Objective To evaluate left univentricular （LUV） pacing for cardiac resynchronization therapy （CRT） using a rate-adaptive atrioven- tricular delay （RAAVD） algorithm to track physiological atrioventricular delay ...Objective To evaluate left univentricular （LUV） pacing for cardiac resynchronization therapy （CRT） using a rate-adaptive atrioven- tricular delay （RAAVD） algorithm to track physiological atrioventricular delay （AVD）. Methods A total of 72 patients with congestive heart failure （CHF） were randomized to RAAVD LUV pacing versus standard biventricular （BiV） pacing in a 1 ： 1 ratio. Echocardiography was used to optimize AVD for both groups. The effects of sequential BiV pacing and LUV pacing with optimized A-V （right atrio-LV） delay using an RAAVD algorithm were compared. The standard deviation （SD） of the S/R ratio in lead VI at five heart rate （HR） segments （Rs/R-SD5）, defined as the ＂tracking index,＂ was used to evaluate the accuracy of the RAAVD algorithm for tracking physiological AVD. Results TheQRS complex duration （132 ± 9.8 vs. 138± 10ms, P 〈 0.05）, the time required for optimization （21 ±5 vs. 50±8min, P〈 0.001）, the mitral regurgitant area （1.9 ± 1.1 vs. 2.5 ± 1.3 em2, P 〈 0.05）, the interventricular mechanical delay time （60.7 ± 13.3 ms vs. 68.3 ± 14.2 ms, P 〈 0.05）, and the average annual cost （13,200 ± 1000 vs. 21,600 ± 2000 RMB, P 〈 0.001） in the RAAVD LUV pacing group were significantly less than those in the standard BiV pacing group. The aortic valve velocity-time integral in the RAAVD LUV pacing group was greater than that in the standard BiV pacing group （22.7 ± 2.2 vs. 21.4 ± 2.1 cm, P 〈 0.05）. The Rs/R-SD5 was 4.08 ± 1.91 in the RAAVD LUV pacing group, and was significantly negatively correlated with improved left ventricular ejection fraction （LVEF） （ALVEF, Pearson＇s r = -0.427, P = 0.009）, and positively correlated with New York Heart Association class （Spearman＇s r - 0.348, P 0.037）. Conclusions RAAVD LUV pacing is as effective as standard BiV pacing, can be more physiological than standard BiV pacing, and can de- crease the average annual cost of CRT.展开更多
Magnetic resonance imaging can be used for preprocedural assessment of complex anatomy for radiofrequency(RF) ablations,e.g.,in a univentricular heart.This case report features the treatment of a young patient with a ...Magnetic resonance imaging can be used for preprocedural assessment of complex anatomy for radiofrequency(RF) ablations,e.g.,in a univentricular heart.This case report features the treatment of a young patient with a functionally univentricular heart who suffered from persistent sudden onset tachycardia with wide complexes that required RF ablation as treatment.展开更多
It is very important to understand that the univentricular heart surgery is just palliative, not being in anyway a definitive or curative surgery, but nowadays it’s the best initial treatment of this complex heart di...It is very important to understand that the univentricular heart surgery is just palliative, not being in anyway a definitive or curative surgery, but nowadays it’s the best initial treatment of this complex heart disease. The fundamental philosophy of treatment of every univentricular heart is to ensure the flow system and/or restrict the lung flow. Thus, initially a patient with univentricular heart who is undergoing surgery may need to ensure systemic flow (reconstruction of the aortic?arch type Norwood), to restrict the lung flow (pulmonary banding) or to provide enough?pulmonary flow (pulmonary-systemic fistulae). However, some heart diseases with univentricular physiology remain “balanced” autonomously, until the “second” stage of palliation is performed (cavo-pulmonary anastomosis type Glenn), but others require performance of pulmonary banding, if there’s no native lung protection and/or repair of the systemic circuit in a first stage, to reach next palliation steps in the best possible conditions.展开更多
Objective To compare the postoperative outcomes of patients with the diagnostic univentricular heart undergoing lateral tunnel （LT） operation with extracardiac conduit （EC） operation. Methods- From June 1996 to Ju...Objective To compare the postoperative outcomes of patients with the diagnostic univentricular heart undergoing lateral tunnel （LT） operation with extracardiac conduit （EC） operation. Methods- From June 1996 to July 2007, 114 consecutive patients with a single ventricle underwent total cavopulmonary connection （TCPC） in Children＇s Heart Center, lAniversity Hospital Giessen and Marburg GmbH, Germany. A LT was performed in 19 （16.7%） patients, and an EC in 95 （83.3%） patients. The mean age of EC group was 50.8±31.6 （ranging from 22 to 212） months, and that of LT group was 61.5±41.2 （ranging from 30 to 168） months. Early and midterm outcomes of two groups were analyzed.Results One died in LT group （5.3%） and three in EC group （3.2%）. The overall mortality was 3.5%. There was no significant difference in mortality between EC-and-LT groups （P〉0.05）. The postoperative pulmonary arterial pressure, oxygen saturation, and effusion time of two groups had no significant difference （all P〉0.05）. No significant difference in the occurrences of complications （arrhythmias, enteropathy, and thrombosis） was found between two groups after operation （P〉0.05）. Conclusions There seems no difference between LT and EC in the clinical results in the early and middle postoperative stage. Glenn anastomosis followed by an EC seems to have some advantages.展开更多
Background:Shunt repair for the Norwood procedure with either the right ventricle to pulmonary artery shunt(RVPAS)or the modified Blalock-Taussig-Thomas Shunt(BTTS)varies by institution and surgeon preference.Shunt ch...Background:Shunt repair for the Norwood procedure with either the right ventricle to pulmonary artery shunt(RVPAS)or the modified Blalock-Taussig-Thomas Shunt(BTTS)varies by institution and surgeon preference.Shunt choice has been informed by landmark trials including the Single Ventricle Reconstruction trial and modern outcomes data may engender future complementary studies.Methods:We conducted a retrospective analysis of all patients who underwent the Norwood procedure from 2014–2022 at a single center to compare outcomes by shunt type.The primary outcome measure was freedom from death or transplant.Secondary outcome measures included hospital length of stay,complications,and unplanned interventions.Results:93 patients underwent the Norwood procedure at a median age of 7 days(IQR 5,9)and 39 weeks gestation(IQR 38,39).67.7%had hypoplastic left heart syndrome.39 patients received a BTTS compared to 54 RVPAS.There was no difference in operative mortality(BTTS 12.8%,RVPAS 9.3%,p=0.58),death or transplant at 1 year(BTTS 15.4%,RVPAS 7.4%,p=0.31),or between 1 and 3 years(BTTS 0%,RVPAS 5.6%,p=0.26).There was a significantly higher rate of pulmonary arterial stenting in the RVPAS group(BTTS 1.21/100 patient-years,RVPAS 15.68/100 patient-years,p=0.01).Conclusions:Similar short-and medium-term survival were seen in BTTS and RVPAS groups with fewer pulmonary artery interventions for BTTS,though our study is underpowered to suggest superior freedom from interventions.These results may serve as a hypothesis-generating study to revisit the SVR trial with a modern cohort in the setting of improved surgical technique and perioperative management.展开更多
Objective:Modifications of the Fontan operation,which are also known as total cavopulmonary connection(TCPC),are widely applied for patients with functionally univentricular hearts(FUH).Herein,we summed up the differe...Objective:Modifications of the Fontan operation,which are also known as total cavopulmonary connection(TCPC),are widely applied for patients with functionally univentricular hearts(FUH).Herein,we summed up the different surgical pathways and clinical outcomes in FUH patients with apicocaval juxtaposition(ACJ)or/and separated hepatic venous(SHV)drainage.Methods:Between January 2009 and December 2019,123 patients who undergone TCPC in our institute were included in this retrospective study.We have included 70 patients with ACJ(Group 1)and 53 patients with SHV(Group 2).Moreover,Group 2 included 17 cases combing with ACJ(32.1%).In Group 1,three different TCPC methods were conducted.While 45 cases were conducted with the extracardiac conduit-TCPC(EC-TCPC)method,24 cases used the intracardiac conduit-TCPC(IC-TCPC)method,and only one case used the lateral tunnel-TCPC(LT-TCPC).In Group 2,four TCPC methods were conducted on patients.Forty cases used the EC-TCPC-common open technique,6 cases with IC-TCPC technique,4 cases with LT-TCPC,and 3 cases with intra-extracardiac conduit-TCPC(IEC-TCPC).Results:There were 7 patients in Group 1 and 14 patients in Group 2 who required early re-operation during hospitalization(p<0.05).Postoperative mean pulmonary artery pressure(mPAP)greater than 15 mmHg emerged as a predictor for early re-operation(p<0.01)and early death(p<0.001)in univariate analysis.Conclusions:TCPC can be performed in these patients and shows beneficial results.Under the Fontan principle of connecting systemic venous to the pulmonary vasculature unimpededly,surgeons should carefully evaluate three components when choosing for the surgical technique:The distance between inferior vena cava(IVC)and the apex;the site of the vertebrae relative to the ACJ;the distance between ACJ and SHV if coexisting.However,the technique should be altered when the postoperative mPAP was greater than 15 mmHg.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
BACKGROUND There is risk of stenosis and thrombosis of the superior vena cava after upper extremity central catheter replacement.This complication is more serious among patients with single ventricle physiology,as it ...BACKGROUND There is risk of stenosis and thrombosis of the superior vena cava after upper extremity central catheter replacement.This complication is more serious among patients with single ventricle physiology,as it might preclude them from undergoing further life-sustaining palliative surgery.AIM To describe complications associated with the use of upper extremity percutaneous intravenous central catheters(PICCs)in children with single ventricle physiology.METHODS A single institution retrospective review of univentricular patients who underwent superior cavopulmonary anastomoses as their stage 2 palliation procedure from January 2014 until December 2018 and had upper body PICCs placed at any point prior to this procedure.Clinical data including ultrasonography,cardiac catheterization,echocardiogram reports and patient notes were used to determine the presence of thrombus or stenosis of the upper extremity and cervical vessels.Data regarding the presence and duration of upper extremity PICCs and upper extremity central venous catheter(CVC),and use of anticoagulation were recorded.RESULTS Seventy-six patients underwent superior cavopulmonary anastomoses,of which 56(73%)had an upper extremity PICC at some point prior to this procedure.Median duration of PICC usage was 24 d(25%,75%:12,39).Seventeen patients(30%)with PICCs also had internal jugular or subclavian central venous catheters(CVCs)in place at some point prior to their superior cavopulmonary anastomoses,median duration 10 d(25%,75%:8,14).Thrombus was detected in association with 2 of the 56 PICCs(4%)and 3 of the 17 CVCs(18%).All five patients were placed on therapeutic dose of low molecular weight heparin at the time of thrombus detection and subsequent cardiac catheterization demonstrated resolution in three of the five patients.No patients developed clinically significant venous stenosis.CONCLUSION Use of upper extremity PICCs in patients with single ventricle physiology prior to super cavopulmonary anastomosis is associated with a low rate of catheterassociated thrombosis.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
Background: The primary concern of the Fontan procedure has been atrial arrhythmias, but little attention has been given to hemodynamic changes. This study’s purpose was to examine the hemodynamics and arrhythmias of...Background: The primary concern of the Fontan procedure has been atrial arrhythmias, but little attention has been given to hemodynamic changes. This study’s purpose was to examine the hemodynamics and arrhythmias of the Intra/extracardiac fenestrated Fontan, and determine any advantages/disadvantages of this newer Fontan procedure. Methods: Patients undergoing the intra/ extracardiac fenestrated Fontan procedure at this institute between December 2009 and June 2017 were included in this retrospective evaluation of preoperative and postoperative hemodynamics during cardiac catheterization. End Diastolic Pressure (EDP), Pulmonary Artery Pressure (PAP), Left Atrial Pressure (LAP), and Transpulmonary Gradient (TPG) were the hemodynamic markers of interest. Occurrence of arrhythmias was also examined preoperatively, less than 2 weeks postoperatively, and greater than 2 weeks postoperatively. Morbidities and mortalities were also evaluated. Results: Fourteen patients underwent the procedure between December 2009 and June 2017. Preoperative data was available in all of these patients. Postoperative data was partially incomplete for atrial arrhythmias and hemodynamic data. EDP rose from 9.29 ± 3.50 mmHg to 10.33 ± 3.12 mmHg. PAP rose from 12.57 ± 2.50 mmHg to 14.27 mmHg. TPG dropped from 5.00 ± 2.29 mmHg to 3.25 ± 1.67 mmHg. LAP rose from 7.57 ± 2.87 mmHg to 10.30 ± 1.95 mmHg. Atrioventricular valve (AVV) regurgitation remained the same pre and postoperatively. 8 of 13 patients developed arrhythmias at less than 2 weeks postoperatively, and 5 of 12 patients developed arrhythmias at greater than 2 weeks postoperatively. There were no morbidities or mortalities. Conclusions: We found a statistically significant change in EDP, PAP, LAP, and TPG levels. It is unclear whether this is an advantage or disadvantage. The significance of these changes is unclear in this small population of patients. Further evaluation of hemodynamics, arrhythmias, morbidities and mortalities associated with the intra/extracardiac Fontan procedure is needed.展开更多
<u>Background:</u><span style="font-family:Verdana;"> Extracorporeal</span><span style="font-family:;" "=""> </span><span style="font-fami...<u>Background:</u><span style="font-family:Verdana;"> Extracorporeal</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">membrane oxygenation is a rescue life support technique used in life threatening</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">conditions of refractory respiratory and/or cardiac distress. Indication for extracorporeal life support in children</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">depends on age and varies from pulmonary to cardiac pathologies. In some cases</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> it may be used as a bridge to a</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">therapeutic procedure.</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">We described here the management of respiratory failure due to hemoptysis in a child with a Fontan circulation</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">and veno-venous extracorporeal membrane oxygenation which served as a bridge to angio-embolization. Hemoptysis can be a life threatening condition which can lead to hypovolemic shock and impaired alveolar gas</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">exchange. The latter can result in respiratory failure and consequent asphyxia.</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">When hemoptysis occurs in a patient with a univentricular</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">heart and a Fontan circulation, management of this</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">clinical situation can be challenging due to the particular physiology of the latter. Total cavopulmonary connection is a palliative surgical repair which constitutes Fontan circulation as a definitive treatment in patients with a univentricular heart. </span><u><span style="font-family:Verdana;">Methods:</span></u><span style="font-family:Verdana;"> Case report description of a 16 year-old boy with a univentricular heart and a Fontan circulation</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">who presented hemoptysis managed with a veno-venous extracorporeal membrane oxygenation (ECMO) as a</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">bridge to angio-embolization. </span><u><span style="font-family:Verdana;">Results:</span></u><span style="font-family:Verdana;"> Hemoptysis due to diffuse intra-alveolar hemorrhage from collateral circulation was successfully treated</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">in this young patient with pulmonary vascular embolization. This allowed to wean the patient from</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">extra-corporeal membrane oxygenation. </span><u><span style="font-family:Verdana;">Conclusion:</span></u><span style="font-family:Verdana;"> Veno-venous ECMO can be life-saving as a bridge to angio-embolization for severe hemoptysis in</span></span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">patients with Fontan circulation. The reported case allows to underline that </span><span style="font-family:Verdana;">our multidisciplinary approach in</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">this complex pediatric patient surely</span><span style="font-family:Verdana;"> participated to improve outcome.</span>展开更多
文摘Objective To evaluate left univentricular （LUV） pacing for cardiac resynchronization therapy （CRT） using a rate-adaptive atrioven- tricular delay （RAAVD） algorithm to track physiological atrioventricular delay （AVD）. Methods A total of 72 patients with congestive heart failure （CHF） were randomized to RAAVD LUV pacing versus standard biventricular （BiV） pacing in a 1 ： 1 ratio. Echocardiography was used to optimize AVD for both groups. The effects of sequential BiV pacing and LUV pacing with optimized A-V （right atrio-LV） delay using an RAAVD algorithm were compared. The standard deviation （SD） of the S/R ratio in lead VI at five heart rate （HR） segments （Rs/R-SD5）, defined as the ＂tracking index,＂ was used to evaluate the accuracy of the RAAVD algorithm for tracking physiological AVD. Results TheQRS complex duration （132 ± 9.8 vs. 138± 10ms, P 〈 0.05）, the time required for optimization （21 ±5 vs. 50±8min, P〈 0.001）, the mitral regurgitant area （1.9 ± 1.1 vs. 2.5 ± 1.3 em2, P 〈 0.05）, the interventricular mechanical delay time （60.7 ± 13.3 ms vs. 68.3 ± 14.2 ms, P 〈 0.05）, and the average annual cost （13,200 ± 1000 vs. 21,600 ± 2000 RMB, P 〈 0.001） in the RAAVD LUV pacing group were significantly less than those in the standard BiV pacing group. The aortic valve velocity-time integral in the RAAVD LUV pacing group was greater than that in the standard BiV pacing group （22.7 ± 2.2 vs. 21.4 ± 2.1 cm, P 〈 0.05）. The Rs/R-SD5 was 4.08 ± 1.91 in the RAAVD LUV pacing group, and was significantly negatively correlated with improved left ventricular ejection fraction （LVEF） （ALVEF, Pearson＇s r = -0.427, P = 0.009）, and positively correlated with New York Heart Association class （Spearman＇s r - 0.348, P 0.037）. Conclusions RAAVD LUV pacing is as effective as standard BiV pacing, can be more physiological than standard BiV pacing, and can de- crease the average annual cost of CRT.
文摘Magnetic resonance imaging can be used for preprocedural assessment of complex anatomy for radiofrequency(RF) ablations,e.g.,in a univentricular heart.This case report features the treatment of a young patient with a functionally univentricular heart who suffered from persistent sudden onset tachycardia with wide complexes that required RF ablation as treatment.
文摘It is very important to understand that the univentricular heart surgery is just palliative, not being in anyway a definitive or curative surgery, but nowadays it’s the best initial treatment of this complex heart disease. The fundamental philosophy of treatment of every univentricular heart is to ensure the flow system and/or restrict the lung flow. Thus, initially a patient with univentricular heart who is undergoing surgery may need to ensure systemic flow (reconstruction of the aortic?arch type Norwood), to restrict the lung flow (pulmonary banding) or to provide enough?pulmonary flow (pulmonary-systemic fistulae). However, some heart diseases with univentricular physiology remain “balanced” autonomously, until the “second” stage of palliation is performed (cavo-pulmonary anastomosis type Glenn), but others require performance of pulmonary banding, if there’s no native lung protection and/or repair of the systemic circuit in a first stage, to reach next palliation steps in the best possible conditions.
文摘Objective To compare the postoperative outcomes of patients with the diagnostic univentricular heart undergoing lateral tunnel （LT） operation with extracardiac conduit （EC） operation. Methods- From June 1996 to July 2007, 114 consecutive patients with a single ventricle underwent total cavopulmonary connection （TCPC） in Children＇s Heart Center, lAniversity Hospital Giessen and Marburg GmbH, Germany. A LT was performed in 19 （16.7%） patients, and an EC in 95 （83.3%） patients. The mean age of EC group was 50.8±31.6 （ranging from 22 to 212） months, and that of LT group was 61.5±41.2 （ranging from 30 to 168） months. Early and midterm outcomes of two groups were analyzed.Results One died in LT group （5.3%） and three in EC group （3.2%）. The overall mortality was 3.5%. There was no significant difference in mortality between EC-and-LT groups （P〉0.05）. The postoperative pulmonary arterial pressure, oxygen saturation, and effusion time of two groups had no significant difference （all P〉0.05）. No significant difference in the occurrences of complications （arrhythmias, enteropathy, and thrombosis） was found between two groups after operation （P〉0.05）. Conclusions There seems no difference between LT and EC in the clinical results in the early and middle postoperative stage. Glenn anastomosis followed by an EC seems to have some advantages.
文摘Background:Shunt repair for the Norwood procedure with either the right ventricle to pulmonary artery shunt(RVPAS)or the modified Blalock-Taussig-Thomas Shunt(BTTS)varies by institution and surgeon preference.Shunt choice has been informed by landmark trials including the Single Ventricle Reconstruction trial and modern outcomes data may engender future complementary studies.Methods:We conducted a retrospective analysis of all patients who underwent the Norwood procedure from 2014–2022 at a single center to compare outcomes by shunt type.The primary outcome measure was freedom from death or transplant.Secondary outcome measures included hospital length of stay,complications,and unplanned interventions.Results:93 patients underwent the Norwood procedure at a median age of 7 days(IQR 5,9)and 39 weeks gestation(IQR 38,39).67.7%had hypoplastic left heart syndrome.39 patients received a BTTS compared to 54 RVPAS.There was no difference in operative mortality(BTTS 12.8%,RVPAS 9.3%,p=0.58),death or transplant at 1 year(BTTS 15.4%,RVPAS 7.4%,p=0.31),or between 1 and 3 years(BTTS 0%,RVPAS 5.6%,p=0.26).There was a significantly higher rate of pulmonary arterial stenting in the RVPAS group(BTTS 1.21/100 patient-years,RVPAS 15.68/100 patient-years,p=0.01).Conclusions:Similar short-and medium-term survival were seen in BTTS and RVPAS groups with fewer pulmonary artery interventions for BTTS,though our study is underpowered to suggest superior freedom from interventions.These results may serve as a hypothesis-generating study to revisit the SVR trial with a modern cohort in the setting of improved surgical technique and perioperative management.
文摘Objective:Modifications of the Fontan operation,which are also known as total cavopulmonary connection(TCPC),are widely applied for patients with functionally univentricular hearts(FUH).Herein,we summed up the different surgical pathways and clinical outcomes in FUH patients with apicocaval juxtaposition(ACJ)or/and separated hepatic venous(SHV)drainage.Methods:Between January 2009 and December 2019,123 patients who undergone TCPC in our institute were included in this retrospective study.We have included 70 patients with ACJ(Group 1)and 53 patients with SHV(Group 2).Moreover,Group 2 included 17 cases combing with ACJ(32.1%).In Group 1,three different TCPC methods were conducted.While 45 cases were conducted with the extracardiac conduit-TCPC(EC-TCPC)method,24 cases used the intracardiac conduit-TCPC(IC-TCPC)method,and only one case used the lateral tunnel-TCPC(LT-TCPC).In Group 2,four TCPC methods were conducted on patients.Forty cases used the EC-TCPC-common open technique,6 cases with IC-TCPC technique,4 cases with LT-TCPC,and 3 cases with intra-extracardiac conduit-TCPC(IEC-TCPC).Results:There were 7 patients in Group 1 and 14 patients in Group 2 who required early re-operation during hospitalization(p<0.05).Postoperative mean pulmonary artery pressure(mPAP)greater than 15 mmHg emerged as a predictor for early re-operation(p<0.01)and early death(p<0.001)in univariate analysis.Conclusions:TCPC can be performed in these patients and shows beneficial results.Under the Fontan principle of connecting systemic venous to the pulmonary vasculature unimpededly,surgeons should carefully evaluate three components when choosing for the surgical technique:The distance between inferior vena cava(IVC)and the apex;the site of the vertebrae relative to the ACJ;the distance between ACJ and SHV if coexisting.However,the technique should be altered when the postoperative mPAP was greater than 15 mmHg.
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BACKGROUND There is risk of stenosis and thrombosis of the superior vena cava after upper extremity central catheter replacement.This complication is more serious among patients with single ventricle physiology,as it might preclude them from undergoing further life-sustaining palliative surgery.AIM To describe complications associated with the use of upper extremity percutaneous intravenous central catheters(PICCs)in children with single ventricle physiology.METHODS A single institution retrospective review of univentricular patients who underwent superior cavopulmonary anastomoses as their stage 2 palliation procedure from January 2014 until December 2018 and had upper body PICCs placed at any point prior to this procedure.Clinical data including ultrasonography,cardiac catheterization,echocardiogram reports and patient notes were used to determine the presence of thrombus or stenosis of the upper extremity and cervical vessels.Data regarding the presence and duration of upper extremity PICCs and upper extremity central venous catheter(CVC),and use of anticoagulation were recorded.RESULTS Seventy-six patients underwent superior cavopulmonary anastomoses,of which 56(73%)had an upper extremity PICC at some point prior to this procedure.Median duration of PICC usage was 24 d(25%,75%:12,39).Seventeen patients(30%)with PICCs also had internal jugular or subclavian central venous catheters(CVCs)in place at some point prior to their superior cavopulmonary anastomoses,median duration 10 d(25%,75%:8,14).Thrombus was detected in association with 2 of the 56 PICCs(4%)and 3 of the 17 CVCs(18%).All five patients were placed on therapeutic dose of low molecular weight heparin at the time of thrombus detection and subsequent cardiac catheterization demonstrated resolution in three of the five patients.No patients developed clinically significant venous stenosis.CONCLUSION Use of upper extremity PICCs in patients with single ventricle physiology prior to super cavopulmonary anastomosis is associated with a low rate of catheterassociated thrombosis.
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Background: The primary concern of the Fontan procedure has been atrial arrhythmias, but little attention has been given to hemodynamic changes. This study’s purpose was to examine the hemodynamics and arrhythmias of the Intra/extracardiac fenestrated Fontan, and determine any advantages/disadvantages of this newer Fontan procedure. Methods: Patients undergoing the intra/ extracardiac fenestrated Fontan procedure at this institute between December 2009 and June 2017 were included in this retrospective evaluation of preoperative and postoperative hemodynamics during cardiac catheterization. End Diastolic Pressure (EDP), Pulmonary Artery Pressure (PAP), Left Atrial Pressure (LAP), and Transpulmonary Gradient (TPG) were the hemodynamic markers of interest. Occurrence of arrhythmias was also examined preoperatively, less than 2 weeks postoperatively, and greater than 2 weeks postoperatively. Morbidities and mortalities were also evaluated. Results: Fourteen patients underwent the procedure between December 2009 and June 2017. Preoperative data was available in all of these patients. Postoperative data was partially incomplete for atrial arrhythmias and hemodynamic data. EDP rose from 9.29 ± 3.50 mmHg to 10.33 ± 3.12 mmHg. PAP rose from 12.57 ± 2.50 mmHg to 14.27 mmHg. TPG dropped from 5.00 ± 2.29 mmHg to 3.25 ± 1.67 mmHg. LAP rose from 7.57 ± 2.87 mmHg to 10.30 ± 1.95 mmHg. Atrioventricular valve (AVV) regurgitation remained the same pre and postoperatively. 8 of 13 patients developed arrhythmias at less than 2 weeks postoperatively, and 5 of 12 patients developed arrhythmias at greater than 2 weeks postoperatively. There were no morbidities or mortalities. Conclusions: We found a statistically significant change in EDP, PAP, LAP, and TPG levels. It is unclear whether this is an advantage or disadvantage. The significance of these changes is unclear in this small population of patients. Further evaluation of hemodynamics, arrhythmias, morbidities and mortalities associated with the intra/extracardiac Fontan procedure is needed.
文摘<u>Background:</u><span style="font-family:Verdana;"> Extracorporeal</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">membrane oxygenation is a rescue life support technique used in life threatening</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">conditions of refractory respiratory and/or cardiac distress. Indication for extracorporeal life support in children</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">depends on age and varies from pulmonary to cardiac pathologies. In some cases</span><span style="font-family:Verdana;">,</span><span style="font-family:Verdana;"> it may be used as a bridge to a</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">therapeutic procedure.</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">We described here the management of respiratory failure due to hemoptysis in a child with a Fontan circulation</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">and veno-venous extracorporeal membrane oxygenation which served as a bridge to angio-embolization. Hemoptysis can be a life threatening condition which can lead to hypovolemic shock and impaired alveolar gas</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">exchange. The latter can result in respiratory failure and consequent asphyxia.</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">When hemoptysis occurs in a patient with a univentricular</span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">heart and a Fontan circulation, management of this</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">clinical situation can be challenging due to the particular physiology of the latter. Total cavopulmonary connection is a palliative surgical repair which constitutes Fontan circulation as a definitive treatment in patients with a univentricular heart. </span><u><span style="font-family:Verdana;">Methods:</span></u><span style="font-family:Verdana;"> Case report description of a 16 year-old boy with a univentricular heart and a Fontan circulation</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">who presented hemoptysis managed with a veno-venous extracorporeal membrane oxygenation (ECMO) as a</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">bridge to angio-embolization. </span><u><span style="font-family:Verdana;">Results:</span></u><span style="font-family:Verdana;"> Hemoptysis due to diffuse intra-alveolar hemorrhage from collateral circulation was successfully treated</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">in this young patient with pulmonary vascular embolization. This allowed to wean the patient from</span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">extra-corporeal membrane oxygenation. </span><u><span style="font-family:Verdana;">Conclusion:</span></u><span style="font-family:Verdana;"> Veno-venous ECMO can be life-saving as a bridge to angio-embolization for severe hemoptysis in</span></span><span style="font-family:;" "=""> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">patients with Fontan circulation. The reported case allows to underline that </span><span style="font-family:Verdana;">our multidisciplinary approach in</span></span><span style="font-family:;" "=""> </span><span style="font-family:Verdana;">this complex pediatric patient surely</span><span style="font-family:Verdana;"> participated to improve outcome.</span>
