Objectives: Hypoglycemia is a recognized danger in pediatric patients. Extended period of preoperative fasting in this subset of patients is not well tolerated with metabolic derangements. The oral carbohydrate loadin...Objectives: Hypoglycemia is a recognized danger in pediatric patients. Extended period of preoperative fasting in this subset of patients is not well tolerated with metabolic derangements. The oral carbohydrate loading preoperatively can ameliorate many adverse effects. The aim of this study was to compare the glycemic profile in pediatric cardiac surgical patients kept fasting preoperatively with those fed oral clear solutions of carbohydrate half hour prior to induction of anaesthesia. Also we tried to establish a correlation with other factors contributing to preoperative hypoglycemia. Methodology: We planned a randomized controlled study. Group A included patients who were kept fasting according to the ASA guidelines preoperatively and Group B included patients who received 2 ml per kg of body weight of 10% Dextrose water as oral feeds half hour before the expected time of start of anaesthesia. Results: The mean (SD) preoperative BG concentrations were higher in group B (102.5 ±16.97) as compared to group A (64.08 ± 25.37) (p value -0.86 and -0.67) (pvalue Conclusion: Preoperative oral carbohydrate preloading can develop as the easiest and cheapest path to better perioperative blood glucose concentration management in congenital cardiac disease children.展开更多
Cyanotic congenital heart disease(CCHD),a term describing the most severe congenital heart diseases are characterized by the anatomic malformation of a right to left shunt.Although the incidence of CCHD are far less t...Cyanotic congenital heart disease(CCHD),a term describing the most severe congenital heart diseases are characterized by the anatomic malformation of a right to left shunt.Although the incidence of CCHD are far less than the that of congenital heart diseases(CHD),patients with CCHD always present severe clinical features such as hypoxia,dyspnea,and heart failure.Chronic hypoxia induces hypoxemia that significantly contributes to poor prognosis in CCHD.Current studies have demonstrated that the prolyl-4-hydroxylase2(PHD2,encoded by EGLN1)/hypoxia-inducible factor-1A(HIF-1A)pathway is a key regulator of hypoxic response.Thus,we aim to assess the associations of single polymorphisms(SNPs)of the EGLN1 gene and hypoxic response in CCHD.A missense variant of EGLN1 c.380G>C(rs1209790)was found in 46 patients(46/126),with lower hypoxia incidence and higher rate of collateral vessel formation,compared with the wild type(P<0.05).In vitro experiments,during hypoxia,EGLN1 mutation reduced EGLN1 expression compared with the wild type,with higher HIF-1A,VEGF and EPO expression levels in the mutant.No difference in HK1 expression was observed between the mutant and wild type.CCHD patients with c.380G>C showed improved response to hypoxia compared with the wild-type counterparts.The EGLN1 c.380G>C mutation improves hypoxic response through the PHD2/HIF-1A pathway,which may provide a molecular mechanism for hypoxic response in CCHD.The effects of the EGLN1 c.380G>C mutation on CCHD prognosis deserve further investigation.展开更多
AIM:To evaluate the impact of long term permanent hypoxemia noticed in patients with non operated congenital cyanogenic cyanotic cardiopathy on liver stiffness.METHODS:We included ten adult patients with non operated ...AIM:To evaluate the impact of long term permanent hypoxemia noticed in patients with non operated congenital cyanogenic cyanotic cardiopathy on liver stiffness.METHODS:We included ten adult patients with non operated inoperate cyanotic cardiopathy and ten matched patients for age and gender admitted to the gastroenterology department for proctologic diseases;Clinical and laboratory data were collected[age,gender,body mass index,oxygen saturation,glutamate oxaloacetate transaminase(GOT),glutamate pyruvate transaminase(GPT),glycemia and cholesterol].Measurement of hepatic stiffness by transient elastography was carried out in all patients using the Fibroscan device.All patients underwent an echocardiography to eliminate congestive heart failure.RESULTS:Among the patients with cyanotic cardiopathy,median liver stiffness 5.9±1.3 kPa was greater than control group(4.7±0.4 kPa)(P=0.008).Median levels of GOT,GPT,gamma-glutamyltransferase,glycemia and cholesterol were comparable in cardiopathy and control group.In regression analysis including age,gender,body mass index,oxygen saturation,GOT,GPT,glycemia,cholesterol showed that only oxygen saturation was related to liver stiffness(r=-0.63 P=0.002).CONCLUSION:Chronic permanent hypoxemia can induce mild increase of liver stiffness,but further studies are needed to explore the histological aspects of liver injury induced by chronic permanent hypoxemia.展开更多
Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospec...Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.展开更多
Background:The levels of ghrelin and tumor necrosis factor alpha (TNF-a) are considered biological markers of congenital heart diseases (CHD).The present meta-analysis was conducted to investigate the clinical signifi...Background:The levels of ghrelin and tumor necrosis factor alpha (TNF-a) are considered biological markers of congenital heart diseases (CHD).The present meta-analysis was conducted to investigate the clinical significance of serum levels of ghrelin and TNF-α in children with (CHD).Methods:Chinese and English scientific literature databases were searched to retrieve published studies relevant to ghrelin,TNF-α and CHD.Manual search was additionally employed to identify other relevant studies from cross-references.The retrieved studies were screened on the basis of our stringent inclusion and exclusion criteria to select high quality case-control studies for meta-analysis.Results:We initially retrieved 108 published studies (20 in Chinese and 88 in English) from database searches.Finally,6 case-control studies (5 in English and 1 in Chinese) were enrolled in our meta-analysis,and contained a total of 160 cyanotic congenital heart disease (CCHD) patients and 215 acyanotic congenital heart disease (ACHD) patients,along with 162 healthy controls.The results of meta-analysis showed that serum levels of ghrelin and TNF-α in CCHD or ACHD children were significantly higher than those in healthy controls.Conclusion:Our meta-analysis results showed that serum levels of ghrelin and TNF-α are elevated in children with CHD,and could be used as effective biologic markers in early diagnosis of CHD.展开更多
<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;&...<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;" "=""> dysplasia (Ellis-Van Creveld syndrome) such as skeletal and joint abnormalities often have concomitant congenital cardiac anomalies. Presence of cardiorespiratory symptoms in children with Ellis-Van Craved syndrome warrants a thorough cardiologic evaluation to recognize and treat underlying congenital heart anomaly. <b>Aim:</b> A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and <span>accomplish successful repair of the underlying cardiac lesion to reduce the cardiac</span> related morbidity and improve the patient survival. <b>Case Presentation:</b> Ten year</span><span style="font-family:;" "="">s</span><span style="font-family:;" "=""> old boy with chondroectodermal dysplasia (dental anomalies, genu valgum and other skeletal abnormalities) presented with dyspnea and cyanosis. Cardiac evaluation by 2D echo revealed an atrioventricular (AV) canal septal defect with AV valve regurgitation and a common atrium. Angiocardiography showed a goose neck deformity of the left ventricular outflow tract. <span>The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO<sub>2</sub> of 0.7) </span>and O<sub>2</sub> saturation in the common atrium was 0.7. The pulmonary venous connections to the common atrium were anomalous. Atriotomy on cardiopulmonary bypass and on a cardioplegic arrest discerned a partial AV canal septal defect with a common bridging leaflet, clefts in septal leaflets of tricuspid and mitral vlalves, an incompletely closed interventricular communication, and a common atrium with highly anomalous pulmonary venous insertions well anterior (8</span><span style="font-family:;" "=""> </span><span style="font-family:;" "="">cm) to vena caval orifices. Intracardiac repair was performed with two patches of Goertex to partition the common atrium into the pulmonary a展开更多
Objective Many physiological and pathological conditions,including cyanotic congenital heart diseases(CCHD),are accompanied by chronic hypoxia,which might interfere with the transcription process.However,the transcrip...Objective Many physiological and pathological conditions,including cyanotic congenital heart diseases(CCHD),are accompanied by chronic hypoxia,which might interfere with the transcription process.However,the transcriptome profile in peripheral blood under hypoxia is still unidentified.The present work aimed to explore the transcriptional profile alteration of peripheral blood in chronic hypoxia.Methods The present study used a chronic hypoxia rat model to simulate the hypoxic state of CCHD patients.Two groups of Sprague-Dawley rats(n=6 per group)were either exposed to hypoxia(10%O2)or normoxia(21%O2)for 3 weeks.Body weight was measured weekly.Peripheral blood was collected and total RNA was extracted for RNA-Seq at the end of the hypoxia treatment.After quality assessment,the library was sequenced by the Illumina Hiseq platform.The differentially expressed genes were screened(false discovery rate<0.05 and fold change>2).The functional annotation analysis and cluster analysis of differentially expressed genes were performed based on the adjusted P-value(padj<0.05).Results Compared with the control group,the body weight of the rats in the hypoxia group was significantly lowered(P<0.01).RNA-Seq results showed that the transcriptome patterns of the two groups had significant differences.In total,872 genes were identified as differentially expressed.Among all,803 genes were downregulated,while only 69 genes were up-regulated in the hypoxia group.The functional enrichment analysis of the 872 genes showed that multiple biological processes involved,such as porphyrin-containing compound metabolic process,hemoglobin complex and oxygen transporter activity.Conclusions Our study demonstrated the transcriptional profile alteration in peripheral blood of chronic hypoxia rat model.This study provided basic data and directions to further understand the physiological and pathological changes in patients with CCHD.展开更多
BACKGROUND Double outlet right ventricle(DORV)is a rare and complex congenital heart defect,and the surgical repairs vary with type and pathophysiology consequences.Due to prolonged progressive hypoxemia,severe polycy...BACKGROUND Double outlet right ventricle(DORV)is a rare and complex congenital heart defect,and the surgical repairs vary with type and pathophysiology consequences.Due to prolonged progressive hypoxemia,severe polycythemia is common in patients with DORV,which ultimately leads to coagulation dysfunction and increases the risk of thrombosis and infarction.Consequently,the anesthetic management is challenging and how to manage severe polycythemia and avoid hypoxia-related complications in such patients is of great significance.CASE SUMMARY Herein,we report the anesthetic management of a 10-year-old female patient with a DORV.She lived in the low-oxygen Qinghai-Tibet Plateau,and presented with severe polycythemia(hemoglobin,24.8 g/dL;hematocrit,75%).She underwent a modified Fontan surgery,which was satisfactory and without any perioperative complications.Our anesthetic management highlights the importance of perioperative hemodilution in decreasing the risk of thromboembolism and the importance of correcting coagulopathy in preventing hemorrhage.CONCLUSION Anesthetic management is challenging in rare cyanotic congenital heart disease patients with severe polycythemia.It is important to adopt perioperative hemodilution and correction of coagulopathy in preventing thrombosis and hemorrhage.展开更多
文摘Objectives: Hypoglycemia is a recognized danger in pediatric patients. Extended period of preoperative fasting in this subset of patients is not well tolerated with metabolic derangements. The oral carbohydrate loading preoperatively can ameliorate many adverse effects. The aim of this study was to compare the glycemic profile in pediatric cardiac surgical patients kept fasting preoperatively with those fed oral clear solutions of carbohydrate half hour prior to induction of anaesthesia. Also we tried to establish a correlation with other factors contributing to preoperative hypoglycemia. Methodology: We planned a randomized controlled study. Group A included patients who were kept fasting according to the ASA guidelines preoperatively and Group B included patients who received 2 ml per kg of body weight of 10% Dextrose water as oral feeds half hour before the expected time of start of anaesthesia. Results: The mean (SD) preoperative BG concentrations were higher in group B (102.5 ±16.97) as compared to group A (64.08 ± 25.37) (p value -0.86 and -0.67) (pvalue Conclusion: Preoperative oral carbohydrate preloading can develop as the easiest and cheapest path to better perioperative blood glucose concentration management in congenital cardiac disease children.
基金This work is supported by the National Nature Science Foundation of China(81570218)Major Project of Chongqing Municipal Health Bureau(56-20141009)clinical project of Children’s Hospital of Chongqing Medical University(hjyn2012-6).
文摘Cyanotic congenital heart disease(CCHD),a term describing the most severe congenital heart diseases are characterized by the anatomic malformation of a right to left shunt.Although the incidence of CCHD are far less than the that of congenital heart diseases(CHD),patients with CCHD always present severe clinical features such as hypoxia,dyspnea,and heart failure.Chronic hypoxia induces hypoxemia that significantly contributes to poor prognosis in CCHD.Current studies have demonstrated that the prolyl-4-hydroxylase2(PHD2,encoded by EGLN1)/hypoxia-inducible factor-1A(HIF-1A)pathway is a key regulator of hypoxic response.Thus,we aim to assess the associations of single polymorphisms(SNPs)of the EGLN1 gene and hypoxic response in CCHD.A missense variant of EGLN1 c.380G>C(rs1209790)was found in 46 patients(46/126),with lower hypoxia incidence and higher rate of collateral vessel formation,compared with the wild type(P<0.05).In vitro experiments,during hypoxia,EGLN1 mutation reduced EGLN1 expression compared with the wild type,with higher HIF-1A,VEGF and EPO expression levels in the mutant.No difference in HK1 expression was observed between the mutant and wild type.CCHD patients with c.380G>C showed improved response to hypoxia compared with the wild-type counterparts.The EGLN1 c.380G>C mutation improves hypoxic response through the PHD2/HIF-1A pathway,which may provide a molecular mechanism for hypoxic response in CCHD.The effects of the EGLN1 c.380G>C mutation on CCHD prognosis deserve further investigation.
文摘AIM:To evaluate the impact of long term permanent hypoxemia noticed in patients with non operated congenital cyanogenic cyanotic cardiopathy on liver stiffness.METHODS:We included ten adult patients with non operated inoperate cyanotic cardiopathy and ten matched patients for age and gender admitted to the gastroenterology department for proctologic diseases;Clinical and laboratory data were collected[age,gender,body mass index,oxygen saturation,glutamate oxaloacetate transaminase(GOT),glutamate pyruvate transaminase(GPT),glycemia and cholesterol].Measurement of hepatic stiffness by transient elastography was carried out in all patients using the Fibroscan device.All patients underwent an echocardiography to eliminate congestive heart failure.RESULTS:Among the patients with cyanotic cardiopathy,median liver stiffness 5.9±1.3 kPa was greater than control group(4.7±0.4 kPa)(P=0.008).Median levels of GOT,GPT,gamma-glutamyltransferase,glycemia and cholesterol were comparable in cardiopathy and control group.In regression analysis including age,gender,body mass index,oxygen saturation,GOT,GPT,glycemia,cholesterol showed that only oxygen saturation was related to liver stiffness(r=-0.63 P=0.002).CONCLUSION:Chronic permanent hypoxemia can induce mild increase of liver stiffness,but further studies are needed to explore the histological aspects of liver injury induced by chronic permanent hypoxemia.
文摘Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.
文摘Background:The levels of ghrelin and tumor necrosis factor alpha (TNF-a) are considered biological markers of congenital heart diseases (CHD).The present meta-analysis was conducted to investigate the clinical significance of serum levels of ghrelin and TNF-α in children with (CHD).Methods:Chinese and English scientific literature databases were searched to retrieve published studies relevant to ghrelin,TNF-α and CHD.Manual search was additionally employed to identify other relevant studies from cross-references.The retrieved studies were screened on the basis of our stringent inclusion and exclusion criteria to select high quality case-control studies for meta-analysis.Results:We initially retrieved 108 published studies (20 in Chinese and 88 in English) from database searches.Finally,6 case-control studies (5 in English and 1 in Chinese) were enrolled in our meta-analysis,and contained a total of 160 cyanotic congenital heart disease (CCHD) patients and 215 acyanotic congenital heart disease (ACHD) patients,along with 162 healthy controls.The results of meta-analysis showed that serum levels of ghrelin and TNF-α in CCHD or ACHD children were significantly higher than those in healthy controls.Conclusion:Our meta-analysis results showed that serum levels of ghrelin and TNF-α are elevated in children with CHD,and could be used as effective biologic markers in early diagnosis of CHD.
文摘<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;" "=""> dysplasia (Ellis-Van Creveld syndrome) such as skeletal and joint abnormalities often have concomitant congenital cardiac anomalies. Presence of cardiorespiratory symptoms in children with Ellis-Van Craved syndrome warrants a thorough cardiologic evaluation to recognize and treat underlying congenital heart anomaly. <b>Aim:</b> A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and <span>accomplish successful repair of the underlying cardiac lesion to reduce the cardiac</span> related morbidity and improve the patient survival. <b>Case Presentation:</b> Ten year</span><span style="font-family:;" "="">s</span><span style="font-family:;" "=""> old boy with chondroectodermal dysplasia (dental anomalies, genu valgum and other skeletal abnormalities) presented with dyspnea and cyanosis. Cardiac evaluation by 2D echo revealed an atrioventricular (AV) canal septal defect with AV valve regurgitation and a common atrium. Angiocardiography showed a goose neck deformity of the left ventricular outflow tract. <span>The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO<sub>2</sub> of 0.7) </span>and O<sub>2</sub> saturation in the common atrium was 0.7. The pulmonary venous connections to the common atrium were anomalous. Atriotomy on cardiopulmonary bypass and on a cardioplegic arrest discerned a partial AV canal septal defect with a common bridging leaflet, clefts in septal leaflets of tricuspid and mitral vlalves, an incompletely closed interventricular communication, and a common atrium with highly anomalous pulmonary venous insertions well anterior (8</span><span style="font-family:;" "=""> </span><span style="font-family:;" "="">cm) to vena caval orifices. Intracardiac repair was performed with two patches of Goertex to partition the common atrium into the pulmonary a
基金the National Science Fund for Distinguished Young Scholars(81525002,2016-2020).
文摘Objective Many physiological and pathological conditions,including cyanotic congenital heart diseases(CCHD),are accompanied by chronic hypoxia,which might interfere with the transcription process.However,the transcriptome profile in peripheral blood under hypoxia is still unidentified.The present work aimed to explore the transcriptional profile alteration of peripheral blood in chronic hypoxia.Methods The present study used a chronic hypoxia rat model to simulate the hypoxic state of CCHD patients.Two groups of Sprague-Dawley rats(n=6 per group)were either exposed to hypoxia(10%O2)or normoxia(21%O2)for 3 weeks.Body weight was measured weekly.Peripheral blood was collected and total RNA was extracted for RNA-Seq at the end of the hypoxia treatment.After quality assessment,the library was sequenced by the Illumina Hiseq platform.The differentially expressed genes were screened(false discovery rate<0.05 and fold change>2).The functional annotation analysis and cluster analysis of differentially expressed genes were performed based on the adjusted P-value(padj<0.05).Results Compared with the control group,the body weight of the rats in the hypoxia group was significantly lowered(P<0.01).RNA-Seq results showed that the transcriptome patterns of the two groups had significant differences.In total,872 genes were identified as differentially expressed.Among all,803 genes were downregulated,while only 69 genes were up-regulated in the hypoxia group.The functional enrichment analysis of the 872 genes showed that multiple biological processes involved,such as porphyrin-containing compound metabolic process,hemoglobin complex and oxygen transporter activity.Conclusions Our study demonstrated the transcriptional profile alteration in peripheral blood of chronic hypoxia rat model.This study provided basic data and directions to further understand the physiological and pathological changes in patients with CCHD.
基金The 1.3.5.Project for Disciplines of Excellence,No.2018HXFH046West China Hospital,Sichuan University and the National Natural Science Foundation of China,No.81971806.
文摘BACKGROUND Double outlet right ventricle(DORV)is a rare and complex congenital heart defect,and the surgical repairs vary with type and pathophysiology consequences.Due to prolonged progressive hypoxemia,severe polycythemia is common in patients with DORV,which ultimately leads to coagulation dysfunction and increases the risk of thrombosis and infarction.Consequently,the anesthetic management is challenging and how to manage severe polycythemia and avoid hypoxia-related complications in such patients is of great significance.CASE SUMMARY Herein,we report the anesthetic management of a 10-year-old female patient with a DORV.She lived in the low-oxygen Qinghai-Tibet Plateau,and presented with severe polycythemia(hemoglobin,24.8 g/dL;hematocrit,75%).She underwent a modified Fontan surgery,which was satisfactory and without any perioperative complications.Our anesthetic management highlights the importance of perioperative hemodilution in decreasing the risk of thromboembolism and the importance of correcting coagulopathy in preventing hemorrhage.CONCLUSION Anesthetic management is challenging in rare cyanotic congenital heart disease patients with severe polycythemia.It is important to adopt perioperative hemodilution and correction of coagulopathy in preventing thrombosis and hemorrhage.
