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
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 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
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
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 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
