BACKGROUND We analyzed the main features of an infant diagnosed with temporary neonatal cyanosis in order to strengthen our understanding of the disease.CASE SUMMARY We report a patient diagnosed with temporary neonat...BACKGROUND We analyzed the main features of an infant diagnosed with temporary neonatal cyanosis in order to strengthen our understanding of the disease.CASE SUMMARY We report a patient diagnosed with temporary neonatal cyanosis.The main clinical characteristics,gene mutation and treatment are discussed and a review of related literature was conducted.The neonate aged 1 d and 5 h was admitted to hospital due to cyanosis after birth.The main clinical manifestation was cyanosis,which was not improved by auxiliary ventilation and the patient showed no obvious shortness of breath or methemoglobinemia.Gene mutation analysis showed a heterozygous c.190C>T mutation in the HBG2 gene associated with transient neonatal cyanosis,which was derived from his mother.Symptomatic supportive treatment was given for 2 mo.The neonate was discharged and gradually improved with oral administration of vitamin C and vitamin B2 for 2 wk.CONCLUSION There is no special treatment for temporary neonatal cyanosis caused by heterozygous mutation of the HBG2 gene.展开更多
Berry Syndrome is a rare combination of congenital cardiac abnormalities firstly reported in 1982.1 It consists of aortopulmonary window, anomalous origin of the right pulmonary artery (RPA) from ascending aorta, in...Berry Syndrome is a rare combination of congenital cardiac abnormalities firstly reported in 1982.1 It consists of aortopulmonary window, anomalous origin of the right pulmonary artery (RPA) from ascending aorta, intact ventricular septum, interruption of the aortic arch with patent ductus arteriosus(PDA). This is the 26th case reported in literature2 and the first report in Hong Kong. Delayed recognition can result in potential lethal condition.展开更多
Objectives:Over the last decade,neonatal repair has been advocated for many congenital heart diseases.However,specific subgroups of complex congenital heart disease still require temporary palliation for which both su...Objectives:Over the last decade,neonatal repair has been advocated for many congenital heart diseases.However,specific subgroups of complex congenital heart disease still require temporary palliation for which both surgical and endovascular techniques are currently available.We reviewed our institutional experience with shunt palliation with an emphasis on risk factors for early mortality.Methods:This is a single-center retrospective study on 175 patients undergoing surgery for central shunt or modified Blalock-Taussig shunt.All data were extracted from a prospectively collected computerized database.We identified risk factors for early mortality by uni-and multi-variable analysis.All data were censored at the time of death or shunt take-down operation.Results:Mean age and weight at surgery were 24 days(IQR[7–95])and 3.4 kg(IQR[2.9–4.8]),respectively,with 96 neonates(55%).Most patients had a biventricular heart disease(115 patients,66%),and 51 patients(29.1%)had univentricular heart disease.Thoracotomy was performed in 129 patients(74%).Cardiopulmonary bypass was used in 23 patients(13%).The median intensive care and overall length of stay were 4 days(IQR[2–9])and 18 days(IQR[13–29]),respectively.In-hospital mortality was 8.6%(15/175).By multivariable regression analysis,prematurity(HR 5.6[2.1–14.7]),CPB use(HR 6.7[2.2–18.6]),unplanned<30-day reoperation(HR 3.5[1.2–10])or catheterization(HR 4.5[1.2–16.9])were all significant predictors of early mortality.Conclusions:Procedural-related mortality remains high(8.6%)in surgical shunt palliation.For patients with prematurity,low weight at birth,or if the use of cardiopulmonary bypass is contemplated,alternative endovascular techniques of palliation should be considered together with longitudinal follow-up studies.展开更多
Objective:Despite increasing enthusiasm for neonatal repair,patients with ductal-dependent circulation(pulmonary/systemic)or restrictive pulmonary blood flow still require initial palliation.Ductal stenting has emerge...Objective:Despite increasing enthusiasm for neonatal repair,patients with ductal-dependent circulation(pulmonary/systemic)or restrictive pulmonary blood flow still require initial palliation.Ductal stenting has emerged as an endovascular approach whereas modified-Blalock-Taussig and central shunt remain surgical references.In this study,we analyzed the relationship between pulmonary artery growth,sites of shunt connection,or antegrade pulmonary blood flow in surgically placed shunts.The need for secondary catheter-based interventions or pulmonary arterioplasty was also investigated.Methods:A retrospective single-center study analyzing 175 patients undergoing surgery for a central or modified-Blalock-Taussig shunt.Outcome growth variables were right pulmonary artery/left pulmonary artery diameters/Z scores,the indexed sum area(right pulmonary artery+left pulmonary artery),and the pulmonary symmetry index.Three imaging modalities were used:angiography,computed tomography,and echocardiography.Results:At baseline,pulmonary arteries were larger in patients with antegrade pulmonary blood flow(Nakata index 137 vs.114,p=0.047)as well as in patients receiving a modified-Blalock-Taussig shunt(Nakata index 138 vs.84,p<0.001).At the time of shunt takedown,both the right pulmonary artery and left pulmonary artery had normalized their diameter.The Nakata index increased from 134 to 233 mm^(2)/m^(2)(p<0.001).The pulmonary artery index remained stable(0.86)over time.During the inter-stage period,shunt-related pulmonary artery stenosis and juxta-ductal stenosis were diagnosed in 16(10%)and 17 patients(11%),respectively.Conclusions:Surgical shunt palliation allows normal pulmonary artery growth.Pulmonary artery stenosis was either shunt-related(10%)or secondary to juxta-ductal stenosis(11%).Close echographic follow-up allows early diagnosis and treatment of juxta-ductal stenosis.展开更多
<span style="font-family:Verdana;">Congenital cyanogenic heart disease (CCHD) is a malformation of the heart and large vessels characterized by an oxygen desaturation in the arterial blood, responsible...<span style="font-family:Verdana;">Congenital cyanogenic heart disease (CCHD) is a malformation of the heart and large vessels characterized by an oxygen desaturation in the arterial blood, responsible for cyanosis. The general objective was to study the profile of CCHD in Senegalese hospitals. This is a retrospective study carried out over a period of 8 years (January 1, 2010 - December 31, 2017) and including all children aged 0 to 16 years followed for a CCHD. The hospital prevalence was 0.87% for 420 cases collected. The sex ratio was 1.44 and the average age at diagnosis was 16 months. First degree parental consanguinity was noted in </span><span><span style="font-family:Verdana;">36 cases (30.78%). The main reasons for consultation were breathing difficult</span><span style="font-family:Verdana;">y</span></span><span> <span style="font-family:Verdana;">in 242 cases (57.62%) and fever in 136 patients (32.36%). Apart from cyanosis, the clinical signs were dominated by the heart murmur in 313 cases (74.7%), tachycardia in 283 cases (67.38%) and digital hippocratism in 162 cases (38.57%). Cardiomegaly was found in 239 patients (83.36%). The main types of CCHD were tetralogy of Fallot and transposition of the large vessels. In biology, 206 patients (49.05%) presented polyglobulia. A complete surgical cure was carried out in 22 patients (5.24%). Complications were anoxic crisis (52 cases) and hemorrhagic syndrome (17 cases). There were 97 deaths (28.28%) during hospitalization. The diagnosis of CCHD is late in our country and surgical management is poor explaining the high mortality</span><span style="font-family:Verdana;">.</span></span>展开更多
Background:Communication between the right pulmonary artery(RPA)and left atrium(LA)is a rare cause of central cyanosis in pediatric patients.Case presentation:We describe a 3-year-old female patient with an oxygen sat...Background:Communication between the right pulmonary artery(RPA)and left atrium(LA)is a rare cause of central cyanosis in pediatric patients.Case presentation:We describe a 3-year-old female patient with an oxygen saturation of 70%at admission.The echocardiogram indicated an abnormal color flow Doppler in the LA and she underwent standard cardiac catheterization.The angiography of pulmonary artery revealed a 7.4 mm×7.6 mm fistula between the RPA and LA and achieved successful closure using ventricular septal defect occlusion.Conclusion:The fistula between pulmonary artery and left atrium is an extremely rare but treatable congenital defect.It should be considered in differential diagnosis of cyanosis in children.展开更多
Background Cyanotic patients have potential growth retardation and malnutrition due to hypoxemia and other reasons. Ghrelin is a novel endogenous growth hormone secretagogue that has effects on growth and cardiovascul...Background Cyanotic patients have potential growth retardation and malnutrition due to hypoxemia and other reasons. Ghrelin is a novel endogenous growth hormone secretagogue that has effects on growth and cardiovascular activities. The aim of this study was to evaluate the plasma level and myocardial expression of ghrelin and insulin-like growth factor-1 (IGF-1) using an immature piglet model of chronic cyanotic congenital heart defects with decreased pulmonary blood flow. Methods Twelve weanling Chinese piglets underwent procedures of main pulmonary artery-left atrium shunt with pulmonary artery banding or sham operation as control. Four weeks later, hemodynamic parameters were measured. Enzyme-linked immunosorbent assay for plasma ghrelin and IGF-1 level measurement were performed. Ventricular ghrelin and IGF-1 mRNA expressions were measured by quantitative real-time polymerase chain reaction. Results Four weeks after surgical procedure, the cyanotic model produced lower arterial oxygen tension ((68.73±15.09) mmHg), arterial oxygen saturation ((82.35±8.63)%), and higher arterial carbon dioxide tension ((51.83±6.12) mmHg), hematocrit ((42.67±3.83)%) and hemoglobin concentration ((138.17±16.73) g/L) than the control piglets ((194.08±98.79) mmHg, (96.43±7.91)%, (36.9±4.73) mmHg, (31.17±3.71)%, (109.83±13.75) g/L) (all P 〈0.05). Plasma ghrelin level was significantly higher in the cyanotic model group in comparison to the control (P=0.004), and the plasma IGF-1 level was significantly lower than control (P=0.030). Compared with control animals, the expression of ghrelin mRNAs in the ventricular myocardium was significantly decreased in the cyanotic model group (P=0.000), and the expression of IGF-1 mRNAs was elevated (P=0.001). Conclusions Chronic cyanotic congenital heart defects model was successfully established. Plasma ghrelin level and myocardial IGF-1 mRNA expression were significantly up-regulated, while plasma iGF-1 level and myocardial ghrelin mRNA expression were down-regulated in the chronic cyanotic immature piglets. The ghrelin system may be an important part of the network regulating cardiac performance.展开更多
Background The present study was aimed to compare the effects of bilateral and unilateral bidirectional superior cavopulmonary shunt (b-BDG and u-BDG) on pulmonary artery growth and clinical outcomes. Methods The 51...Background The present study was aimed to compare the effects of bilateral and unilateral bidirectional superior cavopulmonary shunt (b-BDG and u-BDG) on pulmonary artery growth and clinical outcomes. Methods The 51 subjects enrolled in this study were divided into two groups: those receiving b-BDG (n=21) and those receiving u-BDG (n=30). Clinical records were reviewed retrospectively at a mean of 43.3 months after BDG procedures. Chi square and t-tests were performed to analyze the data. Results Left and right pulmonary artery diameters increased 27%-37% in both groups. The pulmonary artery index increased 37.2% after b-BDG and 27.0% after u-BDG, b-BDG patients experienced a significant decrease in mean hemoglobin concentration and hematocrit value, and a correlated change in postoperative diameter of left pulmonary artery (LPA) and pulmonary artery index (y=0.2719, x=-1.8278; R=0.564, P=-0.008). The change ratio of hemoglobin and postoperative LPA were also correlated in b-BDG patients (y= -0.0522x + 0.3539; R=-0.479, P=-0.028). Only one b-BDG patient versus twelve u-BDG patients needed total cavopulmonary connections 31.8 months after BDG surgery (P=-0.0074). Moreover, only one (4.8%) b-BDG patient but eight u-BDG patients (26.7%) developed pulmonary arteriovenous malformations. Conclusions b-BDG increases bilateral pulmonary blood flow and promotes growth of bilateral pulmonary arteries, with preferable physiological outcomes to u-BDG. Results may imply that subsequent Fontan repair may not always be needed. Chin Med J 2009; 122(2): 129-135展开更多
Background Cyanotic congenital heart defects with decreased pulmonary blood flow due to lung ischemia,hypoxia,and others lead to infant morbidity and mortality more than acyanotic heart disease does.Despite the great ...Background Cyanotic congenital heart defects with decreased pulmonary blood flow due to lung ischemia,hypoxia,and others lead to infant morbidity and mortality more than acyanotic heart disease does.Despite the great effort of medical research,their genetic link and underlying microRNAs molecular mechanisms remain obscure.In this study,we aimed to investigate microRNAs regulation during cyanotic defects in lung of immature piglets.Methods Cyanotic piglet model was induced by main pulmonary artery-left atrium shunt with distal pulmonary artery banding.Four weeks later,hemodynamic parameters confirmed the development of cyanotic defects and pulmonary lobe RNA was extracted from all animals.We studied the repertoire of porcine lung microRNAs by Solexa deep sequencing technology and quantified highly expressed microRNAs by microarray hybridization.Furthermore,we quantitated selected microRNAs from cyanotic and control piglets by quantitative RT-PCR.Results After surgical procedure 4 weeks later,the cyanotic model produced lower arterial oxygen tension,arterial oxygen saturation,and higher arterial carbon dioxide tension,hematocrit and hemoglobin concentration than controls (all P 〈0.05).In 1273 miRNAs expressed in the immature piglets lungs,2 most abundant microRNAs (miR-370 and miR-320) demonstrated significant difference between cyanotic and control group (all P 〈0.05).Conclusion Our results extended lung microRNA profile in immature piglets and suggested that miR-370 and miR-320 are significantly up-regulated in cyanotic lung tissues.展开更多
Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliativ...Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.展开更多
基金Supported by National Natural Science Foundation of China,No.81771626
文摘BACKGROUND We analyzed the main features of an infant diagnosed with temporary neonatal cyanosis in order to strengthen our understanding of the disease.CASE SUMMARY We report a patient diagnosed with temporary neonatal cyanosis.The main clinical characteristics,gene mutation and treatment are discussed and a review of related literature was conducted.The neonate aged 1 d and 5 h was admitted to hospital due to cyanosis after birth.The main clinical manifestation was cyanosis,which was not improved by auxiliary ventilation and the patient showed no obvious shortness of breath or methemoglobinemia.Gene mutation analysis showed a heterozygous c.190C>T mutation in the HBG2 gene associated with transient neonatal cyanosis,which was derived from his mother.Symptomatic supportive treatment was given for 2 mo.The neonate was discharged and gradually improved with oral administration of vitamin C and vitamin B2 for 2 wk.CONCLUSION There is no special treatment for temporary neonatal cyanosis caused by heterozygous mutation of the HBG2 gene.
文摘Berry Syndrome is a rare combination of congenital cardiac abnormalities firstly reported in 1982.1 It consists of aortopulmonary window, anomalous origin of the right pulmonary artery (RPA) from ascending aorta, intact ventricular septum, interruption of the aortic arch with patent ductus arteriosus(PDA). This is the 26th case reported in literature2 and the first report in Hong Kong. Delayed recognition can result in potential lethal condition.
文摘Objectives:Over the last decade,neonatal repair has been advocated for many congenital heart diseases.However,specific subgroups of complex congenital heart disease still require temporary palliation for which both surgical and endovascular techniques are currently available.We reviewed our institutional experience with shunt palliation with an emphasis on risk factors for early mortality.Methods:This is a single-center retrospective study on 175 patients undergoing surgery for central shunt or modified Blalock-Taussig shunt.All data were extracted from a prospectively collected computerized database.We identified risk factors for early mortality by uni-and multi-variable analysis.All data were censored at the time of death or shunt take-down operation.Results:Mean age and weight at surgery were 24 days(IQR[7–95])and 3.4 kg(IQR[2.9–4.8]),respectively,with 96 neonates(55%).Most patients had a biventricular heart disease(115 patients,66%),and 51 patients(29.1%)had univentricular heart disease.Thoracotomy was performed in 129 patients(74%).Cardiopulmonary bypass was used in 23 patients(13%).The median intensive care and overall length of stay were 4 days(IQR[2–9])and 18 days(IQR[13–29]),respectively.In-hospital mortality was 8.6%(15/175).By multivariable regression analysis,prematurity(HR 5.6[2.1–14.7]),CPB use(HR 6.7[2.2–18.6]),unplanned<30-day reoperation(HR 3.5[1.2–10])or catheterization(HR 4.5[1.2–16.9])were all significant predictors of early mortality.Conclusions:Procedural-related mortality remains high(8.6%)in surgical shunt palliation.For patients with prematurity,low weight at birth,or if the use of cardiopulmonary bypass is contemplated,alternative endovascular techniques of palliation should be considered together with longitudinal follow-up studies.
文摘Objective:Despite increasing enthusiasm for neonatal repair,patients with ductal-dependent circulation(pulmonary/systemic)or restrictive pulmonary blood flow still require initial palliation.Ductal stenting has emerged as an endovascular approach whereas modified-Blalock-Taussig and central shunt remain surgical references.In this study,we analyzed the relationship between pulmonary artery growth,sites of shunt connection,or antegrade pulmonary blood flow in surgically placed shunts.The need for secondary catheter-based interventions or pulmonary arterioplasty was also investigated.Methods:A retrospective single-center study analyzing 175 patients undergoing surgery for a central or modified-Blalock-Taussig shunt.Outcome growth variables were right pulmonary artery/left pulmonary artery diameters/Z scores,the indexed sum area(right pulmonary artery+left pulmonary artery),and the pulmonary symmetry index.Three imaging modalities were used:angiography,computed tomography,and echocardiography.Results:At baseline,pulmonary arteries were larger in patients with antegrade pulmonary blood flow(Nakata index 137 vs.114,p=0.047)as well as in patients receiving a modified-Blalock-Taussig shunt(Nakata index 138 vs.84,p<0.001).At the time of shunt takedown,both the right pulmonary artery and left pulmonary artery had normalized their diameter.The Nakata index increased from 134 to 233 mm^(2)/m^(2)(p<0.001).The pulmonary artery index remained stable(0.86)over time.During the inter-stage period,shunt-related pulmonary artery stenosis and juxta-ductal stenosis were diagnosed in 16(10%)and 17 patients(11%),respectively.Conclusions:Surgical shunt palliation allows normal pulmonary artery growth.Pulmonary artery stenosis was either shunt-related(10%)or secondary to juxta-ductal stenosis(11%).Close echographic follow-up allows early diagnosis and treatment of juxta-ductal stenosis.
文摘<span style="font-family:Verdana;">Congenital cyanogenic heart disease (CCHD) is a malformation of the heart and large vessels characterized by an oxygen desaturation in the arterial blood, responsible for cyanosis. The general objective was to study the profile of CCHD in Senegalese hospitals. This is a retrospective study carried out over a period of 8 years (January 1, 2010 - December 31, 2017) and including all children aged 0 to 16 years followed for a CCHD. The hospital prevalence was 0.87% for 420 cases collected. The sex ratio was 1.44 and the average age at diagnosis was 16 months. First degree parental consanguinity was noted in </span><span><span style="font-family:Verdana;">36 cases (30.78%). The main reasons for consultation were breathing difficult</span><span style="font-family:Verdana;">y</span></span><span> <span style="font-family:Verdana;">in 242 cases (57.62%) and fever in 136 patients (32.36%). Apart from cyanosis, the clinical signs were dominated by the heart murmur in 313 cases (74.7%), tachycardia in 283 cases (67.38%) and digital hippocratism in 162 cases (38.57%). Cardiomegaly was found in 239 patients (83.36%). The main types of CCHD were tetralogy of Fallot and transposition of the large vessels. In biology, 206 patients (49.05%) presented polyglobulia. A complete surgical cure was carried out in 22 patients (5.24%). Complications were anoxic crisis (52 cases) and hemorrhagic syndrome (17 cases). There were 97 deaths (28.28%) during hospitalization. The diagnosis of CCHD is late in our country and surgical management is poor explaining the high mortality</span><span style="font-family:Verdana;">.</span></span>
基金This work was supported by a grant from the National Science Foundation of China[Grant No.81700286].
文摘Background:Communication between the right pulmonary artery(RPA)and left atrium(LA)is a rare cause of central cyanosis in pediatric patients.Case presentation:We describe a 3-year-old female patient with an oxygen saturation of 70%at admission.The echocardiogram indicated an abnormal color flow Doppler in the LA and she underwent standard cardiac catheterization.The angiography of pulmonary artery revealed a 7.4 mm×7.6 mm fistula between the RPA and LA and achieved successful closure using ventricular septal defect occlusion.Conclusion:The fistula between pulmonary artery and left atrium is an extremely rare but treatable congenital defect.It should be considered in differential diagnosis of cyanosis in children.
基金This work was supported by a grant from the National Natural Science Foundation of China (No. 30972958).
文摘Background Cyanotic patients have potential growth retardation and malnutrition due to hypoxemia and other reasons. Ghrelin is a novel endogenous growth hormone secretagogue that has effects on growth and cardiovascular activities. The aim of this study was to evaluate the plasma level and myocardial expression of ghrelin and insulin-like growth factor-1 (IGF-1) using an immature piglet model of chronic cyanotic congenital heart defects with decreased pulmonary blood flow. Methods Twelve weanling Chinese piglets underwent procedures of main pulmonary artery-left atrium shunt with pulmonary artery banding or sham operation as control. Four weeks later, hemodynamic parameters were measured. Enzyme-linked immunosorbent assay for plasma ghrelin and IGF-1 level measurement were performed. Ventricular ghrelin and IGF-1 mRNA expressions were measured by quantitative real-time polymerase chain reaction. Results Four weeks after surgical procedure, the cyanotic model produced lower arterial oxygen tension ((68.73±15.09) mmHg), arterial oxygen saturation ((82.35±8.63)%), and higher arterial carbon dioxide tension ((51.83±6.12) mmHg), hematocrit ((42.67±3.83)%) and hemoglobin concentration ((138.17±16.73) g/L) than the control piglets ((194.08±98.79) mmHg, (96.43±7.91)%, (36.9±4.73) mmHg, (31.17±3.71)%, (109.83±13.75) g/L) (all P 〈0.05). Plasma ghrelin level was significantly higher in the cyanotic model group in comparison to the control (P=0.004), and the plasma IGF-1 level was significantly lower than control (P=0.030). Compared with control animals, the expression of ghrelin mRNAs in the ventricular myocardium was significantly decreased in the cyanotic model group (P=0.000), and the expression of IGF-1 mRNAs was elevated (P=0.001). Conclusions Chronic cyanotic congenital heart defects model was successfully established. Plasma ghrelin level and myocardial IGF-1 mRNA expression were significantly up-regulated, while plasma iGF-1 level and myocardial ghrelin mRNA expression were down-regulated in the chronic cyanotic immature piglets. The ghrelin system may be an important part of the network regulating cardiac performance.
基金This work was supported by a grant from Beijing Natural Science Foundation (No. 7072049).
文摘Background The present study was aimed to compare the effects of bilateral and unilateral bidirectional superior cavopulmonary shunt (b-BDG and u-BDG) on pulmonary artery growth and clinical outcomes. Methods The 51 subjects enrolled in this study were divided into two groups: those receiving b-BDG (n=21) and those receiving u-BDG (n=30). Clinical records were reviewed retrospectively at a mean of 43.3 months after BDG procedures. Chi square and t-tests were performed to analyze the data. Results Left and right pulmonary artery diameters increased 27%-37% in both groups. The pulmonary artery index increased 37.2% after b-BDG and 27.0% after u-BDG, b-BDG patients experienced a significant decrease in mean hemoglobin concentration and hematocrit value, and a correlated change in postoperative diameter of left pulmonary artery (LPA) and pulmonary artery index (y=0.2719, x=-1.8278; R=0.564, P=-0.008). The change ratio of hemoglobin and postoperative LPA were also correlated in b-BDG patients (y= -0.0522x + 0.3539; R=-0.479, P=-0.028). Only one b-BDG patient versus twelve u-BDG patients needed total cavopulmonary connections 31.8 months after BDG surgery (P=-0.0074). Moreover, only one (4.8%) b-BDG patient but eight u-BDG patients (26.7%) developed pulmonary arteriovenous malformations. Conclusions b-BDG increases bilateral pulmonary blood flow and promotes growth of bilateral pulmonary arteries, with preferable physiological outcomes to u-BDG. Results may imply that subsequent Fontan repair may not always be needed. Chin Med J 2009; 122(2): 129-135
基金This study was supported by grants from the National Natural Science Foundation of China (No. 81100116), the Beijing Natural Science Foundation (No. 7112046), and Capital Citizens' Health Project Cultivation of Beijing Municipal Science and Technology Commissions (No. Z 111100074911001).Acknowledgments: The authors are grateful to Dr. LI Gang and Dr. XU Yu-lin for the animal experiment's assistance, and ZHANG Yanbin and ZHANG Shao-bin for their bioinformatics assistance.
文摘Background Cyanotic congenital heart defects with decreased pulmonary blood flow due to lung ischemia,hypoxia,and others lead to infant morbidity and mortality more than acyanotic heart disease does.Despite the great effort of medical research,their genetic link and underlying microRNAs molecular mechanisms remain obscure.In this study,we aimed to investigate microRNAs regulation during cyanotic defects in lung of immature piglets.Methods Cyanotic piglet model was induced by main pulmonary artery-left atrium shunt with distal pulmonary artery banding.Four weeks later,hemodynamic parameters confirmed the development of cyanotic defects and pulmonary lobe RNA was extracted from all animals.We studied the repertoire of porcine lung microRNAs by Solexa deep sequencing technology and quantified highly expressed microRNAs by microarray hybridization.Furthermore,we quantitated selected microRNAs from cyanotic and control piglets by quantitative RT-PCR.Results After surgical procedure 4 weeks later,the cyanotic model produced lower arterial oxygen tension,arterial oxygen saturation,and higher arterial carbon dioxide tension,hematocrit and hemoglobin concentration than controls (all P 〈0.05).In 1273 miRNAs expressed in the immature piglets lungs,2 most abundant microRNAs (miR-370 and miR-320) demonstrated significant difference between cyanotic and control group (all P 〈0.05).Conclusion Our results extended lung microRNA profile in immature piglets and suggested that miR-370 and miR-320 are significantly up-regulated in cyanotic lung tissues.
基金supported by Medical Scientific Research Foundation of Guangdong Province,China (No.A2018038)
文摘Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.