph nodes enlarged and calcified, and multiple bronchus and pulmonary vessels were compressed and narrowed. Pulmonary function was mainly manifested as obstructive ventilate dysfunction and decreased diffusion volume. Under bronchoscopy, the bronchial mucosa showed pigmentation, bronchial distortion or multiple stenosis, even occlusion, and bronchial mucosa edema or congestion. Echocardiography and catheterization of the right heart showed that pulmonary hypertension and diastolic cardiac dysfunction were common complications of fibrillary mediastinum. Pulmonary ventilation perfusion imaging showed impaired blood perfusion in 87.5% of patients and impaired ventilation perfusion in 37.5% of patients. The symptoms of some patients alleviated after anti-infective and symptomatic treatment, but the mediastinal fibrosis was irreversible, and the efficacy of anti-tuberculosis and glucocorticoid therapy was limited. ConclusionsFor patients with clinical consideration of fibrous mediastinum, chest enhanced CT should be performed for clear diagnosis. Relevant examinations, such as pulmonary function, endoscopic, echocardiography, should be conducted to evaluate whether the disease involves airway, pulmonary vessels, pericardium, superior vena cava and esophagus, as well as the degree of functional involvement. Attention should be paid to the evaluation of patients with pulmonary hypertension and diastolic cardiac insufficiency.
ObjectiveTo observe the relationship of serum tumor necrosis factor α (TNF-α), interleukin 6 (IL-6), and C-reactive protein (CRP) with obstructive sleep apnea hypopnea syndrome (OSAHS) associated pulmonary hypertension (OSAHS-PH). MethodsFrom September 2013 to October 2014, 38 OSAHS patients, 32 OSAHS-PH patients and 35 healthy subjects were enrolled from the General Hospital of Ningxia Medical University. OSAHS was diagnosed by polysomnography. The pulmonary artery systolic pressure (PASP) was measured by echocardiograph, and the diagnose criteria for pulmonary hypertension was PASP≥40 mm Hg. Serum TNF-α, IL-6, CRP and endothelin 1 (ET-1) were detected by enzyme-linked immunosorbent assay. The correlation between TNF-α, IL-6, CRP, ET-1 and PASP was analyzed. ResultsThe serum levels of TNF-α, IL-6, CRP and ET-1 were remarkably different among three groups (F=55.34, 25.05, 23.85, 34.06 respectively; all P < 0.05). The levels of TNF-α, IL-6, CRP and ET-1 in the OSAHS group were higher than those in the healthy group, and lower than those in the OSAHS-PH group (all P < 0.05). The PASP was positively correlated with the levels of the four factors (r=0.755, 0.762, 0.747, 0.759 respectively; all P < 0.01). ConclusionThe levels of serum TNF-α, IL-6 and CRP are correlated with pulmonary hypertension and they may be involved in the process of OSAHS-PH.
Aiming at the problems of obscure clinical auscultation features of pulmonary hypertension associated with congenital heart disease and the complexity of existing machine-aided diagnostic algorithms, an algorithm based on the statistical characteristics of the high-frequency components of the second heart sound signal is proposed. Firstly, an endpoint detection adaptive segmentation method is employed to extract the second heart sounds. Subsequently, the high-frequency component of the heart sound is decomposed using the discrete wavelet transform. Statistical features including the Hurst exponent, Lempel-Ziv information and sample entropy are extracted from this component. Finally, the extracted features are utilized to train an extreme gradient boosting algorithm (XGBoost) classifier, which achieves an accuracy of 80.45% in triple classification. Notably, this method eliminates the need for a noise reduction algorithm, allows for swift feature extraction, and achieves effective multi-classification using only three features. It is promising for early screening of pulmonary hypertension associated with congenital heart disease.