ObjectiveTo summarize our experience of surgical treatment for right pulmonary artery originated from the ascending aorta by using adult diameter artificial vascular and study the operative indication, design, method, and therapeutic efficacy. MethodsWe retrospectively analyzed clinical data of 11 patients with right pulmonary artery originated from ascending aorta in The Second Affiliated Hospital of Harbin Medical University from May 2008 through December 2013, who were treated by using adult diameter artificial vascular. The patients ranged from 4 months to 25 months old, weighted 4-15 kg. Among of them, 4 patients had persistent truncus arteriosus and 7 had aortopulmonary septal defect. All patients were complicated with moderate pulmonary hypertension. All the patients underwent one stage surgical repair under extracorporeal circulation and cardiac arrest. During the surgery, end to side anastomosis was done between the right pulmonary artery and 16-18 mm diameter artificial blood vessels. And artificial blood vessel was connected to the main pulmonary artery or right ventricle outflow tract incision from the aorta above. ResultsThe average operation time was 179-325 (224±68) min. The average cardiopulmonary bypass (CPB) time was 81-208 (117±54) min. The average aortic clamping time was 29-63(42±21) min. The mean residence time in ICU was 71-197 (109±42) hours. The average assisted mechanical ventilator time was 59-191 (91±26) hours. The average length of stay in hospital was 21-39 (28±11) days. Low cardiac output syndromes caused by pulmonary arterial hypertension occurred in 5 patients including 2 deaths and 3 patients with good recovery by reducing the pulmonary arterial pressure and peritoneal dialysis. The result of postoperative cardiac color ultrasound examination of 9 survival patients showed vascular prosthesis, no distortion, no stenosis of the anastomosis, deformity correction satisfaction. Nine patients were followed up for 3-60 months. The results of echocardiography showed no anastomosis and artificial vascular stenosis, and the pulmonary arterial pressure decreased significantly. ConclusionThe right pulmonary artery originated from the ascending aorta in children should be operated as soon as possible. Compared the adult diameter artificial vascular treatment for one stage repair of right pulmonary artery from the ascending aorta with other operation methods, both short-term and long-term effects are good. Postoperative low cardiac output syndrome is a common complication.
ObjectiveTo investigate the effect and incidence of residual leakage after surgical repair of large ventricular septal defects with moderate to severe pulmonary hypertension using cross-linkage mattress suture, a suture method invented by us, as compared with interrupted mattress suture. MethodsWe retrospectively analyzed the clinical data of 41 patients of large ventricular septal defect with moderate to severe pulmonary hypertension underwent surgery using cross-linkage mattress suture in Beijing Anzhen Hospital from February 2011 through April 2013. The 41 patients were as a cross-linkage group (average age 18.7±12.3 years, the ratio of male to female 31:10). Another 41 patients, who were repaired using interrupted mattress suture, were retrospectively chosen by matching age, size and location of the defects, pulmonary artery pressure and vascular resistance with members from the cross-linkage group, and were assigned as a control group (average age 17.4±11.8 years, the ratio of male to female 31:10). ResultsThere was no operative mortality and no new perioperative atrioventricular conduction block. Postoperative echocardiography revealed the incidence of residual leakage was 31.7% (13/41) in the control group, 0% (0/41) in the cross-linkage group with a statistical difference (χ2=13.164, P=0.000). With a follow-up of 18.2±6.1 months, no late death and no new atrioventricular conduction block occurred. There was no statistical difference in New York Heart Association functional class or pulmonary artery pressure measured through echocardiography between the two groups. While there was a statistical difference in incidence of residual leakage between the the cross-linkage group and the control group (0% (0/41) versus 26.8% (11/41), χ2=10.499, P=0.001). ConclusionThere is a high incidence of residual leakage after the surgical repair of large ventricular septal defect complicated with moderate to severe pulmonary hypertension using interrupted mattress suture, while the use of cross-linkage mattress suture can effectively reduce the incidence of residual leakage.
Pulmonary arterial hypertension is a kind of intractable disease which threatens human health severely. The results of operation are unsatisfactory. Clinical drug therapy is the major treatment which aims to relieve symptoms, improve the quality of life, and prevent the disease from progressing. Over the last several years, the studies of stem cells provide a new direction for the treatment of pulmonary arterial hypertension. It's demonstrated that the therapeutic effects of stem cells are better than that of the traditional methods. With the deepening of the researches, the therapy of stem cells is more and more compelling. The therapy of stem cells for pulmonary arterial hypertension is reviewed in this paper.
Objective To make an individualized treatment plan concerning a newborn with meconium aspiration syndrome combined with persistent pulmonary hypertension. Methods Based on the clinical questions raised by a newborn with meconium aspiration syndrome combined with persistent pulmonary hypertension, we searched The Cochrane Library (Issue 3, 2009), MEDLINE (1980 to June 2009), ACP Journal Club (1991 to June 2009), and Chinese Journal Fulltext Database (1994 to June 2009) for systematic reviews, randomized controlled trials (RCTs) and case-control studies. The quality of the included studies was assessed. Results A total of 9 RCTs, 1 health economic evaluation, 1 meta analysis, and 2 systematic reviews were considered eligible. The evidence indicated that the use of ECMO in infants of PPHN had shown a decreased risk of death, but not cost-saving from a societal perspective; iNO treatment could improve the PaO2 and resulted in a reduction in the incidence of requirement for ECMO; there were not randomized controlled trials regarding the treatment of PPHN by hyperventilation, high-frequency ventilation, alkali infusion, pulmonary vasodilators (magnesium sulfate, tolazoline, prostaglandin or prostacyclin, milrinone), surfactant therapy; oral sildenafil could lower oxygenation index (OI) and result in a reduction in the incidence of death. The individualized treatment plans of oral sildenafil were developed based on the available evidence, existing conditions of the hospital, and the values of children with families. After 1 month of treatment, the FiO2 returned to normal and symptoms were alleviated. Conclusion The treatment efficacies and the survival rates in meconium aspiration syndrome combined with PPHN have been improved by determining an individualized treatment plan according to evidence-based methods.
Objective To investigate the preventive effect of simvastatin，a 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitor，on hypoxic pulmonary hypertension and the relation between it and the angiotensin Ⅱ receptor-1（AT1R） expression in pulmonary arteriole.Methods Thirty male Sprague-Drawley rats were randomly allocated into three groups：a control group，a hypoxic group and a simvastatin preventive group.The animal model of hypoxic pulmonary hypertension was established by exposing the rats to normobaric hypoxic condition（8 h×6 d×3 w），and the preventive group were treated with simvastatin 10 mg/kg before hypoxic processing while the control and hypoxic groups were treated with sodium chloride.The mean pulmonary pressure（mPAP），serum cholesterol concentration，right ventricular hypertrophy index [RV/（LV+S）]，percentage of the wall thickness in the external diameter（WT%），percentage of the wall area in the total vascular area（WA%），and the AT1R expression in pulmonary arterioles were measured.Results When compared with the hypoxic group，in the preventive group，the mPAP and RV/（LV+S）obviously reduced [（22.6±3.86）mm Hg vs （29.3±2.27）mm Hg，（25.13±0.75）% vs （33.18±1.58）%，Plt;0.01 respectively]，the indices of wall thickness of rat pulmonary arteriole and area also decreased significantly [WT%：（15.98±1.96）% vs （25.14±1.85）%；WA%：（54.60±3.94）% vs 74.77±4.52）%；Plt;0.01 respectively]，and the positive degree of AT1R still lessened noticeably（1.23±0.09 vs 1.57±0.13，Plt;0.01）.All of the indices above in the hypoxic group increased markedly compared with the control group（Plt;0.01 respectively）.However，the differences of serum cholesterol among three groups were not significant（Pgt;0.05）.Conclusions Simvastatin can suppress the expression of AT1R in pulmonary vessel and prevent hypoxic pulmonary hypertension.
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 investigate the clinical characteristics and treatment for Type II pulmonary vascular anomaly ( pulmonary arteriovenous malformations) of hepatopulmonary syndrome ( HPS)presenting with hemothorax. Methods A case of Type II pulmonary vascular anomaly of HPS presenting with recurrent hemothorax was described. The clinical data was analyzed and the related literature was reviewed. Results A 72-year-old male patient with Type II pulmonary vascular dilatations of HPS was described to present with recurrent dyspnea and encapsulated pleural effusions. After 4 procedures of thoracentesis, a total of 2510 mL of bloody pleural effusions was drained. The routine analysis of pleural fluid showed the count of red cells exceeded 100 ×109 / L, whereas cytologic examination and tumor biomarkers were negative. Then CTPA and pulmonary angiogramrevealed a Type II pulmonary vascular anomaly of HPS combined with hemothorax. The PaO2 of arterial blood in upright and supine position was 58. 3 mm Hg and 66. 3 mm Hg, respectively. Hypoxemia was alleviated and hemothorax was controlled after embolization of malformed blood vessels. Fromliterature review, similar cases of hemothorax resulted fromrupture of Type II pulmonary vascular anomaly of HPS were not reported. The primary clinical manifestations of HPS were dyspnea and cyanosis. Orthodeoxia and platypnea were most consistent with HPS. The best screening tool for hypoxemia in patients with HPS was P( A-a) O2. The characteristic findings of HPS on chest CT was a lesion or reticulonodular opacities occurring predominantly in the bases of the lungs, which could be enhanced by contrast medium. Pulmonary angiogram was necessary to identify the types of pulmonary vascular dilatations. Hepoxemia of patients with Type II HPS often responded poorly to oxygen therapy, whereas embolization of the pulmonary arteriovenous fistulas was helpful to improve anoxia. Conclusions Rupture ofType II pulmonary vascular malformations in HPS was a rare cause of hemothorax. Thrombosis of pulmonary arteriovenous malformations may result in significant improvement in oxygen saturations as well as control of hemothorax. In the setting of liver disease, intrapulmonary vascular dilatations and hypoxemia often suggestthe existence of HPS.
Objective To investigate the dynamic expression of small ubiquitin-related modifiers-1 ( SUMO-1) in lung tissue in different phases of rat model of hypoxic pulmonary hypertension( HPH) .Methods Forty Wistar rats were randomly divided into 5 groups, and exposed to normoxia or to normobaric intermittent hypoxia for 3, 7, 14 or 21 days, respectively. Mean pulmonary arterial pressure( mPAP) , right ventricle hypertrophy index ( RVHI) , and the ratio of the vessel wall area to the total area( WA% ) weremeasured. RT-PCR and in situ hybridization were used to determine the mRNA expression of SUMO-1.Immunohistochemistry and Western blot were used to determine the protein expression of SUMO-1. Results The hypoxic rats developed pulmonary vascular remodeling in pulmonary arterioles after 7 days of hypoxia,with WA% and mPAP significantly higher than those in the normal control. Pulmonary vascular remodeling aggravated with much higherWA% and mPAP afer 14 days of hypoxia, and reached the peak afer 21 days of hypoxia. SUMO-1 mRNA and protein expression markedly increased after 3 days of hypoxia, and reached peak after 14 days. After 21 days of hypoxia, SUMO-1 mRNA expression weakened but still higher than that in the normal control ( P lt; 0. 05) , and SUMO-1 protein expression remained stable. SUMO-1 mRNA and protein expression were positively correlated with mPAP, WA% and RVHI( all P lt; 0. 01) . Conclusion SUMO-1 is transcriptionally induced in lung tissue under chronic hypoxia, and thus involves in the pathogenesis of HPH.