Objective-Double outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair proce...Objective-Double outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure. To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle. Methods From July to November of 2009,6展开更多
The management of pulmonary atresia with intact ventricular septum（PA/IVS） remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular（Font...The management of pulmonary atresia with intact ventricular septum（PA/IVS） remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular（Fontan or one and a half ventricle repair） strategies. Although outcomes have been improved,these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials（RCTs） comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival,and secondary criteria included exercise function,arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless,several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review,we will discuss the currently available data.展开更多
Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-...Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair.Methods:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes.Surgical history and clinical outcomes were reviewed.Results:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle correction.Thirteen patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial fibrillation.Three patients with SND needed a pacemaker implantation.Only Fontan circulation showed an association with SND,while the other two groups heterogeneous types of arrhythmias.Conclusions:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow up.Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.展开更多
Appropriate heart rate in a failing pulmonary ventricle remains unknown, particularly in congenital heart diseasewith unique hemodynamics. A 71-year-old male with repaired tetralogy of Fallot and a pacemaker for a sin...Appropriate heart rate in a failing pulmonary ventricle remains unknown, particularly in congenital heart diseasewith unique hemodynamics. A 71-year-old male with repaired tetralogy of Fallot and a pacemaker for a sinusnode dysfunction suffered from heart failure symptoms with preserved left ventricular function. Simply changingthe pacemaker’s lower rate from 60 to 75 bpm, New York Heart Association classification improved from III to II,and hemodynamic parameters drastically improved. We regarded this case as informative. Appropriate heart ratecould be higher in congenital patients with failing right and non-failing left ventricles than in adults with malfunctioningLV.展开更多
Left ventricle(LV) pseudoaneurysm is a late mechanical complication of myocardial infarction.A giant LV pseudoaneurysm is a rare presentation.We report a case of giant LV pseudoaneurysm in a post-MI patient who presen...Left ventricle(LV) pseudoaneurysm is a late mechanical complication of myocardial infarction.A giant LV pseudoaneurysm is a rare presentation.We report a case of giant LV pseudoaneurysm in a post-MI patient who presented with gross congestive heart failure.The patient had a successful surgical repair of the aneurysm and had a favorable 3-mo outcome.The imaging modality and surgical treatment of the pseudoaneurysm are discussed.展开更多
Left ventricular (LV) pseudoaneurysm is a late mechanical complication of myocardial infarction (MI). A giant LV pseudoaneurysm is a rare presentation. We report a case of a giant LV pseudoaneurysm in a post MI patien...Left ventricular (LV) pseudoaneurysm is a late mechanical complication of myocardial infarction (MI). A giant LV pseudoaneurysm is a rare presentation. We report a case of a giant LV pseudoaneurysm in a post MI patient, who presented with hemoptysis. Hemoptysis is a rare clinical presentation of LV pseudoaneurysm. The patient had successful surgical repair of the aneurysm and had a favorable outcome in 9 mo’ follow-up. The imaging modalities and surgical treatment of a pseudoaneurysm is discussed.展开更多
Background:Bidirectional Glenn shunts have long been available as palliative procedures for patients with single ventricle physiology that is,patients unsuitable for biventricular repair.In our country they are perfor...Background:Bidirectional Glenn shunts have long been available as palliative procedures for patients with single ventricle physiology that is,patients unsuitable for biventricular repair.In our country they are performed at an older age than that recommended by the literature.So,we aim to assess post bidirectional Glenn shunt patients to detect the presence of any complications and to evaluate their functional capacity.Methods:This was a descriptive study that included all patients who underwent a bidirectional Glenn shunt and were referred for follow up in Ain shams university hospitals.History taking including NYHA class and physical activity,clinical examination,six-minutes-walk test,laboratory investigations,full echocardiographic assessment were done for all patients.Some patients needed invasive cardiac catheterization.Results:Our registry included 178 patients who underwent bidirectional Glenn procedure referred for follow up in Ain Shams university hospital from January 2019 till July 2020.The mean age of the registry was 18.7±8.26 years(range between 5 and 37 years),85 males(48%)and 93 females(52%).Regarding the basic anatomy,double outlet right ventricle with hypoplastic left ventricle was the commonest.Furthermore,our descriptive study confirmed many characteristic similarities between our patients and patients in developing countries.Our patients underwent bidirectional Glenn shunt at a median age of 6 years which is considered a relatively old age but similar to other studies that have been made in developing countries like Pakistan,India and Iran.There is a significant delay in the operation in Egyptian patients due to lack of patients’awareness,few numbers of primary health care facilities and high economic burden.Conclusion:Although the presence of slight systemic desaturation,our study demonstrated a satisfactory functional capacity among our patients;thus,bidirectional Glenn can be considered an acceptable definite univentricular repair in patients with late presentation.展开更多
Objective: In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in bor...Objective: In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in borderline candidates will lead to adverse clinical outcomes. Therefore, it is important to accurately assess the degree of ventricular development before operation and whether it can tolerate biventricular repair. This review evaluated ventricular development using echocardiography for a better prediction of biventricular repair in borderline ventricle. Data sources: Articles from January 1, 1990 to April 1, 2019 on biventricular repair in borderline ventricle were accessed from PubMed, using keywords including "borderline ventricle,""congenital heart disease,""CHD,""echocardiography," and "biventricular repair." Study selection: Original articles and critical reviews relevant to the review’s theme were selected. Results: Borderline left ventricle (LV):(1) Critical aortic stenosis: the Rhodes score, Congenital Heart Surgeons Society regression equation and another new scoring system was proposed to predict the feasibility of biventricular repair.(2) Aortic arch hypoplasia: the LV size and the diameter of aortic and mitral valve (MV) annulus should be taken into considerations for biventricular repair.(3) Right-dominant unbalanced atrioventricular septal defect (AVSD): atrioventricular valve index (AVVI), left ventricular inflow index (LVII), and right ventricle (RV)/LV inflow angle were the echocardiographic indices for biventricular repair. Borderline RV:(1) pulmonary atresia/intact ventricular septum (PA/IVS): the diameter z-score of tricuspid valve (TV) annulus, ratio of TV to MV diameter, RV inlet length z-score, RV area z-score, RV development index, and RV-TV index, etc. Less objective but more practical description is to classify the RV as tripartite, bipartite, and unipartite. The presence or absence of RV sinusoids, RV dependent coronary circulation, and the degree of tricuspid regurgitation should also be noted.(2) Left-dominant unbalanced AVSD: AVVI, LV, and RV volumes, whether apex forming ventricles were the echocardiographic indices for biventricular repair. Conclusions: Although the evaluation of echocardiography cannot guarantee the success of biventricular repair surgery, echocardiography can still provide relatively valuable basis for surgical decision making.展开更多
文摘Objective-Double outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure. To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle. Methods From July to November of 2009,6
文摘The management of pulmonary atresia with intact ventricular septum（PA/IVS） remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular（Fontan or one and a half ventricle repair） strategies. Although outcomes have been improved,these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials（RCTs） comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival,and secondary criteria included exercise function,arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless,several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review,we will discuss the currently available data.
文摘Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical repair.Methods:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic syndromes.Surgical history and clinical outcomes were reviewed.Results:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle correction.Thirteen patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial fibrillation.Three patients with SND needed a pacemaker implantation.Only Fontan circulation showed an association with SND,while the other two groups heterogeneous types of arrhythmias.Conclusions:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow up.Continuous follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.
文摘Appropriate heart rate in a failing pulmonary ventricle remains unknown, particularly in congenital heart diseasewith unique hemodynamics. A 71-year-old male with repaired tetralogy of Fallot and a pacemaker for a sinusnode dysfunction suffered from heart failure symptoms with preserved left ventricular function. Simply changingthe pacemaker’s lower rate from 60 to 75 bpm, New York Heart Association classification improved from III to II,and hemodynamic parameters drastically improved. We regarded this case as informative. Appropriate heart ratecould be higher in congenital patients with failing right and non-failing left ventricles than in adults with malfunctioningLV.
文摘Left ventricle(LV) pseudoaneurysm is a late mechanical complication of myocardial infarction.A giant LV pseudoaneurysm is a rare presentation.We report a case of giant LV pseudoaneurysm in a post-MI patient who presented with gross congestive heart failure.The patient had a successful surgical repair of the aneurysm and had a favorable 3-mo outcome.The imaging modality and surgical treatment of the pseudoaneurysm are discussed.
文摘Left ventricular (LV) pseudoaneurysm is a late mechanical complication of myocardial infarction (MI). A giant LV pseudoaneurysm is a rare presentation. We report a case of a giant LV pseudoaneurysm in a post MI patient, who presented with hemoptysis. Hemoptysis is a rare clinical presentation of LV pseudoaneurysm. The patient had successful surgical repair of the aneurysm and had a favorable outcome in 9 mo’ follow-up. The imaging modalities and surgical treatment of a pseudoaneurysm is discussed.
基金The study was approved by Research Ethics Committee at the Faculty of Medicine Ain Shams University(FWA 000017585).Approval No.FMASU M D 65/2019.
文摘Background:Bidirectional Glenn shunts have long been available as palliative procedures for patients with single ventricle physiology that is,patients unsuitable for biventricular repair.In our country they are performed at an older age than that recommended by the literature.So,we aim to assess post bidirectional Glenn shunt patients to detect the presence of any complications and to evaluate their functional capacity.Methods:This was a descriptive study that included all patients who underwent a bidirectional Glenn shunt and were referred for follow up in Ain shams university hospitals.History taking including NYHA class and physical activity,clinical examination,six-minutes-walk test,laboratory investigations,full echocardiographic assessment were done for all patients.Some patients needed invasive cardiac catheterization.Results:Our registry included 178 patients who underwent bidirectional Glenn procedure referred for follow up in Ain Shams university hospital from January 2019 till July 2020.The mean age of the registry was 18.7±8.26 years(range between 5 and 37 years),85 males(48%)and 93 females(52%).Regarding the basic anatomy,double outlet right ventricle with hypoplastic left ventricle was the commonest.Furthermore,our descriptive study confirmed many characteristic similarities between our patients and patients in developing countries.Our patients underwent bidirectional Glenn shunt at a median age of 6 years which is considered a relatively old age but similar to other studies that have been made in developing countries like Pakistan,India and Iran.There is a significant delay in the operation in Egyptian patients due to lack of patients’awareness,few numbers of primary health care facilities and high economic burden.Conclusion:Although the presence of slight systemic desaturation,our study demonstrated a satisfactory functional capacity among our patients;thus,bidirectional Glenn can be considered an acceptable definite univentricular repair in patients with late presentation.
文摘Objective: In recent years, attempting the biventricular pathway or biventricular conversions in patients with borderline ventricle has become a hot topic. However, inappropriate pursuit of biventricular repair in borderline candidates will lead to adverse clinical outcomes. Therefore, it is important to accurately assess the degree of ventricular development before operation and whether it can tolerate biventricular repair. This review evaluated ventricular development using echocardiography for a better prediction of biventricular repair in borderline ventricle. Data sources: Articles from January 1, 1990 to April 1, 2019 on biventricular repair in borderline ventricle were accessed from PubMed, using keywords including "borderline ventricle,""congenital heart disease,""CHD,""echocardiography," and "biventricular repair." Study selection: Original articles and critical reviews relevant to the review’s theme were selected. Results: Borderline left ventricle (LV):(1) Critical aortic stenosis: the Rhodes score, Congenital Heart Surgeons Society regression equation and another new scoring system was proposed to predict the feasibility of biventricular repair.(2) Aortic arch hypoplasia: the LV size and the diameter of aortic and mitral valve (MV) annulus should be taken into considerations for biventricular repair.(3) Right-dominant unbalanced atrioventricular septal defect (AVSD): atrioventricular valve index (AVVI), left ventricular inflow index (LVII), and right ventricle (RV)/LV inflow angle were the echocardiographic indices for biventricular repair. Borderline RV:(1) pulmonary atresia/intact ventricular septum (PA/IVS): the diameter z-score of tricuspid valve (TV) annulus, ratio of TV to MV diameter, RV inlet length z-score, RV area z-score, RV development index, and RV-TV index, etc. Less objective but more practical description is to classify the RV as tripartite, bipartite, and unipartite. The presence or absence of RV sinusoids, RV dependent coronary circulation, and the degree of tricuspid regurgitation should also be noted.(2) Left-dominant unbalanced AVSD: AVVI, LV, and RV volumes, whether apex forming ventricles were the echocardiographic indices for biventricular repair. Conclusions: Although the evaluation of echocardiography cannot guarantee the success of biventricular repair surgery, echocardiography can still provide relatively valuable basis for surgical decision making.
