The case report presented in this edition by Mu et al.The report presents a case of atrial septal defect(ASD)associated with electrocardiographic changes,noting that the crochetage sign resolved after Selective His Bu...The case report presented in this edition by Mu et al.The report presents a case of atrial septal defect(ASD)associated with electrocardiographic changes,noting that the crochetage sign resolved after Selective His Bundle Pacing(S-HBP)without requiring surgical closure.The mechanisms behind the appearance and resolution of the crochetage sign remain unclear.The authors observed the dis-appearance of the crochetage sign post-S-HBP,suggesting a possible correlation between these specific R waves and the cardiac conduction system.This editorial aims to explore various types of ASD and their relationship with the cardiac con-duction system,highlighting the diagnostic significance of the crochetage sign in ASD.展开更多
Background:Closure of an atrial septal defect(ASD)reduces right-side heart volumes by abolishing shunting with simultaneous improvement of the left ventricle(LV)filling and functions due to ventricular interdependence,...Background:Closure of an atrial septal defect(ASD)reduces right-side heart volumes by abolishing shunting with simultaneous improvement of the left ventricle(LV)filling and functions due to ventricular interdependence,thereby improving symptoms.Furthermore,studies conducted on atrial volume changes after ASD closure are limited.Cardiac magnetic resonance(CMR)is considered as the gold standard method for measuring cardiac volume and mass.Objective:We aimed to study the effect of transcatheter and surgical closure of secundum ASD on cardiac volumes and systolic functions as well as the fate of tricuspid regurgitation(TR),using CMR analysis.Methods:We prospectively enrolled 30 adult patients with isolated secundum ASD who were referred to ASD closure.CMR evaluation of cardiac chambers indexed volumes,systolic function,myocardial mass index,and tricuspid regurgitant fraction were done at before and 6 months after closure.Results:RV volumes decreased in both groups when compared to baseline(p-value 0.001),the device group had more reduction in volumes and more improvement in RV function after closure(p-value 0.001)when compared to the surgical arm.The changes in the RV mass index were insignificant between both groups(p-value 0.31).Functional TR improved to the same extent in both groups.Left ventricular end diastolic volume index(LVEDVI)and LV mass index increased sig-nificantly in both groups when compared to baseline in both groups but with no difference between groups p-value 0.01),left ventricular end systolic volume index(LVESVI)changes were insignificant.LV systolic function improved in patients who underwent device closure only(63.53±3.85 vs.67.13±4.34,p-value 0.01).There was a significant reduction in right atrial(RA)volumes and an insignificant decrease in left atrial(LA)volumes,with no difference between groups.Conclusion:Transcatheter and surgical secundum ASD closure resulted in volumetric changes in some cardiac chambers with better improvement in bi-ventricular systolic function in the transcatheter arm and no difference in the TR reduction between the two groups at 6 months follow-up by CMR.展开更多
Background:There is no gold standard sedation method for pediatric cardiac catheterization.In congenital heart diseases with intracardiac shunts,hemodynamic parameters are prone to change depending on the ventilation ...Background:There is no gold standard sedation method for pediatric cardiac catheterization.In congenital heart diseases with intracardiac shunts,hemodynamic parameters are prone to change depending on the ventilation conditions and anesthetics,although few studies have examined these effects.The purpose of this study was to investigate the effects of two different sedation methods on the hemodynamic parameters.Methods:This study retrospectively evaluated consecutive patients with ventricular septal defect(VSD)below 1 year of age who underwent cardiac catheterization at Aichi Children’s Health and Medical Center,who were divided into age-and VSD diameter-matched general anesthesia(GA)and monitored anesthesia care(MAC)under the natural airway groups(n=40 each),for comparison of hemodynamic parameters.Results:In the GA group,arterial blood pH and arterial partial pressure of oxygen were significantly higher(p<0.01),whereas arterial partial pressure of carbon dioxide was significantly lower than in the MAC group(p<0.01).Mean pulmonary artery pressure(p<0.05)and systemic blood pressure(p<0.01)were lower in the GA group.Pulmonary vascular resistance index(p<0.01)and systemic vascular resistance index(p<0.01)were also significantly lower in the GA group than the MAC group.There were no significant differences in pulmonary blood flow index,systemic blood flow index,and pulmonary/systemic blood flow ratio between the two groups.Conclusions:Cardiac catheterization under GA in VSD patients results in different hemodynamic parameters compared to that under MAC.In particular,when using pulmonary artery pressure and pulmonary vascular resistance measured under GA for judgment regarding the surgical indications or perioperative management,consideration should be given to the fact that these parameters might be lower compared to those measured under MAC.展开更多
Li-Fraumeni syndrome(LFS)is a well-defined autosomal dominant predisposition syndrome due to TP53 germline mutation that causes many cancer malig-nancies.This early-onset syndrome poses a state of widespread malignanc...Li-Fraumeni syndrome(LFS)is a well-defined autosomal dominant predisposition syndrome due to TP53 germline mutation that causes many cancer malig-nancies.This early-onset syndrome poses a state of widespread malignancy.Such an inherited condition possessing defective p53,guardian of the genome,in the germline has the potential to cause multiple cancers by predominantly affecting mesenchyme(connective tissues,blood cells),breast,brain,and adrenal cortex organs.The tumors initially identified in LFS can eventually propagate to cause secondary malignancies.LFS contributes to multiple cancers in individuals with defective p53 inheritance.When suspected to possess any mass,patients with other co-morbidities,in particular those with certain cardiovascular conditions,undergo screening using high-throughput techniques like transthoracic and transesophageal echocardiography or cardiothoracic magnetic resonance imaging to locate and interpret the size of the mass.In LFS cases,it is certain to presume these masses as cancers and plan their management employing invasive surgeries after performing all efficient diagnostic tools.There are only poor predictions to rule out the chances of any other pathology.This criterion emphasizes the necessity to speculate alternative precision diagnostic methods to affirm such new growth or masses encountered in LFS cases.Moreover,it has all the possibilities to ultimately influence surgical procedures that may be invasive or complicate operative prognosis.Hence,it is essential to strategize an ideal protocol to diagnose any new unexplored mass in the LFS community.In this editorial,we discuss the importance of diagnostic approaches on naïve pristine masses in LFS.展开更多
Percutaneous left atrial appendage(LAA)occlusion evolved as an alternative treatment to the patients who are contraindicated or cannot tolerate oral anticoagulants with nonvalvular atrial fibrillation(AF)at risk of st...Percutaneous left atrial appendage(LAA)occlusion evolved as an alternative treatment to the patients who are contraindicated or cannot tolerate oral anticoagulants with nonvalvular atrial fibrillation(AF)at risk of stroke or systemic embolism.[1]Abnormal hemodynamic changes in elder atrial septal defect(ASD)patients cause remodeling of the left atrium,which eventually leads to right heart failure.[2]As the ASDs elderly are associated with a higher incidence of AF,simultaneous transcatheter ASD and LAA closure has become a new effective therapeutic strategy.However,only a limited number of articles involving cardiac tamponade complications have been published in the literature.What’s more,previous studies involving early hemodynamically irrelevant pericardial effusion after the procedure attribute to multiple repositioning attempts of LAA occluder or delivery sheath injured the atrial wall.展开更多
A 54-year-old female patient with congenital heart disease had a persistent complete left bundle branch block three months after closure by an Amplatzer ventricular septal defect occluder. Nine months later, the patie...A 54-year-old female patient with congenital heart disease had a persistent complete left bundle branch block three months after closure by an Amplatzer ventricular septal defect occluder. Nine months later, the patient suffered from chest distress, palpitation, and sweating at daily activities, and her 6-min walk distance decreased significantly (155 m). Her echocardiography showed increased left ventricular end-diastolic diameter with left ventricular ejection fraction of 37%. Her symptoms reduced significantly one week after received cardiac resynchronization therapy. She had no symptoms at daily activities, and her echo showed left ventricular ejection fraction of 46%and 53%. Moreover, left ventricular end-diastolic diameter decreased 6 and 10 months after cardiac resynchronization therapy, and 6-min walk dis-tance remarkably increased. This case demonstrated that persistent complete left bundle branch block for nine months after transcatheter closure with ventricular septal defect Amplatzer occluder could lead to left ventricular enlargement and a significant decrease in left ventricular systolic function. Cardiac resynchronization therapy decreased left ventricular end-diastolic diameter and increased left ventricular ejection fraction, thereby improving the patient’s heart functions.展开更多
Objectives:We aimed to evaluate the effect of percutaneous atrial septal defect(ASD)closure in children using right heart indices and serum galectin-3(Gal-3),as potential biomarkers of right heart remodeling.Methods:T...Objectives:We aimed to evaluate the effect of percutaneous atrial septal defect(ASD)closure in children using right heart indices and serum galectin-3(Gal-3),as potential biomarkers of right heart remodeling.Methods:This case–control prospective study included 40 children with ASD and 25 control subjects.An echocardiographic evaluation was performed before the procedure,as well as 24 h,1 month,and 6 months after intervention.Serum Gal-3 was measured before,and 1 month after the procedure.Results:Serum Gal-3 concentration,right atrial(RA)dimensions,right ventricular(RV)dimensions,indexed RA area,and right index of myocardial performance(RIMP)were significantly increased in children with ASD compared with control subjects while tricuspid annular plane systolic excursion(TAPSE)was significantly decreased.Six months after closure,RA,and RV dimensions significantly decreased and RVfunction improved(RIMP decreased and TAPSE increased).Gal-3 oncentration significantly decreased 1 month after ASD closure,but it did not reach normal range compared with control subjects.A positive correlation between Gal-3 and age at closure,RA area,RV dimensions,and RIMP was observed.A positive correlation was observed between the decrease in Gal-3 concentration and the decrease in RA area and RV dimensions 1 month after ASD closure.A significant negative correlation was observed between TAPSE and Gal-3 concentration before and after intervention.Conclusions:Percutaneous ASD closure can improve right-sided indices and decrease serum Gal-3 concentration.Gal-3 can be used as a sensitive biomarker of right heart remodeling,with a decrease in Gal-3 concentration suggesting reversal of maladaptive remodeling.展开更多
Transcatheter treatments are widespread, having the advantages of being less invasive than surgery with quicker recovery times and reduced physical and psychological consequences. However correct patient selection is ...Transcatheter treatments are widespread, having the advantages of being less invasive than surgery with quicker recovery times and reduced physical and psychological consequences. However correct patient selection is vital to optimise outcomes. In the case of an isolated atrial septal defect (ASD), transcatheter closure is preferred. Whilst multiple or large ASDs or ventricular septal defects (VSDs) are best treated through the transthoracic approach. Furthermore, the development of the transcatheter approach has yielded devices that can be used in the transthoracic approach resulting in hybrid techniques. This article aims to evaluate both transcatheter devices and open-heart surgery in the treatment of cardiac septal defects. A brief discussion follows on from the causes and history of cardiac defect treatments.展开更多
Background:Ventricular crypts are quite a common finding during cardiac imaging,but their etiology is unclear.A possible final result of a spontaneous ventricular septal defect closure has been supposed but never inve...Background:Ventricular crypts are quite a common finding during cardiac imaging,but their etiology is unclear.A possible final result of a spontaneous ventricular septal defect closure has been supposed but never investigated in earlier studies.Method:From January 1997 to December 2020,all newborns diagnosed to have a ventricular septal defect were prospectively entered in our database and those with an isolated defect were included in the study.Ventricular septal defects were classified into four types:perimembranous,trabecular muscular,inlet and outlet.A long-term follow up was performed in order to visualize the possible residual formation of a septal myocardial crypt.Results:A total of 376 isolated ventricular septal defects(314 muscular and 54 perimembranous,4 inlet,4 outlet)were detected.Follow up ranged from 1 to 23 years and showed that,among muscular type,a spontaneous closure occurred in 284(91%),26 did not close(8,28%),2 required surgical intervention(0,63%),3 were lost at follow up(0,95%).During this period,after spontaneous defect closure closure,20 crypts were found(6,4%).Conclusion:This study shows that a muscular ventricular septal defect may evolve in the 6.4%of cases in a residual septal crypt.Although septal crypts occur more frequently in patients affected by hypertrophic and hypertensive cardiomyopathy,they may also represent the evolution of a spontaneous closure of a muscular interventricular defect.展开更多
Objective The purpose of this study is to investgate changes of cTnI in myocardial ischemic and reperfusion injury during correction of cardiac defects in children. Methods From June, 1999 to May,2000,45 children (30 ...Objective The purpose of this study is to investgate changes of cTnI in myocardial ischemic and reperfusion injury during correction of cardiac defects in children. Methods From June, 1999 to May,2000,45 children (30 male, 15 female) undergoing correction of cardiac defects were divided into three groups randomly: group Ⅰ no myocardial ischemia,group Ⅱ myocardial ischemia less than 60 minutes, group Ⅲmyocardial ischemia 】 60 minutes. There were no significant differences in the three groups in age, sex ratio, C/T ratio, or left ventricular function. Blood samples for analysis were collected before skin incision and at time intervals up to 6 days postoperatively. Analysis of creatine kinase MB.LDH and cardiac-specific troponin I was used for the detection of myocardial damage. Meantime, the ECG was checked for myocardial infarction. After the reperfusion, myocardial tissue was obtained from the free wall of right ventricle myocardial structure studies. Results The level of cTnI was increased展开更多
Background: DiGeorge syndrome (also known as velo-cardio-facial syndrome) is a rare multisystem genetic disorder occurring in approximately 1 in 4000 to 1 in 6000 live births [1]. Although advances in genetic screenin...Background: DiGeorge syndrome (also known as velo-cardio-facial syndrome) is a rare multisystem genetic disorder occurring in approximately 1 in 4000 to 1 in 6000 live births [1]. Although advances in genetic screening have improved diagnosis in developed countries, the condition remains underdiagnosed in developing nations such as the Republic of Moldova, where access to genetic testing and family planning services is limited. Routine prenatal screening usually includes regular ultrasounds, monitoring of blood pressure, complete blood counts, coagulation studies, glucose, urine protein, and urine culture. Current ultrasound techniques have limitations in detecting this syndrome due to variability in interpretation, and genetic testing is often performed based on clinical discretion. The ultrasound could potentially point towards a genetic problem, as in DiGeorge, if multiple cardiac malformations are spotted in utero, but most cases such as this one are diagnosed after birth while being described as totally normal on prenatal ultrasound. Purpose: This study aims to highlight the diagnostic challenges and the need for comprehensive evaluation in identifying DiGeorge syndrome, emphasizing the importance of considering the syndrome as a whole rather than focusing on isolated organ system issues. Method: We present a case report of a 6-month-old girl who, after an uneventful pregnancy and normal prenatal ultrasound, presented with cardiac insufficiency. Following extensive investigations and multiple surgical interventions, DiGeorge syndrome was diagnosed at 9 months of age. Results: The patient’s diagnosis was delayed due to the lack of prenatal markers and the reliance on separate investigations of affected organ systems. Despite several interventions aimed at managing her symptoms, the final diagnosis was made after observing the association of multiple clinical features and conducting comprehensive genetic testing. Conclusions: This case underscores the importance of a holistic approach to diagnosis, which involves a thorough patient history, integration of diverse diagnostic tests, and recognition of the syndrome’s multi-system nature. It highlights the necessity for improved diagnostic protocols and increased awareness in regions with limited access to advanced genetic testing to prevent delays in identifying DiGeorge syndrome and to facilitate timely and appropriate management.展开更多
BACKGROUND Transcatheter device closure of atrial septal defect(ASD) guided by fluoroscopy and/or transesophageal echocardiography is a mature technology. Little study has focused on whether the technology can be guid...BACKGROUND Transcatheter device closure of atrial septal defect(ASD) guided by fluoroscopy and/or transesophageal echocardiography is a mature technology. Little study has focused on whether the technology can be guided totally by transthoracic echocardiography(TTE),even in pregnant women with ASD.AIM To evaluate the safety and efficacy of totally TTE guided transcatheter device closure of ASD in pregnant women.METHODS Six pregnant women(gestational age 20-26 wk) with ASD underwent transcatheter device closure totally guided by TTE at our cardiac center from January 2015 to August 2017. A routine transcatheter procedure without fluoroscopy or intubation and a domestic occluder were used in this study.RESULTS All patients had successful closure with good clinical results,and the overall immediate complete closure rate was 100%. The size of the occluder deployed ranged from 20 to 32 mm(26.7 ± 4.3 mm),the procedure time ranged from 30 to50 min(41.7 ± 7.5 min),and the length of hospital stay was 2-3 d(mean 2.2 ± 0.4 d). There were no serious cardiovascular related complications,and transient arrhythmias occurred in one patient during the procedure. During the follow-up period(3 mo to 2 years),no occluder dislodgement,residual fistulas,or thromboses occurred. All of the patients underwent vaginal delivery between 36 and 38 wk of gestation.CONCLUSION Totally TTE guided transcatheter device closure of ASD in pregnant women may be safe and effective.展开更多
Background:Although transcatheter closure of atrial septal defect(ASD)is safe and effective for patients with sufficient rim,ASD patients with absent and/or malaligned aortic and/or superior rim have higher risks of d...Background:Although transcatheter closure of atrial septal defect(ASD)is safe and effective for patients with sufficient rim,ASD patients with absent and/or malaligned aortic and/or superior rim have higher risks of device embolization and cardiac erosion.We have treated such high-risk patients using a Figulla Flex II(FFII)device shaped flared and straddling behind the aorta because this method would avoid such serious complications.However,its long-term efficacy and safety remain unclear.Therefore,the midterm efficacy and safety of this method were studied.Methods:We retrospectively evaluated the outcome of 47 consecutive patients with such rim(age 6–73 years,weight 17–75 kg,31 females)treated with this method at our hospital between February 2016 and September 2019.To make the flared and straddling shape,we selected a FFII 4–6 mm larger than the balloon sizing diameter by stop-flow technique.We also studied the device shape,the disc pressure to the Valsalva wall and their changes over 6 months by transesophageal echocardiography.Results:All procedures were successful,and leakage disappeared within 1 year.During a mean follow up of 37±12 months,complications included a transient sinus node dysfunction and one I°atrioventricular block within 3 months.Whole device shape changed from bulky to thin:the device waist and thickness significantly decreased by around 1.5 mm and 3.5 mm,respectively(p<0.05),but the two discs remained flared and straddling behind the aorta over 6 months;therefore,the disc edges seldom pressed the Valsalva wall perpendicularly,even though the inner plane of either disc often slightly pressed the wall.Conclusions:ASD closure using a FFII shaped flared and straddling behind the aorta is probably effective and safe for patients with absent and/or malaligned aortic and/or superior rim although requiring care for conduction disorders.展开更多
This review aims to sum up the improvements witnessed in the field of interventional cardiology during recent times. The last decade has witnessed significant technical advances in the field of radiological imaging an...This review aims to sum up the improvements witnessed in the field of interventional cardiology during recent times. The last decade has witnessed significant technical advances in the field of radiological imaging and also in interventional cardiology which has helped to offer more non-invasive solutions for the management of congenital heart defects. This has resulted from the use of advanced 3-dimensional fusion imaging instead of conventional 2-dimensional angiography, applying interactive real-time enhancement and using computed tomography and Magnetic Resonance Imaging for interventional procedures. Similarly the introduction of next generation devices, have not only improved the final outcome of the procedure but also has helped in reducing the challenges that were faced before and with the initial generation of devices. These advances have helped not only in reducing the radiation exposure, the use of contrast medium dose but also have resulted in improved early survival. The field of neonatal cardiology has advanced at an unprecedented pace. The transcatheter closure of patent ductus arteriosus has evolved over time and now it has been made possible at much lower body weight. Similarly, early use of stents for aortic coarctation has been found effective in some patients, especially when surgical intervention had been denied. The application of the hybrid approach for the management of complex congenital heart defects has also been effectively applied. More recently transcatheter placement of the pulmonary valve has been introduced for severely stenotic and/or regurgitant pulmonary valve in adolescents and adults. It is anticipated that in near future, this procedure would be available for relatively younger patients. In conclusion: last 2 decades have improvised pediatric interventional cardiology to incorporate less invasive solutions for CHD. The current advances in radio-diagnostic imaging, gadgetry and technical expertise have improved significantly and led to manage many of such defects by trans-catheter approach. This has led also, to replace the early surgical intervention with a more subtle hybrid approach, thus reducing not only the major surgical trauma but also been found to be cost-effective due to a shorter hospital stay. But a learning curve for performers is required for optimum results and also such procedures should be performed in a fully developed facility with an optimum surgical backup.展开更多
基金Supported by Guangzhou Municipal Science and Technology Bureau's 2024 Basic and Applied Basic Research Topic,China,No.2024A04J4491,and No.2024A04J4254the Scientific Research Project of Guangdong Provincial Bureau of Traditional Chinese Medicine,China,No.2022ZYYJ01the Soft Science Research Program of Luohu District,Shenzhen,China,No.LX202402016.
文摘The case report presented in this edition by Mu et al.The report presents a case of atrial septal defect(ASD)associated with electrocardiographic changes,noting that the crochetage sign resolved after Selective His Bundle Pacing(S-HBP)without requiring surgical closure.The mechanisms behind the appearance and resolution of the crochetage sign remain unclear.The authors observed the dis-appearance of the crochetage sign post-S-HBP,suggesting a possible correlation between these specific R waves and the cardiac conduction system.This editorial aims to explore various types of ASD and their relationship with the cardiac con-duction system,highlighting the diagnostic significance of the crochetage sign in ASD.
文摘Background:Closure of an atrial septal defect(ASD)reduces right-side heart volumes by abolishing shunting with simultaneous improvement of the left ventricle(LV)filling and functions due to ventricular interdependence,thereby improving symptoms.Furthermore,studies conducted on atrial volume changes after ASD closure are limited.Cardiac magnetic resonance(CMR)is considered as the gold standard method for measuring cardiac volume and mass.Objective:We aimed to study the effect of transcatheter and surgical closure of secundum ASD on cardiac volumes and systolic functions as well as the fate of tricuspid regurgitation(TR),using CMR analysis.Methods:We prospectively enrolled 30 adult patients with isolated secundum ASD who were referred to ASD closure.CMR evaluation of cardiac chambers indexed volumes,systolic function,myocardial mass index,and tricuspid regurgitant fraction were done at before and 6 months after closure.Results:RV volumes decreased in both groups when compared to baseline(p-value 0.001),the device group had more reduction in volumes and more improvement in RV function after closure(p-value 0.001)when compared to the surgical arm.The changes in the RV mass index were insignificant between both groups(p-value 0.31).Functional TR improved to the same extent in both groups.Left ventricular end diastolic volume index(LVEDVI)and LV mass index increased sig-nificantly in both groups when compared to baseline in both groups but with no difference between groups p-value 0.01),left ventricular end systolic volume index(LVESVI)changes were insignificant.LV systolic function improved in patients who underwent device closure only(63.53±3.85 vs.67.13±4.34,p-value 0.01).There was a significant reduction in right atrial(RA)volumes and an insignificant decrease in left atrial(LA)volumes,with no difference between groups.Conclusion:Transcatheter and surgical secundum ASD closure resulted in volumetric changes in some cardiac chambers with better improvement in bi-ventricular systolic function in the transcatheter arm and no difference in the TR reduction between the two groups at 6 months follow-up by CMR.
文摘Background:There is no gold standard sedation method for pediatric cardiac catheterization.In congenital heart diseases with intracardiac shunts,hemodynamic parameters are prone to change depending on the ventilation conditions and anesthetics,although few studies have examined these effects.The purpose of this study was to investigate the effects of two different sedation methods on the hemodynamic parameters.Methods:This study retrospectively evaluated consecutive patients with ventricular septal defect(VSD)below 1 year of age who underwent cardiac catheterization at Aichi Children’s Health and Medical Center,who were divided into age-and VSD diameter-matched general anesthesia(GA)and monitored anesthesia care(MAC)under the natural airway groups(n=40 each),for comparison of hemodynamic parameters.Results:In the GA group,arterial blood pH and arterial partial pressure of oxygen were significantly higher(p<0.01),whereas arterial partial pressure of carbon dioxide was significantly lower than in the MAC group(p<0.01).Mean pulmonary artery pressure(p<0.05)and systemic blood pressure(p<0.01)were lower in the GA group.Pulmonary vascular resistance index(p<0.01)and systemic vascular resistance index(p<0.01)were also significantly lower in the GA group than the MAC group.There were no significant differences in pulmonary blood flow index,systemic blood flow index,and pulmonary/systemic blood flow ratio between the two groups.Conclusions:Cardiac catheterization under GA in VSD patients results in different hemodynamic parameters compared to that under MAC.In particular,when using pulmonary artery pressure and pulmonary vascular resistance measured under GA for judgment regarding the surgical indications or perioperative management,consideration should be given to the fact that these parameters might be lower compared to those measured under MAC.
文摘Li-Fraumeni syndrome(LFS)is a well-defined autosomal dominant predisposition syndrome due to TP53 germline mutation that causes many cancer malig-nancies.This early-onset syndrome poses a state of widespread malignancy.Such an inherited condition possessing defective p53,guardian of the genome,in the germline has the potential to cause multiple cancers by predominantly affecting mesenchyme(connective tissues,blood cells),breast,brain,and adrenal cortex organs.The tumors initially identified in LFS can eventually propagate to cause secondary malignancies.LFS contributes to multiple cancers in individuals with defective p53 inheritance.When suspected to possess any mass,patients with other co-morbidities,in particular those with certain cardiovascular conditions,undergo screening using high-throughput techniques like transthoracic and transesophageal echocardiography or cardiothoracic magnetic resonance imaging to locate and interpret the size of the mass.In LFS cases,it is certain to presume these masses as cancers and plan their management employing invasive surgeries after performing all efficient diagnostic tools.There are only poor predictions to rule out the chances of any other pathology.This criterion emphasizes the necessity to speculate alternative precision diagnostic methods to affirm such new growth or masses encountered in LFS cases.Moreover,it has all the possibilities to ultimately influence surgical procedures that may be invasive or complicate operative prognosis.Hence,it is essential to strategize an ideal protocol to diagnose any new unexplored mass in the LFS community.In this editorial,we discuss the importance of diagnostic approaches on naïve pristine masses in LFS.
基金supported by the Ph.D. Launch Programs Foundation of Liaoning Province (2019-BS-266)
文摘Percutaneous left atrial appendage(LAA)occlusion evolved as an alternative treatment to the patients who are contraindicated or cannot tolerate oral anticoagulants with nonvalvular atrial fibrillation(AF)at risk of stroke or systemic embolism.[1]Abnormal hemodynamic changes in elder atrial septal defect(ASD)patients cause remodeling of the left atrium,which eventually leads to right heart failure.[2]As the ASDs elderly are associated with a higher incidence of AF,simultaneous transcatheter ASD and LAA closure has become a new effective therapeutic strategy.However,only a limited number of articles involving cardiac tamponade complications have been published in the literature.What’s more,previous studies involving early hemodynamically irrelevant pericardial effusion after the procedure attribute to multiple repositioning attempts of LAA occluder or delivery sheath injured the atrial wall.
文摘A 54-year-old female patient with congenital heart disease had a persistent complete left bundle branch block three months after closure by an Amplatzer ventricular septal defect occluder. Nine months later, the patient suffered from chest distress, palpitation, and sweating at daily activities, and her 6-min walk distance decreased significantly (155 m). Her echocardiography showed increased left ventricular end-diastolic diameter with left ventricular ejection fraction of 37%. Her symptoms reduced significantly one week after received cardiac resynchronization therapy. She had no symptoms at daily activities, and her echo showed left ventricular ejection fraction of 46%and 53%. Moreover, left ventricular end-diastolic diameter decreased 6 and 10 months after cardiac resynchronization therapy, and 6-min walk dis-tance remarkably increased. This case demonstrated that persistent complete left bundle branch block for nine months after transcatheter closure with ventricular septal defect Amplatzer occluder could lead to left ventricular enlargement and a significant decrease in left ventricular systolic function. Cardiac resynchronization therapy decreased left ventricular end-diastolic diameter and increased left ventricular ejection fraction, thereby improving the patient’s heart functions.
文摘Objectives:We aimed to evaluate the effect of percutaneous atrial septal defect(ASD)closure in children using right heart indices and serum galectin-3(Gal-3),as potential biomarkers of right heart remodeling.Methods:This case–control prospective study included 40 children with ASD and 25 control subjects.An echocardiographic evaluation was performed before the procedure,as well as 24 h,1 month,and 6 months after intervention.Serum Gal-3 was measured before,and 1 month after the procedure.Results:Serum Gal-3 concentration,right atrial(RA)dimensions,right ventricular(RV)dimensions,indexed RA area,and right index of myocardial performance(RIMP)were significantly increased in children with ASD compared with control subjects while tricuspid annular plane systolic excursion(TAPSE)was significantly decreased.Six months after closure,RA,and RV dimensions significantly decreased and RVfunction improved(RIMP decreased and TAPSE increased).Gal-3 oncentration significantly decreased 1 month after ASD closure,but it did not reach normal range compared with control subjects.A positive correlation between Gal-3 and age at closure,RA area,RV dimensions,and RIMP was observed.A positive correlation was observed between the decrease in Gal-3 concentration and the decrease in RA area and RV dimensions 1 month after ASD closure.A significant negative correlation was observed between TAPSE and Gal-3 concentration before and after intervention.Conclusions:Percutaneous ASD closure can improve right-sided indices and decrease serum Gal-3 concentration.Gal-3 can be used as a sensitive biomarker of right heart remodeling,with a decrease in Gal-3 concentration suggesting reversal of maladaptive remodeling.
文摘Transcatheter treatments are widespread, having the advantages of being less invasive than surgery with quicker recovery times and reduced physical and psychological consequences. However correct patient selection is vital to optimise outcomes. In the case of an isolated atrial septal defect (ASD), transcatheter closure is preferred. Whilst multiple or large ASDs or ventricular septal defects (VSDs) are best treated through the transthoracic approach. Furthermore, the development of the transcatheter approach has yielded devices that can be used in the transthoracic approach resulting in hybrid techniques. This article aims to evaluate both transcatheter devices and open-heart surgery in the treatment of cardiac septal defects. A brief discussion follows on from the causes and history of cardiac defect treatments.
文摘Background:Ventricular crypts are quite a common finding during cardiac imaging,but their etiology is unclear.A possible final result of a spontaneous ventricular septal defect closure has been supposed but never investigated in earlier studies.Method:From January 1997 to December 2020,all newborns diagnosed to have a ventricular septal defect were prospectively entered in our database and those with an isolated defect were included in the study.Ventricular septal defects were classified into four types:perimembranous,trabecular muscular,inlet and outlet.A long-term follow up was performed in order to visualize the possible residual formation of a septal myocardial crypt.Results:A total of 376 isolated ventricular septal defects(314 muscular and 54 perimembranous,4 inlet,4 outlet)were detected.Follow up ranged from 1 to 23 years and showed that,among muscular type,a spontaneous closure occurred in 284(91%),26 did not close(8,28%),2 required surgical intervention(0,63%),3 were lost at follow up(0,95%).During this period,after spontaneous defect closure closure,20 crypts were found(6,4%).Conclusion:This study shows that a muscular ventricular septal defect may evolve in the 6.4%of cases in a residual septal crypt.Although septal crypts occur more frequently in patients affected by hypertrophic and hypertensive cardiomyopathy,they may also represent the evolution of a spontaneous closure of a muscular interventricular defect.
文摘Objective The purpose of this study is to investgate changes of cTnI in myocardial ischemic and reperfusion injury during correction of cardiac defects in children. Methods From June, 1999 to May,2000,45 children (30 male, 15 female) undergoing correction of cardiac defects were divided into three groups randomly: group Ⅰ no myocardial ischemia,group Ⅱ myocardial ischemia less than 60 minutes, group Ⅲmyocardial ischemia 】 60 minutes. There were no significant differences in the three groups in age, sex ratio, C/T ratio, or left ventricular function. Blood samples for analysis were collected before skin incision and at time intervals up to 6 days postoperatively. Analysis of creatine kinase MB.LDH and cardiac-specific troponin I was used for the detection of myocardial damage. Meantime, the ECG was checked for myocardial infarction. After the reperfusion, myocardial tissue was obtained from the free wall of right ventricle myocardial structure studies. Results The level of cTnI was increased
文摘Background: DiGeorge syndrome (also known as velo-cardio-facial syndrome) is a rare multisystem genetic disorder occurring in approximately 1 in 4000 to 1 in 6000 live births [1]. Although advances in genetic screening have improved diagnosis in developed countries, the condition remains underdiagnosed in developing nations such as the Republic of Moldova, where access to genetic testing and family planning services is limited. Routine prenatal screening usually includes regular ultrasounds, monitoring of blood pressure, complete blood counts, coagulation studies, glucose, urine protein, and urine culture. Current ultrasound techniques have limitations in detecting this syndrome due to variability in interpretation, and genetic testing is often performed based on clinical discretion. The ultrasound could potentially point towards a genetic problem, as in DiGeorge, if multiple cardiac malformations are spotted in utero, but most cases such as this one are diagnosed after birth while being described as totally normal on prenatal ultrasound. Purpose: This study aims to highlight the diagnostic challenges and the need for comprehensive evaluation in identifying DiGeorge syndrome, emphasizing the importance of considering the syndrome as a whole rather than focusing on isolated organ system issues. Method: We present a case report of a 6-month-old girl who, after an uneventful pregnancy and normal prenatal ultrasound, presented with cardiac insufficiency. Following extensive investigations and multiple surgical interventions, DiGeorge syndrome was diagnosed at 9 months of age. Results: The patient’s diagnosis was delayed due to the lack of prenatal markers and the reliance on separate investigations of affected organ systems. Despite several interventions aimed at managing her symptoms, the final diagnosis was made after observing the association of multiple clinical features and conducting comprehensive genetic testing. Conclusions: This case underscores the importance of a holistic approach to diagnosis, which involves a thorough patient history, integration of diverse diagnostic tests, and recognition of the syndrome’s multi-system nature. It highlights the necessity for improved diagnostic protocols and increased awareness in regions with limited access to advanced genetic testing to prevent delays in identifying DiGeorge syndrome and to facilitate timely and appropriate management.
基金Supported by Chinese National and Fujian Provincial Key Clinical Specialty Construction Programs
文摘BACKGROUND Transcatheter device closure of atrial septal defect(ASD) guided by fluoroscopy and/or transesophageal echocardiography is a mature technology. Little study has focused on whether the technology can be guided totally by transthoracic echocardiography(TTE),even in pregnant women with ASD.AIM To evaluate the safety and efficacy of totally TTE guided transcatheter device closure of ASD in pregnant women.METHODS Six pregnant women(gestational age 20-26 wk) with ASD underwent transcatheter device closure totally guided by TTE at our cardiac center from January 2015 to August 2017. A routine transcatheter procedure without fluoroscopy or intubation and a domestic occluder were used in this study.RESULTS All patients had successful closure with good clinical results,and the overall immediate complete closure rate was 100%. The size of the occluder deployed ranged from 20 to 32 mm(26.7 ± 4.3 mm),the procedure time ranged from 30 to50 min(41.7 ± 7.5 min),and the length of hospital stay was 2-3 d(mean 2.2 ± 0.4 d). There were no serious cardiovascular related complications,and transient arrhythmias occurred in one patient during the procedure. During the follow-up period(3 mo to 2 years),no occluder dislodgement,residual fistulas,or thromboses occurred. All of the patients underwent vaginal delivery between 36 and 38 wk of gestation.CONCLUSION Totally TTE guided transcatheter device closure of ASD in pregnant women may be safe and effective.
文摘Background:Although transcatheter closure of atrial septal defect(ASD)is safe and effective for patients with sufficient rim,ASD patients with absent and/or malaligned aortic and/or superior rim have higher risks of device embolization and cardiac erosion.We have treated such high-risk patients using a Figulla Flex II(FFII)device shaped flared and straddling behind the aorta because this method would avoid such serious complications.However,its long-term efficacy and safety remain unclear.Therefore,the midterm efficacy and safety of this method were studied.Methods:We retrospectively evaluated the outcome of 47 consecutive patients with such rim(age 6–73 years,weight 17–75 kg,31 females)treated with this method at our hospital between February 2016 and September 2019.To make the flared and straddling shape,we selected a FFII 4–6 mm larger than the balloon sizing diameter by stop-flow technique.We also studied the device shape,the disc pressure to the Valsalva wall and their changes over 6 months by transesophageal echocardiography.Results:All procedures were successful,and leakage disappeared within 1 year.During a mean follow up of 37±12 months,complications included a transient sinus node dysfunction and one I°atrioventricular block within 3 months.Whole device shape changed from bulky to thin:the device waist and thickness significantly decreased by around 1.5 mm and 3.5 mm,respectively(p<0.05),but the two discs remained flared and straddling behind the aorta over 6 months;therefore,the disc edges seldom pressed the Valsalva wall perpendicularly,even though the inner plane of either disc often slightly pressed the wall.Conclusions:ASD closure using a FFII shaped flared and straddling behind the aorta is probably effective and safe for patients with absent and/or malaligned aortic and/or superior rim although requiring care for conduction disorders.
文摘This review aims to sum up the improvements witnessed in the field of interventional cardiology during recent times. The last decade has witnessed significant technical advances in the field of radiological imaging and also in interventional cardiology which has helped to offer more non-invasive solutions for the management of congenital heart defects. This has resulted from the use of advanced 3-dimensional fusion imaging instead of conventional 2-dimensional angiography, applying interactive real-time enhancement and using computed tomography and Magnetic Resonance Imaging for interventional procedures. Similarly the introduction of next generation devices, have not only improved the final outcome of the procedure but also has helped in reducing the challenges that were faced before and with the initial generation of devices. These advances have helped not only in reducing the radiation exposure, the use of contrast medium dose but also have resulted in improved early survival. The field of neonatal cardiology has advanced at an unprecedented pace. The transcatheter closure of patent ductus arteriosus has evolved over time and now it has been made possible at much lower body weight. Similarly, early use of stents for aortic coarctation has been found effective in some patients, especially when surgical intervention had been denied. The application of the hybrid approach for the management of complex congenital heart defects has also been effectively applied. More recently transcatheter placement of the pulmonary valve has been introduced for severely stenotic and/or regurgitant pulmonary valve in adolescents and adults. It is anticipated that in near future, this procedure would be available for relatively younger patients. In conclusion: last 2 decades have improvised pediatric interventional cardiology to incorporate less invasive solutions for CHD. The current advances in radio-diagnostic imaging, gadgetry and technical expertise have improved significantly and led to manage many of such defects by trans-catheter approach. This has led also, to replace the early surgical intervention with a more subtle hybrid approach, thus reducing not only the major surgical trauma but also been found to be cost-effective due to a shorter hospital stay. But a learning curve for performers is required for optimum results and also such procedures should be performed in a fully developed facility with an optimum surgical backup.