Congenital left ventricular aneurysm or diverticulum are rare cardiac malformations described in 809 cases since the first description in 1816, being associated with other cardiac, vascular or thoraco-abdominal abnorm...Congenital left ventricular aneurysm or diverticulum are rare cardiac malformations described in 809 cases since the first description in 1816, being associated with other cardiac, vascular or thoraco-abdominal abnormalities in about 70%. It appears to be a developmental anomaly, starting in the 4th embryonic week. In an experimental study, targeted knockdown of cardiac troponin T in the chick was performed at day 3, after the heart tube has formed. Morpholino treatment of gene TNNT2 at this stage led to the development of left ventricular diver- ticula （LVD） in the primitive left ventricular wall. Diagnosis of left ventricular aneurysms （LVA）/LVD can be made after exclusion of coro- nary artery disease, local or systemic inflammation or traumatic causes as well as cardiomyopathies. Clinically, most of LVA and LVD are asymptomatic or may cause systemic embolization, congestive heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia or sudden cardiac death. Diagnosis is established by imaging studies （echocardiography, magnetic resonance imaging or left ventricular angiography） visualizing the structural changes and accompanying abnormalities. Mode of treatment has to be individually tai- lored and depends on clinical presentation, accompanying abnormalities and possible complications, options include surgical resection （espe- cially in symptomatic patients）, anticoagulation after systemic embolization, radiofrequency ablation or implantation of an implantable car- dioverter defibrillator （ICD） in case of symptomatic ventricular tachycardias, and occasionally combined with class I- or III-antiarrhythmic drugs. Cardiac death occurs usually in childhood, is significantly more frequent in LVA patients and caused by congestive heart failure in most of the cases, whereas patients diagnosed with LVD died more frequently from rupture of the LVD.展开更多
Objective: To study the value of detecting fetal congenital heart disease (CHD) using the five transverse planes technique of fetal echocardiography. Methods: Nine hundred and eighty-two high-risk pregnancies for feta...Objective: To study the value of detecting fetal congenital heart disease (CHD) using the five transverse planes technique of fetal echocardiography. Methods: Nine hundred and eighty-two high-risk pregnancies for fetal CHD were included in this study, the fetal heart was scanned with the five transverse planes technique of fetal echocardiography described by Yagel, autopsy was conducted when pregnancy was terminated. Blood from fetal heart was collected for fetal chromosome analysis. A close follow-up was given for normal fetal heart pregnancies and neonatal echocardiography was performed to check the accuracy of prenatal diagnosis. Results: (1) Forty-six cases (4.68%) were found to have fetal heart abnormalities in this study, 69.56% of them were diagnosed by single four-chamber view, another 30.43% fetal CHD were found by combining other views; (2) Forty-one parents of prenatal fetuses with CHD chose to terminate pregnancy, thirty-two of them gave consent to conduct autopsy, 93.75% of which yielded unanimous conclusion between prenatal fetal echocardiography and autopsy; (3) Thirty-two of 46 cases underwent fetal chromosome analysis, 8 cases (25%) were found to have abnormal chromosome; (4) Five cases were found to have right ventricle and atrium a little bigger than those on the left side, with the unequal condition being the same after birth, but there were no clinical manifestations and they are healthy for the time being; (5) Nine hundred and thirty-six cases were not found with abnormality in this study, but one case was diagnosed with ventricular septal defect after birth, one case was diagnosed with patent ductus arteriosus, one case had atrial septal defect after birth. Conclusions: (1) The detected CHD rate was 4.68% by screening fetal heart with five transverse planes according to Yagel’s description of high risk population basis for CHD. The coinciding rate of prenatal diagnosis and autopsy was 93.75%; (2) The sensitivity of detecting fetal heart abnormality is 92%, the specificity is 99.6% using the five transverse planes technique of fetal echocardiography; (3) Fetuses with mild or moderate disproportion of right and left side in the heart are potentially healthy babies.展开更多
Objective.To assess the feature of pulmonary blood flow distribution after total cavopulmonary connection(TCPC)of different types,and to provide the selection of the best type.Methods. Thirty-two consecutive survival ...Objective.To assess the feature of pulmonary blood flow distribution after total cavopulmonary connection(TCPC)of different types,and to provide the selection of the best type.Methods. Thirty-two consecutive survival patients after TCPC underwent radionuclide lung perfusion imaging. According to the radionuclide counts in the left and right lungs,analyses of the distribution of blood flow from superior venous cava(SVC) and inferior venous cava(IVC)and the whole pulmonary blood flow in both lungs were made. All patients were divided into 4 groups by the the anastomosis between IVC and pulmonary artery.Results. GroupⅠ:The flow ratio of the IVC to left lung was greater than that to the right lung,P≤0.01;the flow ratio of the SVC to right lung was greater than that to the left lung,P≤0.01;and the whole pulmonary blood flow went dominantly to the left lung,P≤0.05,which is not in line with physiological distribution. GroupⅡ:the flows from the SVC and IVC were mixed in the middle of the junction and ran evenly into the right and left lungs,the whole pulmonary blood flow went to both lungs,P≥0.05. Group Ⅲ:the flow ratio of the SVC to both lungs were the same,P≥0.05,and major part from IVC went to the right lung,P≤0.01;the pulmonary blood flow go dominantly to the right lung,P≤0.05,which is in accord with physiological distribution. Group Ⅳ:the flows from the right SVC went to right lung by 100%,P≤0.01,and that from the left SVC went to left lung by 100% too,P≤0.01;the flows from IVC went dominantly to the left lung,with little part to the right lung ,P≤0.05.Conclusions. Different types of TCPC can result in different pulmonary blood distributions. The best flow distribution between the left and right lungs can be obtained by an offset of the IVC anastomosis toward the RPA with widening anastomosis for the patients without persist left superior venous cava(PLSVC).展开更多
Gastric teratoma (GT) is a seldom seen congenital abnormality. GT always occurs in children. The greater curvature and posterior wall of the stomach are the most common sites involving GT. We diagnosed a case of GT lo...Gastric teratoma (GT) is a seldom seen congenital abnormality. GT always occurs in children. The greater curvature and posterior wall of the stomach are the most common sites involving GT. We diagnosed a case of GT located on the inferior wall of the cardiac orifice in a 20-year-old man. To the best of our knowledge, this is the first case of GT located on the wall of the cardiac orifice in an adult in the English literature. We report this unusual case as an addition to this rare disease usually found in children. Computed tomography combined with endoscopic ultrasonography can be selected to diagnose GT.展开更多
Dear Editor,Berry syndrome is a rare combination of congenital cardiac malformations characterized by four abnormal features;namely,an aortopulmonary window(APW),aortic origin of the right pulmonary artery,hypoplasia ...Dear Editor,Berry syndrome is a rare combination of congenital cardiac malformations characterized by four abnormal features;namely,an aortopulmonary window(APW),aortic origin of the right pulmonary artery,hypoplasia or interruption of the aortic arch,with an intact ventricular septum.The disease was first reported by Berry in 1982,who estimated the incidence within the population with congenital cardiac malformations to be 0.046%(Berry et al.,1982).Until recently,展开更多
文摘Congenital left ventricular aneurysm or diverticulum are rare cardiac malformations described in 809 cases since the first description in 1816, being associated with other cardiac, vascular or thoraco-abdominal abnormalities in about 70%. It appears to be a developmental anomaly, starting in the 4th embryonic week. In an experimental study, targeted knockdown of cardiac troponin T in the chick was performed at day 3, after the heart tube has formed. Morpholino treatment of gene TNNT2 at this stage led to the development of left ventricular diver- ticula （LVD） in the primitive left ventricular wall. Diagnosis of left ventricular aneurysms （LVA）/LVD can be made after exclusion of coro- nary artery disease, local or systemic inflammation or traumatic causes as well as cardiomyopathies. Clinically, most of LVA and LVD are asymptomatic or may cause systemic embolization, congestive heart failure, valvular regurgitation, ventricular wall rupture, ventricular tachycardia or sudden cardiac death. Diagnosis is established by imaging studies （echocardiography, magnetic resonance imaging or left ventricular angiography） visualizing the structural changes and accompanying abnormalities. Mode of treatment has to be individually tai- lored and depends on clinical presentation, accompanying abnormalities and possible complications, options include surgical resection （espe- cially in symptomatic patients）, anticoagulation after systemic embolization, radiofrequency ablation or implantation of an implantable car- dioverter defibrillator （ICD） in case of symptomatic ventricular tachycardias, and occasionally combined with class I- or III-antiarrhythmic drugs. Cardiac death occurs usually in childhood, is significantly more frequent in LVA patients and caused by congestive heart failure in most of the cases, whereas patients diagnosed with LVD died more frequently from rupture of the LVD.
基金Project supported by the Start-up Fund for Study-abroad Returnee, Ministry of Education, China
文摘Objective: To study the value of detecting fetal congenital heart disease (CHD) using the five transverse planes technique of fetal echocardiography. Methods: Nine hundred and eighty-two high-risk pregnancies for fetal CHD were included in this study, the fetal heart was scanned with the five transverse planes technique of fetal echocardiography described by Yagel, autopsy was conducted when pregnancy was terminated. Blood from fetal heart was collected for fetal chromosome analysis. A close follow-up was given for normal fetal heart pregnancies and neonatal echocardiography was performed to check the accuracy of prenatal diagnosis. Results: (1) Forty-six cases (4.68%) were found to have fetal heart abnormalities in this study, 69.56% of them were diagnosed by single four-chamber view, another 30.43% fetal CHD were found by combining other views; (2) Forty-one parents of prenatal fetuses with CHD chose to terminate pregnancy, thirty-two of them gave consent to conduct autopsy, 93.75% of which yielded unanimous conclusion between prenatal fetal echocardiography and autopsy; (3) Thirty-two of 46 cases underwent fetal chromosome analysis, 8 cases (25%) were found to have abnormal chromosome; (4) Five cases were found to have right ventricle and atrium a little bigger than those on the left side, with the unequal condition being the same after birth, but there were no clinical manifestations and they are healthy for the time being; (5) Nine hundred and thirty-six cases were not found with abnormality in this study, but one case was diagnosed with ventricular septal defect after birth, one case was diagnosed with patent ductus arteriosus, one case had atrial septal defect after birth. Conclusions: (1) The detected CHD rate was 4.68% by screening fetal heart with five transverse planes according to Yagel’s description of high risk population basis for CHD. The coinciding rate of prenatal diagnosis and autopsy was 93.75%; (2) The sensitivity of detecting fetal heart abnormality is 92%, the specificity is 99.6% using the five transverse planes technique of fetal echocardiography; (3) Fetuses with mild or moderate disproportion of right and left side in the heart are potentially healthy babies.
文摘Objective.To assess the feature of pulmonary blood flow distribution after total cavopulmonary connection(TCPC)of different types,and to provide the selection of the best type.Methods. Thirty-two consecutive survival patients after TCPC underwent radionuclide lung perfusion imaging. According to the radionuclide counts in the left and right lungs,analyses of the distribution of blood flow from superior venous cava(SVC) and inferior venous cava(IVC)and the whole pulmonary blood flow in both lungs were made. All patients were divided into 4 groups by the the anastomosis between IVC and pulmonary artery.Results. GroupⅠ:The flow ratio of the IVC to left lung was greater than that to the right lung,P≤0.01;the flow ratio of the SVC to right lung was greater than that to the left lung,P≤0.01;and the whole pulmonary blood flow went dominantly to the left lung,P≤0.05,which is not in line with physiological distribution. GroupⅡ:the flows from the SVC and IVC were mixed in the middle of the junction and ran evenly into the right and left lungs,the whole pulmonary blood flow went to both lungs,P≥0.05. Group Ⅲ:the flow ratio of the SVC to both lungs were the same,P≥0.05,and major part from IVC went to the right lung,P≤0.01;the pulmonary blood flow go dominantly to the right lung,P≤0.05,which is in accord with physiological distribution. Group Ⅳ:the flows from the right SVC went to right lung by 100%,P≤0.01,and that from the left SVC went to left lung by 100% too,P≤0.01;the flows from IVC went dominantly to the left lung,with little part to the right lung ,P≤0.05.Conclusions. Different types of TCPC can result in different pulmonary blood distributions. The best flow distribution between the left and right lungs can be obtained by an offset of the IVC anastomosis toward the RPA with widening anastomosis for the patients without persist left superior venous cava(PLSVC).
文摘Gastric teratoma (GT) is a seldom seen congenital abnormality. GT always occurs in children. The greater curvature and posterior wall of the stomach are the most common sites involving GT. We diagnosed a case of GT located on the inferior wall of the cardiac orifice in a 20-year-old man. To the best of our knowledge, this is the first case of GT located on the wall of the cardiac orifice in an adult in the English literature. We report this unusual case as an addition to this rare disease usually found in children. Computed tomography combined with endoscopic ultrasonography can be selected to diagnose GT.
文摘Dear Editor,Berry syndrome is a rare combination of congenital cardiac malformations characterized by four abnormal features;namely,an aortopulmonary window(APW),aortic origin of the right pulmonary artery,hypoplasia or interruption of the aortic arch,with an intact ventricular septum.The disease was first reported by Berry in 1982,who estimated the incidence within the population with congenital cardiac malformations to be 0.046%(Berry et al.,1982).Until recently,
