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.
Objective To improve the knowledge of pulmonary artery sarcoma ( PAS) and early diagnosis.Methods The clinical data of 8 patients with PAS confirmed by biopsy from April 2001 to April 2012 in Beijing Anzhen Hospital were retrospectively analyzed. Results There were 5 males and 3 females, with mean age of 46. 75 ±11. 47 years [ range: 32-67 years] . The main clinical manifestations were chest tightness, shortness of breath, intermittent syncope, heart palpitations at exertion, etc. Laboratory examinations showed the patients with PAS have no obvious hypoxemia and most of them have normal D-dimer level. Echocardiography revealed pulmonary hypertension, right ventricular enlargement, and echo of massive lumps in main pulmonary truck. Lower limb veins were normal in color doppler ultrasonography. Chest X-ray revealed prominent pulmonary artery segment, full segment of the right pulmonary artery, an increased hilum and pleural effusion. CT pulmonary angiography showed expansion of pulmonary artery, large filling defect in main pulmonary truck and left or right pulmonary artery, combined with pericardial effusion, pleural effusion. Lung ventilation/perfusion imaging did not match, showing radioactive sparse and defects in multiple lung segments and subsegments, involved 3 to 13 lung segments. Pulmonary angiography showed filling defects in the main pulmonary artery, left or right pulmonary artery. 8 patients were confirmed pathologically after operation. Pathological results showed leiomyosarcoma differentiation in 3 cases, undifferentiated sarcoma in2 cases, and undefined pathological type in 3 cases. All 8 patients were misdiagnosed as pulmonary embolism before surgery. The average days of misdiagnosis were 85. 6 ±21. 5 days. 7 cases were given simple surgical resection, one case underwent surgical resection combined with radiotherapy and chemotherapy. 7 cases were relieved and discharged, and one case died. Conclusion PAS is a rare disease clinically and is easily misdiagnosed as pulmonary embolism. Clinicians should enhance the recognition in order to diagnose early and treat comprehensively.
Abstract: Objective To observe the physical characteristics of decellularized porcine pulmonary valved conduits crosslinked by carbodiimide (EDC). Methods [WTBZ]Twenty porcine pulmonary valved arteries were mobilized on relative asepsis condition. They were cut longitudinally into three samples at the junction position of pulmonary valve (every sample was comprised of a part of the pulmonary conduit wall and the corresponding valve). The samples were randomly divided into three groups by lotdrawing method. Group A was the control group which was made up of the fresh porcine arterial valved conduit samples without any other treatments. Group B was comprised of porcine pulmonary samples decellularized by trypsindetergent digestion. Group Cincluded the decellularized porcine pulmonary samples crosslinked by EDC. We observed the water content, thickness, tensile strength, and shrinkage temperature of all the samples, based on which the physical characeteristics of these samples were analyzed. Results [WTBZ]Complete cellfree-pulmonary conduit matrix was achieved by trypsindetergent digestion. Compared with group A, in group B, the water content of pulmonary wall was significantly higher (P=0.000), while the water content of pulmonary valve was not significantly different; the thickness of pulmonary wall and valve (P=0.000，0.000) and tensile strength of pulmonary wall and valve (Plt;0.01） was significantly lower, while shrinkage temperature was not significantly different. Compared with group B, in group C, the water content of pulmonary wall was significantly lower (P=0.000), while the water content of pulmonary valve, and the thickness of pulmonary wall and valve were not significantly different; the tensile strength of pulmonary wall (Plt;0.01) and valve (P=0.000), and the shrinkage temperature of them (P=0.000, 0.000) were significantly higher. Compared with group A, in group C, the water content of pulmonary wall and valve, and the tensile strength of them were not statistically different, while the thickness of pulmonary wall and valve was significantly lower (P=0.000, 0.000), and the shrinkage temperature of them was significantly higher (P=0.000, 0.000). Conclusion [WTBZ]EDC crosslinking method is available for treating decellularized porcine pulmonary valved conduits in order to enhance its tensile strength, and decrease water content of pulmonary wall.
Abstract: Objective To investigate the safety and feasibility of fast track (FT) treatment in young children with atrioventricular septal defect (CAVSD) and pulmonary artery hypertension (PAH) following surgical repair. Methods A total of 51 young children patients including 24 boys and 27 girls with age at 12.5±8.9 months from 4 to 36 months, underwent CAVSD repair in the pediatric surgery department of Fu Wai Hospital from January 2006 to March 2009. Among them, 21 patients were administered FT management. PICU length of stay and the rate of reintubation were analyzed retrospectively and the decrease of pulmonary artery pressure (PAP) after operation was also measured. Results Twentyone patients under FT treatment were extubated within 8 hours after operation. The mean pulmonary artery pressure(MPAP) decreased significantly after surgery (39.59 mm Hg vs.24.50 mm Hg,t=5514,Plt;0.05). PICU length of stay was 2.05±0.87 d (18 h-3 d). One patient was reintubated due to lung infection, which had nothing to do with the FT treatment. During the followup which lasted for 3 to 6 months, 21 patients had good heart function with no reoperation or death. Conclusion FT treatment is safe and feasible to some CAVSD patients associated with PAH, and shorter PICU length of stay can be achieved. The validation of FT model for the CAVSD patients with severe PAH needs research with large sample.
Objective To compare the vasoactive effects of norepinephrine( NE) and dopamine of different doses on isolated rabbit pulmonary and systemic arteries in septic shock. Methods Six paired pulmonary and systemic arterial rings were prepared fromsix rabbits, and matched randomly assigned into a normal group and a LPS group. The assigned groups were intervened by different doses of NE. Another six paired pulmonary and systemic arterial rings were prepared from another six rabbits. They were assigned to different groups as above and intervened by different doses of dopamine. The LPS groups were pre-incubated in RPMI mediumsupplemented with4 μg/mL LPS to simulate septic shock. The tension of arterial rings was measured and its response to NE and dopamine were studied. Results ( 1) In the normal groups, the contraction of the systemic arteries was ber than the pulmonary arteries in response to low,middle dose of NE, and high dose of dopamine ( all P lt; 0. 05) , and which was weaker in response to middle dose of dopamine and similar in response to high dose of NE( P gt;0. 05) . Both the pulmonary and systemic arteriesrelaxed in response to low dose of dopamine. ( 2) After LPS pre-incubation, the contraction of the systemic arteries was weaker than the pulmonary arteries in response to low dose of dopamine ( P lt;0. 05) , and which was similar in response to low,middle and high dose of NE, and middle, high dose of dopamine. ( 3) Comparing the LPS groups with the normal groups, the contraction in response to middle dose of dopamine increased in the systemic arteries and dreased in the pulmonary arteries ( P lt;0. 05) . Conclusions In septic shock, the vasoactive effect of different doses of NE is not different between pulmonary and systemic arteries. But middle dose of dopamine can increase the contraction of systemic arteries and decrease the contraction of pulmonary arteries.
Objective To extract and identify primary culture rat pulmonary arterial smooth cells ( PASMCs) , and investigate the effects of hypoxia on the proliferation of PASMCs. Methods Rat PASMCs were separated by the method of tissue block anchorage, and the cellular morphology was observed under light microscope. The cells were identified by projection electron microscopy, and α-smooth muscle actin ( α-SMactin)in the cells was identified by immunohistochemistry and immunofluorescence. The primary cultured PASMCs were exposed to normoxic and/ or hypoxia condition for 2, 6, 12, 24, 48 hours respectively, thenMTT assay and PCNA ( proliferating cell nuclear antigen) immunohistochemistry were used to detect the proliferation of PASMCs. Results The cells tended to be long spindle and grew in the “peak-valley”mode under light microscope. Immunology results showed that endochylema was stained in brownish yellow, and the positive rate was beyond 96% . There were dense patch, dense body and many filaments in endochylema under projection electron microscopy. MTT assay demonstrated that the A values of PASMCs expose to hypoxia were higher than that of nomoxia. Comparing with normoxia, the A values of PASMCs exposed to hypoxia increased after 12 hours ( P lt;0. 05) , significantly increased after 24 hours ( P lt;0. 01) . Compared with 2 hours’exposure to hypoxia, the A values increased after 12 hours( P lt; 0. 05) , markedly increased after 24 hours ( P lt; 0. 01 ) , which after 48 hours was similar with 24 hours. The result of PCNA immunohistochemistry was consistent with that of MTT. Conclusions The tissue explants adherent method is simple and convenient, and can easily obtain rat PASMCs with high purity and stability. Hypoxia canpromote the proliferation of PASMCs.
ObjectiveTo improve the knowledge of clinical features,image and pathology of Behcet's disease with the pulmonary artery aneurysm. MethodsOne typical female patient aged 40 years with recurrent pulmonary artery aneurysm caused by Behcet's disease was analyzed including clinical data,image and pathological features.The Pubmed (1967.1 to 2013.10) and Wanfang database (1982.1 to 2013.10) were searched with "Behcet's disease","pulmonary artery aneurysm" as search terms. ResultsRecurrent hemoptysis was major clinical manifestation of pulmonary artery aneurysm caused by Behcet's disease.Features of image including hilar enlargement,pulmonary artery aneurysm and primary thrombus.During the first hospitalization,the patient was diagnosed as pulmonary artery aneurysm.Then pulmonary lobectomy was performed and pathology examination revealed artery aneurysm and primary thrombus.During the second hospitalization,she was diagnosed as pulmonary thromboembolism and it was considered as failure of treatment of anticoagulant therapy.During the third hospitalization,she was diagnosed as pulmonary artery aneurysm of Behcet's disease,and recurrent hemoptysis was fully ameliorated by the combination treatment of glucocorticoid and immunodepressant. ConclusionPulmonary artery aneurysm is the typical feature of pulmonary vascular lesions of Behcet's disease,and recurrent hemoptysis is major clinical manifestation.CT angiography can identify location of pulmonary artery aneurysm.The key drugs of therapy are imunosuppressive drugs in combination with steroids.
ObjectiveIn order to correct left pulmonary artery (LPA) stenosis in tetralogy of Fallot (TOF), a technique for LPA reconstruction by widening pulmonary arterial posterior wall was devised. To evaluate its surgical technique as well as postoperative outcome. MethodsFrom January 2008 through August 2014 year, 1 142 consecutive patients underwent repair of TOF including 44 patients with TOF and LPA stenosis in our hospital. We used widening pulmonary arterial posterior wall for LPA reconstruction in 21 patients. The median age was 22.8 months (range, 1 month to 11 years), and the median weight was 9.1 kg (range, 3.8-29 kg). ResultsThere was one operative death. No death occurred during the follow-up period in the other 20 patients. There were 4 patients with complicate unbalanced pulmonary perfusion postoperatively. Echocardiography at 3-72 months follow-up demonstrated no obvious stenosis was found at LPA in 14 patients, and mild stenosis (32.8±12.7 mm Hg) in 6 patients. Only 1 patient required further interventions because of restenosis of LPA. ConclusionsLPA reconstruction by widening pulmonary arterial posterior wall is an effective method in the management of stenosis of LPA in the patients with TOF. We emphasize division of the arterial duct or ligamentum completely because it will tether and kink the LPA. This procedure retains the better growth potential by increasing autogenous tissue of native tissue.
ObjectiveTo investigate pulmonary artery growth, valvular regurgitation and right heart function after right ventricle-pulmonary artery (RV-PA) anastomosis for right ventricle outflow (RVOT) reconstruction in patients with different types of pulmonary atresia and ventricle septal defect (PA/VSD). MethodsClinical data of 31 PA/VSD patients who underwent right ventricle-pulmonary anastomosis for RVOT reconstruction from November 2002 to September 2012 in Guangdong General Hospital were retrospectively analyzed. There were 20 male and 11 female patients with their age ranging from 14 days to 14.50 years (47.90±53.84 months). Patients were followed up after discharge to evaluate the anastomasis, pulmonary artery growth, pulmonary artery and tricuspid regurgitation and their relationship with follow-up duration. Right ventricular strain and other echocardiography indexes were used to evaluate right heart function. ResultsThree patients died postoperatively. Twenty-seven patients (27/31, 87.1%)were followed up for 4-129 (35.97±28.24)months. There was no late death during follow-up. There was no anastomotic restenosis in patients who received radical surgery. Four patients who received palliative surgery had comparative anastomotic restenosis. The diameters of RV-PA anastomasis, left and right branches of the pulmonary artery during follow-up were significantly larger than early postoperative diameters (P < 0.05). Echocardiography showed that pulmonary artery and tricuspid regurgitation were aggravated, which was not correlated to follow-up duration. Overall right heart function was good during follow-up. ConclusionsClinical outcomes of RV-PA anastomosis for RVOT reconstruction in patients with PA/VSD are satisfactory. RV-PA anastomasis, left and right branches of the pulmonary artery can keep their growth and development. Mid-term and long-term right heart function is good.
Objective To explore the correlation between different ultrasound pulmonary artery systolic pressure (PASP) and high-resolution CT (HRCT) pulmonary artery width (PAD) in patients with chronic obstructive pulmonary disease (COPD). Methods A retrospective analysis was conducted on 473 patients with acute exacerbation of chronic obstructive pulmonary disease who were hospitalized in the First Hospital of Lanzhou University from January 2016 to December 2020. They were divided into four groups according to the degree of PASP elevation: PASP normal group: PASP≤36 mm Hg, 182 cases; mildly elevated group: PASP 37 to 50 mm Hg, 164 cases; moderately elevated group: PASP 51 to 70 mm Hg, 89 cases; severely elevated group: PASP>70 mm Hg, 38 cases. The PAD of chest HRCT and the width of the ascending aorta (AAD) on the same plane were measured, and the ratio of PAD to AAD (PAD/AAD) was calculated. The differences of PAD, AAD, PAD/AAD in different PASP groups of COPD were compared. The correlations between PASP, lung function, blood gas analysis and PAD, PAD/AAD were analyzed. Results With the decrease of FEV1%pred, FVC%pred, FEV1/FVC, PaO2 and SaO2 in the patients, PaCO2 increased, PASP gradually increased, PAD and PAD/AAD gradually increased. PAD and PAD/AAD were significantly different between the severely elevated PASP group and the other three groups, and there were significant differences between the moderately elevated group and the normal group, and between the moderately elevated group and the mildly elevated group. PASP and PaCO2 were positively correlated with PAD and PAD/AAD, and FEV1%pred, FVC%pred, FEV1/FVC, PaO2, SaO2 were negatively correlated with PAD and PAD/AAD. Multivariate logistic regression analysis showed that after adjusting for confounding factors, decreased FEV1%pred was an independent risk factor for PAD/AAD>1 in COPD patients. The receiver operating characteristic curve showed that the width of PAD and PAD/AAD had certain predictive value for PASP. Conclusions There is a significant positive correlation between different degrees of ultrasound PASP and PAD and PAD/AAD in patients with COPD. HRCT PAD has certain predictive value for PASP. The heavier the hypoxia and carbon dioxide retention, the worse the pulmonary ventilation function, the higher the pulmonary artery pressure, the greater the possibility of PAD and PAD/AAD.