【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.
Objective To confirm the changes of pulmonary artery pressure, neo pulmonary artery stenosis and reoperation in children with unilateral absence of pulmonary artery (UAPA) undergoing pulmonary artery reconstruction. Methods The clinical data of the infants with UAPA undergoing pulmonary artery reconstruction in our hospital from February 19, 2019 to April 15, 2021 were analyzed. Changes in pulmonary artery pressure, neo pulmonary artery stenosis and reoperation were followed up. Results Finally 5 patients were collected, including 4 males and 1 female. The operation age ranged from 13 days to 2.7 years. Cardiac contrast-enhanced CT scans were performed in all children, and 2 patients underwent pulmonary vein wedge angiography to confirm the diagnosis and preoperative evaluation. Preoperative transthoracic echocardiography and intraoperative direct pulmonary arterial pressure measurement indicated that all 5 children had pulmonary hypertension, with a mean pulmonary arterial pressure of 31.3±16.0 mm Hg. Pulmonary arterial pressure decreased immediately after pulmonary artery reconstruction to 16.8±4.2 mm Hg. The mean follow-up time was 18.9±4.7 months. All 5 patients survived during the follow-up period, and 1 patient had neo pulmonary artery stenosis or even occlusion and was re-operated. Conclusion Pulmonary artery reconstruction can effectively alleviate the pulmonary hypertension in children with UAPA. The patency of the neo pulmonary artery should be closely followed up after surgery, and re-pulmonary angioplasty should be performed if necessary.
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.
Follow the advance of surgical treatment in complex congenital heart disease, the application of right ventricular(RV)-bypass procedures in RV-hypoplasia/dysfunction was gradually recognized; the pathological pattern of RV-hypoplasia/dysfunction, the histological change of RV-bypass operation and the option on different operative procedure, emphasis on the indication of bidirectional superior cavopulmonary anastomosis(BCPA),the form and method of procedure,and peri-operative management were reviewed in this paper,the optimal age for BCPA, the optimal timing for conversion to Fontan procedure, on pump or off pump, section of the pulmonary trunk, and its difference from hemi-Fontan and 1 1/2 ventricular operation were discussed.
ObjectiveTo analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations.MethodsWe retrospectively analyzed the clinical data of 146 patients undergoing multiple cardiac re-sternotomy operations between 2015 and 2019 in our center. There were 95 males and 51 females with an age of 4.3 (3.1-6.8) years and a weight of 15.3 (13.4-19.0) kg at last operation.ResultsThe top three cardiac malformations were pulmonary atresia (n=51, 34.9%), double outflow of right ventricle (n=36, 24.7%) and functional single ventricle (n=36, 24.7%). A total of 457 sternotomy procedures were performed, with 129 (88.3%) patients undergoing three times of operations and 17 (11.7%) patients undergoing more than three times. Fifty-two (35.6%) patients received bi-ventricular repair, 63 (43.1%) patients received Fontan-type procedures, and 31 (21.2%) patients underwent palliative procedures. Ten (6.8%) patients experienced major accidents during sternotomy, including 7 (4.8%) patients of urgent femoral artery and venous bypass. Eleven (7.5%) patients died with 10 (6.8%) deaths before discharge. The follow-up time was 20.0 (5.8-40.1) months, and 1 patient died during the follow-up. The number of operations was an independent risk factor for the death after operation.ConclusionSeries operations of Fontan in functional single ventricle, repeated stenosis of pulmonary artery or conduit of right ventricular outflow tract post bi-ventricular repair are the major causes for the reoperation. Multiple operations are a huge challenge for CCHD treatment, which should be avoided.