Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospec...Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.展开更多
BACKGROUND Ductal spasm is a rare but life-threatening complication of cardiac catheterization in neonates with pulmonary atresia and an intact ventricular septum.In patients with ductal-dependent pulmonary blood flow...BACKGROUND Ductal spasm is a rare but life-threatening complication of cardiac catheterization in neonates with pulmonary atresia and an intact ventricular septum.In patients with ductal-dependent pulmonary blood flow,ductal spasm may lead to refractory hypoxemia and severe hemodynamic instability,which need to be treated in perfect order.CASE SUMMARY We present a male infant with a gestational age of 39 wk,and his fetal echocardiography showed pulmonary atresia.At 28 d of age,transcatheter pulmonary valvuloplasty with balloon dilatation was performed.Two hours after the operation,the patient's pulse oxygen saturation continued to decrease.The patient was then transferred to receive cardiac catheterization.During catheterization,the invasive blood pressure and pulse oxygen saturation suddenly decreased,and repeated aortography revealed partial occlusion of the ductus arteriosus.It no longer changed when pulse oxygen saturation rose to 51%after approximately 20 min of maintenance therapy.Therefore,a ductal stent was used for implantation.Hemodynamics and hypoxemia were improved.CONCLUSION We should know that ductal spasm may occur during pulmonary atresia and intact ventricular septum cardiac catheterization.Understand the pathophysiology of ductal-dependent pulmonary blood flow and make comprehensive perioperative preparations essential to deal with hemodynamic disorders caused by ductal spasm.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
Objective In patients with pulmonary atresia and intact ventricular septum (PAIVS) without right ventricular-dependent coronaries,catheter techniques including the use of a sniff wire,lasers,and radiofrequency have be...Objective In patients with pulmonary atresia and intact ventricular septum (PAIVS) without right ventricular-dependent coronaries,catheter techniques including the use of a sniff wire,lasers,and radiofrequency have been the most widely used as initial therapy. However,percutaneous perforation and balloon valvuloplasty were associated with higher rate of procedural failure and展开更多
Objective: To evaluate left ventricular regional and global systolic function by measuring left ventricular longitudinal strain (LS) in hypertensive patients with sigmoid-shaped interventricular septum (SIS) by two-di...Objective: To evaluate left ventricular regional and global systolic function by measuring left ventricular longitudinal strain (LS) in hypertensive patients with sigmoid-shaped interventricular septum (SIS) by two-dimensional speckle tracking (2D-STE);in order to explore whether the sigmoid-shaped interventricular septum affects the left ventricular systolic function in patients with hypertension. Methods: Routine echocardiographic parameters were measured in 30 hypertensive patients with SIS (SIS group) and 30 hypertensive patients without SIS (non-SIS group). The left ventricular segments and global LS were measured by 2D-STE, and the two sets of parameters were compared. Results: The value of the thickness of the basal segment of the interventricular septum (IVSBT), the thickness of the middle segment of the interventricular septum (IVSMT) and the ratio of the basal segment of the ventricular septum to the middle segment of the interventricular septum (IVSBT/IVSMT) in SIS group was higher than that in non-SIS group. However, the value of left ventricular outflow tract diameter (LVOTD) in SIS group was lower than that in non-SIS group. There was a significant difference between the two groups (all P Conclusion: SIS affects left ventricular regional systolic function of patients with hypertension. 2D-STE can early evaluate left ventricular longitudinal systolic function in hypertensive patients with SIS.展开更多
Current permanent right ventricular and right atrial endocardial pacing leads are implanted utilizing a central lumen stylet. Right ventricular apex pacing initiates an abnormal asynchronous electrical activation patt...Current permanent right ventricular and right atrial endocardial pacing leads are implanted utilizing a central lumen stylet. Right ventricular apex pacing initiates an abnormal asynchronous electrical activation pattern, which results in asynchronous ventricular contraction and relaxation. When pacing from right atrial appendage, the conduction time between two atria will be prolonged, which results in heterogeneity for both depolarization and repolarization. Six patients with Class Ⅰ indication for permanent pacing were implanted with either single chamber or dual chamber pacemaker. The SelectSecure 3830 4-French （Fr） lumenless lead and the SelectSite C304 8.5-Fr steerable catheter-sheath （Medtronic Inc., USA） were used. Pre-selected pacing sites included inter-atrial septum and right ventricular outflow tract, which were defined by ECG and fluoroscopic criteria. All the implanting procedures were successful without complication. Testing results （mean atrial pacing threshold： 0.87 V; mean P wave amplitude： 2.28 mV; mean ventricular pacing threshold： 0.53V; mean R wave amplitude： 8.75 mV） were satisfactory. It is concluded that implantation of a 4-Fr lumenless pacing lead by using a streerable catheter-sheath to achieve inter-atrial septum or right ventricular outflow tract pacing is safe and feasible.展开更多
Ventricular septum defects(VSDs)are common types of congenital heart diseases caused by developmental defect;they contribute to 25%-30%of all adult congenital heart diseases.The peroxisome proliferator-activated recep...Ventricular septum defects(VSDs)are common types of congenital heart diseases caused by developmental defect;they contribute to 25%-30%of all adult congenital heart diseases.The peroxisome proliferator-activated receptor gamma(PPAR-y)is widely expressed in mammalian tissues and in the immune system,regulating cell differentiation and immune and inflammatory responses.The PPAR-y gene has recently been found crucial for heart development,but the mechanism of action is not clear.This study aims to investigate the effects of the PPAR-y gene in the myocardium on the development of ventricular septation.In this study,we applied Cre-loxP recombination enzyme(CRE)technology to downregulate the expression of the PPAR-y gene in different cardiac tissues,RT-PCR to examine the expression of the c-fos and TGF-B!genes,and histology staining to check the defect of embryonic heart at embryonic day 14.5(E14.5).We found that the downregulation of the PPAR-p gene resulted in a ventricular membranous septation defect of the embryonic heart at E14.5.Furthermore,only conversion of a Tnt:Cre,but not Mef2c:Cre,Tie2:Cre,or Wnt:Cre PPAR-T floxed allele to a null allele resulted in VSD.PPAR-/mi-Orv+embryos showed increascs in atrioventricular(AV)-cushion cells and the expression of c-fos gene but no change in the expression of TGF-B1 at E10.5.Our study demonstrates PPAR-N in the myocardium is required for ventricular septation through regulation of AV-cushion cell proliferation by a Tntc-fos signal.展开更多
Although the majority of patients with perimembranous ventricular septal defect and septal aneurysm remained asymptomatic, some of them presented with serious complications during adulthood and thus required high risk...Although the majority of patients with perimembranous ventricular septal defect and septal aneurysm remained asymptomatic, some of them presented with serious complications during adulthood and thus required high risky surgery. In accordance with other rare condition, the incidence and natural history have not been well documented. This case describes the occurrence of a septic pulmonary emboli associated with right ventricular outflow tract obstruction in a young child.展开更多
Objective: We sought to test the hypothesis that the total QRS voltage without either set of the limb leads (I, II, III) or (R, L, F) may be a better indicator of LVH as compared to the total QRS voltage. Background: ...Objective: We sought to test the hypothesis that the total QRS voltage without either set of the limb leads (I, II, III) or (R, L, F) may be a better indicator of LVH as compared to the total QRS voltage. Background: The total 12 lead QRS voltage has been a validated electrocardiographic criterion for left ventricular hypertrophy (LVH), with an upper limit of175 mm. However, there is some redundancy in this measurement as the output of the limb leads is repeated because leads I, II, III, and R, L, F use the same three electrodes. Methods: 43 unselected, consecutive echocardiograms were examined for evidence of LVH by wall thickness. Electrocardiogram (ECG) of these patients within a week of the echocardiogram were then examined for the total 12 leads QRS voltage, minus I, II, III and total minus R, L, F voltages. ECG findings were then compared with corresponding echocardiographic dimensions. Results: A total QRS voltage of123 mmon ECG yielded a sensitivity of 73% and specificity of 67% for diagnosing LVH with 95% CI = 0.59 - 0.89, p = 0.007. Total minus (R, L and F) value of110 mmon ECG appears to give the best sensitivity (73%), specificity (72%), and accuracy (64% negative predictive value and 82% positive predictive value) for LVH. Conclusion: It appears that total QRS voltage minus either set of the limb leads, especially the total minus R, L and F is a better criterion, with110 mmbeing the best specific, sensitive and accurate index for diagnosing LVH.展开更多
文摘Objectives:To access the effectiveness of our modified right-ventricular overhauling procedure on tricuspid valve(TV)growth in patients with pulmonary atresia with intact ventricular septum(PAIVS).Methods:We retrospectively reviewed 21 patients with PAIVS who underwent modified right ventricular overhauling(mRVoh)between 2008 and 2019 at two institutions.Our mRVoh consisted of wide resection of hypertrophied infundibular and trabecular muscle,peeling off fibrotic endocardial tissue in the right ventricle(RV)cavity,surgical pulmonary valvotomy,and Blalock-Taussig shunt or banding of ductus arteriosus under cardiopulmonary bypass.The TV annulus sizes were measured and analyzed using echocardiography before and after mRVoh.Results:No mortalities were observed during a median follow-up of 3 years(interquartile range:1.3–4.7 years)of follow-up were noted.mRVoh was performed at a median age of 163.5 days(range:21–560 days),including seven neonates and two infants(<60 days).During follow-up,the median TV annular z-score increased significantly from−2.24 to−1.15 before and after mRVoh(p=0.004).In ten patients with a prior history of percutaneous interventions for RV outflow tract(RVOT)widening at least 6 months before mRVoh,the TV annular z-score significantly changed during the period after mRVoh(−2.03 to−1.61,p=0.028)compared with the period before mRVoh(−2.51→–2.03,p=0.575)after percutaneous intervention only.Conclusions:mRVoh in PAIVS patients was positively associated with TV annular growth,and it was more effective than percutaneous RVOT widening interventions without mRVoh.
文摘BACKGROUND Ductal spasm is a rare but life-threatening complication of cardiac catheterization in neonates with pulmonary atresia and an intact ventricular septum.In patients with ductal-dependent pulmonary blood flow,ductal spasm may lead to refractory hypoxemia and severe hemodynamic instability,which need to be treated in perfect order.CASE SUMMARY We present a male infant with a gestational age of 39 wk,and his fetal echocardiography showed pulmonary atresia.At 28 d of age,transcatheter pulmonary valvuloplasty with balloon dilatation was performed.Two hours after the operation,the patient's pulse oxygen saturation continued to decrease.The patient was then transferred to receive cardiac catheterization.During catheterization,the invasive blood pressure and pulse oxygen saturation suddenly decreased,and repeated aortography revealed partial occlusion of the ductus arteriosus.It no longer changed when pulse oxygen saturation rose to 51%after approximately 20 min of maintenance therapy.Therefore,a ductal stent was used for implantation.Hemodynamics and hypoxemia were improved.CONCLUSION We should know that ductal spasm may occur during pulmonary atresia and intact ventricular septum cardiac catheterization.Understand the pathophysiology of ductal-dependent pulmonary blood flow and make comprehensive perioperative preparations essential to deal with hemodynamic disorders caused by ductal spasm.
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Objective In patients with pulmonary atresia and intact ventricular septum (PAIVS) without right ventricular-dependent coronaries,catheter techniques including the use of a sniff wire,lasers,and radiofrequency have been the most widely used as initial therapy. However,percutaneous perforation and balloon valvuloplasty were associated with higher rate of procedural failure and
文摘Objective: To evaluate left ventricular regional and global systolic function by measuring left ventricular longitudinal strain (LS) in hypertensive patients with sigmoid-shaped interventricular septum (SIS) by two-dimensional speckle tracking (2D-STE);in order to explore whether the sigmoid-shaped interventricular septum affects the left ventricular systolic function in patients with hypertension. Methods: Routine echocardiographic parameters were measured in 30 hypertensive patients with SIS (SIS group) and 30 hypertensive patients without SIS (non-SIS group). The left ventricular segments and global LS were measured by 2D-STE, and the two sets of parameters were compared. Results: The value of the thickness of the basal segment of the interventricular septum (IVSBT), the thickness of the middle segment of the interventricular septum (IVSMT) and the ratio of the basal segment of the ventricular septum to the middle segment of the interventricular septum (IVSBT/IVSMT) in SIS group was higher than that in non-SIS group. However, the value of left ventricular outflow tract diameter (LVOTD) in SIS group was lower than that in non-SIS group. There was a significant difference between the two groups (all P Conclusion: SIS affects left ventricular regional systolic function of patients with hypertension. 2D-STE can early evaluate left ventricular longitudinal systolic function in hypertensive patients with SIS.
基金supported by a grant form a Program of Science and Technology Development of Hubei Province (2004AA304B09).
文摘Current permanent right ventricular and right atrial endocardial pacing leads are implanted utilizing a central lumen stylet. Right ventricular apex pacing initiates an abnormal asynchronous electrical activation pattern, which results in asynchronous ventricular contraction and relaxation. When pacing from right atrial appendage, the conduction time between two atria will be prolonged, which results in heterogeneity for both depolarization and repolarization. Six patients with Class Ⅰ indication for permanent pacing were implanted with either single chamber or dual chamber pacemaker. The SelectSecure 3830 4-French （Fr） lumenless lead and the SelectSite C304 8.5-Fr steerable catheter-sheath （Medtronic Inc., USA） were used. Pre-selected pacing sites included inter-atrial septum and right ventricular outflow tract, which were defined by ECG and fluoroscopic criteria. All the implanting procedures were successful without complication. Testing results （mean atrial pacing threshold： 0.87 V; mean P wave amplitude： 2.28 mV; mean ventricular pacing threshold： 0.53V; mean R wave amplitude： 8.75 mV） were satisfactory. It is concluded that implantation of a 4-Fr lumenless pacing lead by using a streerable catheter-sheath to achieve inter-atrial septum or right ventricular outflow tract pacing is safe and feasible.
基金This project was supported by Natural Science Foundation of Hubei Province,China(No.20152015CKB748)Independent Innovation Foundation of Huazhong University of Science and Technology,China(No.0118540198).
文摘Ventricular septum defects(VSDs)are common types of congenital heart diseases caused by developmental defect;they contribute to 25%-30%of all adult congenital heart diseases.The peroxisome proliferator-activated receptor gamma(PPAR-y)is widely expressed in mammalian tissues and in the immune system,regulating cell differentiation and immune and inflammatory responses.The PPAR-y gene has recently been found crucial for heart development,but the mechanism of action is not clear.This study aims to investigate the effects of the PPAR-y gene in the myocardium on the development of ventricular septation.In this study,we applied Cre-loxP recombination enzyme(CRE)technology to downregulate the expression of the PPAR-y gene in different cardiac tissues,RT-PCR to examine the expression of the c-fos and TGF-B!genes,and histology staining to check the defect of embryonic heart at embryonic day 14.5(E14.5).We found that the downregulation of the PPAR-p gene resulted in a ventricular membranous septation defect of the embryonic heart at E14.5.Furthermore,only conversion of a Tnt:Cre,but not Mef2c:Cre,Tie2:Cre,or Wnt:Cre PPAR-T floxed allele to a null allele resulted in VSD.PPAR-/mi-Orv+embryos showed increascs in atrioventricular(AV)-cushion cells and the expression of c-fos gene but no change in the expression of TGF-B1 at E10.5.Our study demonstrates PPAR-N in the myocardium is required for ventricular septation through regulation of AV-cushion cell proliferation by a Tntc-fos signal.
文摘Although the majority of patients with perimembranous ventricular septal defect and septal aneurysm remained asymptomatic, some of them presented with serious complications during adulthood and thus required high risky surgery. In accordance with other rare condition, the incidence and natural history have not been well documented. This case describes the occurrence of a septic pulmonary emboli associated with right ventricular outflow tract obstruction in a young child.
文摘Objective: We sought to test the hypothesis that the total QRS voltage without either set of the limb leads (I, II, III) or (R, L, F) may be a better indicator of LVH as compared to the total QRS voltage. Background: The total 12 lead QRS voltage has been a validated electrocardiographic criterion for left ventricular hypertrophy (LVH), with an upper limit of175 mm. However, there is some redundancy in this measurement as the output of the limb leads is repeated because leads I, II, III, and R, L, F use the same three electrodes. Methods: 43 unselected, consecutive echocardiograms were examined for evidence of LVH by wall thickness. Electrocardiogram (ECG) of these patients within a week of the echocardiogram were then examined for the total 12 leads QRS voltage, minus I, II, III and total minus R, L, F voltages. ECG findings were then compared with corresponding echocardiographic dimensions. Results: A total QRS voltage of123 mmon ECG yielded a sensitivity of 73% and specificity of 67% for diagnosing LVH with 95% CI = 0.59 - 0.89, p = 0.007. Total minus (R, L and F) value of110 mmon ECG appears to give the best sensitivity (73%), specificity (72%), and accuracy (64% negative predictive value and 82% positive predictive value) for LVH. Conclusion: It appears that total QRS voltage minus either set of the limb leads, especially the total minus R, L and F is a better criterion, with110 mmbeing the best specific, sensitive and accurate index for diagnosing LVH.
