Objective To investigate the diagnosis and treatment of pulmonary arterial hypertension ( PAH) due to rare causes. Methods The clinical presentation, laboratory testing, diagnosis and treatment of 4 patients with PAH associated with rare causes in Beijing Anzhen Hospital from January 2001 to March 2008 were analysed retrospectively. Results Primary biliary cirrhosis, hyperthyroidism, antiphospholipid syndrome and pulmonary artery sarcoma may cause PAH, which were improved after corresponding diagnosis and management. Conclusion PAH can result from rare causes. The enhancement of its recognition will help earlier diagnosis and treatment and improve the prognosis.
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 investigate the role of Kv1. 5 in the pathogenesis of pulmonary hypertension simulated by hypobaria and hypoxia, and the effects of dichloroacetate ( DCA) on the Kv1. 5 expression in pulmonary arterial smooth muscle cells ( PASMCs ) and mean pulmonary arterial pressure ( mPAP) . Methods Twenty-four SD rats were randomly divided into a normal group ( N group) , a high altitude group ( HA group) , and a DCA treated group ( DCA group) . The N group were fed in normalconditions, the HA group and DCA group were fed in a hypobaria and hypoxia chamber simulated to an altitude of 5000 meters. In addition, the DCA group rats were gastric gavaged with DCA ( 70 mg · kg - 1 · d - 1 ) .Twenty-one days later, percentage of wall thickness ( WT% ) and percentage of wall area ( WA% ) of the pulmonary arteriole, mPAP, and the ratio of right ventricle / left ventricle and septum ( RV/ LV + S) were evaluated. Real-time PCR, immunohistochemistry and Western blot were carried out to detect the Kv1. 5 expression in PASMCs. Results In the HA group, WT% , and WA% of pulmonary arteriole, mPAP and RV/ ( LV + S) all increased significantly compared with the N group ( P lt;0. 01) . These changes in the DCA group were significantly lower than those in the HA group( P lt; 0. 01) . Furthermore, the protein and mRNA expression of Kv1. 5 in the PASMCs deceased significantly in the HA group compared with the N group( P lt;0. 01) , but recovered in the DCA group ( P lt;0. 01) . Conclusions The expression of Kv1. 5 in PASMCs is tremendously inhibited in rats fed in high altitude, which might be a important role of pulmonaryhypertension. DCA can inhibit the remodeling of pulmonary arterials probably by recovering Kv1. 5 expression.
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.
Objective To observe the influence of edaravone perfusion via the pulmonary artery on postoperative lung tissue and lung function during pulmonary ischemia in deep hypothermic circulatory arrest （DHCA）， and explore its possible mechanism. Methods A total of 24 healthy New Zealand white big-ear rabbits were randomly divided into three groups： （1） control group： DHCA model under cardiopulmonary bypass （CPB） was established； （2）low potassium dextran （LPD）group： LPD solution perfusion via the pulmonary artery after the establishment of DHCA； （3）edaravone group：LPD solution containing edaravone （5 mg/kg） perfusion via the pulmonary artery after the establishment of DHCA. Oxygenation index and lung compliance were observed at the time of baseline condition， recovery of ventilation， 1 hour and 2 hours after recovery of ventilation， and postoperative lung function of the three groups were compared. Malondialdehyde （MDA） and superoxide dismutase （SOD） in pulmonary venous blood were measured. All the rabbits were sacrificed after the operation. HE staining and immunohistochemistry were performed on the lung tissues to investigate lung structure changes and inflammatory reaction. Transmission electron microscopy was used to compare ultrastructural changes of lung.Results There were no statistical difference in oxygenation index， lung compliance， MDA and SOD among the 3 groups under the baseline condition （P＞0.05）. After recovery of ventilation， oxygenation index and lung compliance deteriorated to varying degrees in all 3 groups. Oxygenation index and lung compliance of the control group and LPD group at the time of recovery of ventilation， 1 hour and 2 hours after recovery of ventilation were significantly lower than those of edaravone group （oxygenation index：recovery of ventilation and in control group and edaravone group: 198.25±11.02 mm Hg vs. 244.87±13.05 mm Hg；lung compliance：one hour after recovery ventilation in control group and edaravone group：45.88±1.64 ml/cm H2O vs. 59.75±2.38 ml/cm H2O；P＜0.05）. After CPB removal， MDA levels were increased to varying degrees in all 3 groups. MDA levels of the control group and LPD group at the time of CPB removal， 1 hour and 2 hours after CPB removal were significantly higher than those of edaravone group （P＜0.05）. After CPB removal， SOD levels were decreased to varying degrees in all 3 groups. SOD levels of the control group and LPD group at the time of CPB removal， 1 hour and 2 hours after CPB removal were significantly lower than those of edaravone group （P＜0.05）. HE staining showed clear lung structure， less red blood cell leakage， less inflammatory cell infiltration， and less alveolar fluid accumulation in the edaravone group. Immunohistochemistry showed that integral light density of interleukin 6 （IL-6）in edaravone group was significantly lower than those of the LPD group and control group （14.44±1.75 vs. 20.18±2.22， P＜0.05）. Transmission electron microscopy showed integral basement membrane structure， clear blood gas barrier structure， significantly larger number of type II epithelial cells， abundant but not swollen mitochondria and lamellar bodies in the cytoplasm in the edaravone group， which were destroyed in varying degrees in the LPD group and control group. Conclusion Pulmonary artery perfusion of protective solution in low temperature can significantly reduce lung injury induced by DHCA and CPB. Protective solution containing edaravone in low temperature can better decrease lung injury and protect oxygenation.
Objective To evaluate the clinical value of computer tomography angiography( CTA) in the diagnosis of anomalous pulmonary artery. Methods Clinical data of 9 patients with anomalous pulmonary artery diagnosed by CTA were retrospectively analyzed, and CTA results were compared with those of echocardiographic examination. Results CTA clearly demonstrated the origin sites and topography of anomalous pulmonary arteries in all 9 patients. One patients presented with left pulmonary artery sling, and 8 patients presented with anomalous right pulmonary artery which originated fromthe proximal ascending aorta. Among them1 patient was with isolated anomalous origin of right pulmonary, and the others were complicated with patent ductus artery( 5 cases) , ventricular septal defect ( 3 cases) , and aortopulmonary septal defect ( 1 case) , etc. Conclusions CTA is highly valuable in the diagnosis of anomalous origin of pulmonary artery and associated abnormities by clearly developing the pathologic anatomic features and complicated cardiovascular malformations
Abstract： Objective To evaluate clinical outcomes of pulmonary artery banding for morphologic left ventricular training in corrected transposition of the great arteries.?Methods?A total of 89 patients with corrected transposition of the great arteries underwent surgical repair in Shanghai Children’s Medical Center from January 2007 to December 2011 year. Among them, 11 patients underwent pulmonary artery banding, whose clinical records were retrospectively analyzed. Except that one patient was 12 years, all other patients were 3 to 42 （16.40±11.67） months old and had a body weight of 6 to 32 （11.70±7.20）kg. All the patients were diagnosed by echocardiogram and angiocardiogram.?Results?There was no postoperative death after pulmonary artery banding in 11 patients. The pulmonary arterial pressure/systemic blood pressure ratio （Pp/Ps） was 0.3 to 0.6 （0.44±0.09） preoperatively and 0.6 to 0.8 （0.70±0.04） postoperatively with statistical difference （P＜0.01）. Tricuspid regurgitation was mild in 2 （18.2%） patients, moderate in 5 （45.4%）, severe in 4 （36.4%）preoperatively，and none in 2（18.2%）patients, mild in 7 （63.6%），and mild to moderate in 2 （18.2%）postoperatively. Five patients underwent staged double-switch operation after pulmonary artery banding at 15.20±8.31 months, and 1 patient died. The other 6 patients were followed up for 18.83±3.43 months, and echocardiogram showed tricuspid regurgitation as trivial in 2 （33.3%）, mild in 3 （50.0%）, and moderate in 1 （16.7%）patient.?Conclusions?In patients with corrected transposition of the great arteries, pulmonary artery banding is helpful to reduce tricuspid regurgitation, and morphologic left ventricle can be trained for staged double-switch operation with good clinical outcomes. It is important to follow up these patients regularly to evaluate their morphologic left ventricular function and tricuspid regurgitation after pulmonary artery banding.
Objective To summarize our clinical experience of pulmonary artery banding （PAB） for the treatment of complex congenital heart diseases as a palliative procedure.?Methods?From January 1997 to November 2010, 138 patients with complex congenital heart diseases underwent PAB in Fu Wai Hospital. There were 87 male patients and 51 female patients with their age of 22.2±26.5 months and average body weight of 7.5±4.6 kg. All the 138 patients were divided into 3 groups according to the purpose of PAB：left ventricular retraining （group 1, n=55）, initial procedure for functional single ventricle with unobstructed pulmonary blood flow （group 2, n=32） and initial palliative procedure followed by later biventricular repair （group 3, n=51）. The intraoperative and postoperative clinical parameters of all participants were observed, and follow-up was made for these 3 groups of patients.?Results?The in-hospital mortality of PAB was 5.1% （7/138）. Three patients underwent re-banding procedure to adjust the size of banding. In group 1, there was 1 postoperative death. Among the 55 patients, 36 patients with dextro-transposition of great arteries received PAB at an average age of 19.6±29.5 months, 29 patients of whom underwent concomitant modified Blalock-Taussig shunt. After an average training interval for 42 days, 83.3% of them（30/36）successfully received arterial switch operation. The other 19 patients in group 1 with isolated corrected transposition of great arteries received PAB at an average age of 45.3±27.2 months. Afteran average training interval for 9 months, 42.1% of them （8/19） successfully received double-switch operation. In group 2, there was 2 postoperative death. Thirty-two patients with functional single ventricle and unobstructed pulmonary blood flow received PAB at an average age of 14.1±14.9 months. Their postoperative mean pulmonary artery pressure decreased significantly from 34.00±10.00 mm Hg to 23.00±7.40 mm Hg, and their oxygen saturation of blood （SpO2） significantly decreased from 92.60%±5.90% to 83.30%±6.30%. After a median interval of 2 years, 18.8% of them （6/32） underwent right heart bypass operation. In group 3, there was 4 postoperative death. Fifty-one patients received PAB at an average age of 20.60±25.60 months. After PAB procedure, the ratio of systolic pulmonary artery pressure and systolic blood pressure significantly decreased from 0.81±0.14 to 0.46±0.15, and their SpO2 significantly decreased from 93.10%±7.60% to 85.00%±10.00%. After a median interval of 6 months, 23.5% of them （12/51） received biventricular repair.?ConclusionAlthough PAB is a palliative procedure with comparatively high risks, it still plays an indispensable role in terms of protecting pulmonary vascular beds, retraining ventricular function and two-stage surgical correction for the treatment of complex congenital heart diseases.
Abstract: Objective To summarize the clinical experiences of treatment of pulmonary artery dissection, in order to improve the diagnosis and treatment of the disease. Methods We retrospectively analyzed the clinical data of 4 patients including 3 males and 1 female with pulmonary artery dissection who were treated in Fu Wai Hospital between October 1996 and May 2009. Their age ranged from 17 to 45 years with an average age of 31 years. One patient with pulmonary artery dissection with aortic root aneurysm and chronic type Ⅱ aortic dissection was treated with Bentall’s surgery, total arch replacement and pulmonary artery angioplasty under deep hypothermic circulatory arrest. One patient with pulmonary artery dissection with patent ductus arteriosus (PDA) and severe pulmonary hypertension after a failed PDA transcatheter closure was treated with conservative strategy. For the one patient with pulmonary artery dissection with ventricular septal defect (VSD) and severe pulmonary arterial hypertension, VSD was repaired under cardiopulmonary bypass while left pulmonary artery dissection was left untreated. And conservative treatment was carried out for another patient with pulmonary artery dissection with VSD, PDA and Eisenmenger’s syndrome. Results Three patients recovered and 1 patient died of acute pericardial tamponade due to rupture of the dissection within 60 hours after onset of dissection. Followup was done in 3 cases with 1 lost. The followup time was 3 months and 4 years respectively. The New York Heart Association was class Ⅰ and Ⅱ. Conclusion Dissection of the pulmonary artery is a rare disease with a tendency of rupture and bad prognosis. Symptoms of pulmonary artery dissection are nonspecific, which can cause missed diagnosis. In patients with chronic pulmonary hypertension, the sudden seizure of chest pain, exertional dyspnea, and cyanosis, or worsened hemodynamics and cardiac shock may indicate pulmonary artery dissection which can be easily detected with echocardiography, CT scan and magnetic resonance imaging. Corrective surgery or conservative treatment can prevent rupture and possible death based on different causes of pulmonary artery dissection.
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.