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 plasm展开更多
目的回顾性分析47例右心室双出口（double outlet right ventricle，DORV）病例的病理解剖特点及其手术方式，探讨DORV的个案化手术设计治疗。方法自1997年1月至2006年1月对47例DORV患者行手术治疗，其中男性31例，女性16例；年龄2-26...目的回顾性分析47例右心室双出口（double outlet right ventricle，DORV）病例的病理解剖特点及其手术方式，探讨DORV的个案化手术设计治疗。方法自1997年1月至2006年1月对47例DORV患者行手术治疗，其中男性31例，女性16例；年龄2-26（13．6±12．1）岁。诊断为单纯DORV者36例，DORV合并镜面右位心、内脏转位5例，1例合并下腔静脉闭锁、左上腔静脉畸形引流及无顶冠状静脉窦畸形，2例Taussing—-ing心脏畸形，2例伴有完全型房室隔缺损（其中1例伴有大血管转位），46例患者均伴有肺动脉狭窄（PS），临床表现为紫绀和杵状指。另外1例17岁男性患者术前仅由超声心动图诊断为右位心、室间隔缺损（VSD），无紫绀，术中探查为DORV且无PS，肺动脉压不高。手术方式：（1）心内隧道修补VSD、心外补片加宽流出道和肺动脉36例；（2）内管道连接VSD与主动脉，同时作补片加宽右室流出道或切断（开）肺动脉，封闭其近端、远端与右室切口之间用外管道连接（Rastelli手术）8例；（3）全腔静脉-肺动脉连接术2例；（4）Glenn（格林）手术1例。结果平均带气管导管时间（30．6±18．40）h，平均住ICU时间（2．8±1．5）d，平均住院日（25．4±18．O）d。术后出现并发症6例，其中二次插管1例，胸水2例，腹水2例，气胸1例，低心排1例。手术后早期死亡3例，死亡率6．4％。远期随访28例，行超声心动图检查均无残余梗阻和残余分流。结论明确DORV病理解剖关系，根据不同病变采取不同手术方式予以矫治，是取得良好治疗效果的保证。展开更多
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
<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
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
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 plasm
文摘目的回顾性分析47例右心室双出口（double outlet right ventricle，DORV）病例的病理解剖特点及其手术方式，探讨DORV的个案化手术设计治疗。方法自1997年1月至2006年1月对47例DORV患者行手术治疗，其中男性31例，女性16例；年龄2-26（13．6±12．1）岁。诊断为单纯DORV者36例，DORV合并镜面右位心、内脏转位5例，1例合并下腔静脉闭锁、左上腔静脉畸形引流及无顶冠状静脉窦畸形，2例Taussing—-ing心脏畸形，2例伴有完全型房室隔缺损（其中1例伴有大血管转位），46例患者均伴有肺动脉狭窄（PS），临床表现为紫绀和杵状指。另外1例17岁男性患者术前仅由超声心动图诊断为右位心、室间隔缺损（VSD），无紫绀，术中探查为DORV且无PS，肺动脉压不高。手术方式：（1）心内隧道修补VSD、心外补片加宽流出道和肺动脉36例；（2）内管道连接VSD与主动脉，同时作补片加宽右室流出道或切断（开）肺动脉，封闭其近端、远端与右室切口之间用外管道连接（Rastelli手术）8例；（3）全腔静脉-肺动脉连接术2例；（4）Glenn（格林）手术1例。结果平均带气管导管时间（30．6±18．40）h，平均住ICU时间（2．8±1．5）d，平均住院日（25．4±18．O）d。术后出现并发症6例，其中二次插管1例，胸水2例，腹水2例，气胸1例，低心排1例。手术后早期死亡3例，死亡率6．4％。远期随访28例，行超声心动图检查均无残余梗阻和残余分流。结论明确DORV病理解剖关系，根据不同病变采取不同手术方式予以矫治，是取得良好治疗效果的保证。
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<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>
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.
