Introduction: Ventricular septal defect (VSD) is the most common congenital heart disease of all congenital heart defects. The aim of this study was to investigate the echographic, therapeutic and evolutionary aspects...Introduction: Ventricular septal defect (VSD) is the most common congenital heart disease of all congenital heart defects. The aim of this study was to investigate the echographic, therapeutic and evolutionary aspects of ventricular septal defects (VSD) in the general cardiology department of the Hôpital National Ignace Deen. Methods: A retrospective data collection was carried out from January 2018 to December 2023 including 85 cases of isolated IVC was performed. The variables studied were epidemiological, clinical, paraclinical, therapeutic and evolutionary. Results: Of the 320 patients seen during the study period for congenital heart disease, 85 (26.556%) were isolated IVCs. Age at diagnosis ranged from 3 months to 16 years, with an average age of 3.59 years. The most represented ethnic group was the Fulani (50.58%). The 8.24% came from consanguineous marriage versus 22.35%. 91.76% of children had a history of bronchitis. The most common clinical signs found were systolic murmur (90.58%), growth retardation (51.76%). Only 4 cases (4.70%) had a malformation associated with IVC represented by DiGeorges disease (2.35%) and trisomy 21 (2.35%). Nearly half the patients had type IIb VIC (44.71%). The other half were represented by type 1 (18.82%), type IIa (20%), type III (10.59%) and type IV (5.88%). According to site more than two-thirds of VICs (71.64%) were perimembranous in location, followed by infundibular (16.47%) and muscular (11.76%) VICs. In our study 55.29% presented an indication for both surgical intervention and medical treatment, while 16.47% required only medical treatment. In contrast, 28.23% were placed under exclusive surveillance. Of the 47 patients for whom surgery was indicated, 29 (61.17%) underwent surgical repair, while 18 (38.83%) were awaiting confirmation for surgery. Conclusion: VIC is the most common congenital heart disease. An early detection strategy and the establishment of specialized centers could improve the outcome of these children.展开更多
Background:The optimal surgical timing and clinical outcomes of ventricular septal defect(VSD)closure in neo-nates remain unclear.We aimed to evaluate the clinical outcomes of VSD closure in neonates(age≤30 days).Met...Background:The optimal surgical timing and clinical outcomes of ventricular septal defect(VSD)closure in neo-nates remain unclear.We aimed to evaluate the clinical outcomes of VSD closure in neonates(age≤30 days).Methods:We retrospectively reviewed 50 consecutive neonates who underwent VSD closure for isolated VSDs between August 2003 and June 2021.Indications for the procedure included congestive heart failure/failure to thrive and pulmonary hypertension.Major adverse events(MAEs)were defined as the composite of all-cause mortality,reoperation,persistent atrioventricular block,and significant(≥grade 2)valvular dysfunction.Results:The median age and body weight at operation were 26.0 days(interquartile range[IQR],18.8–28.3)and 3.7 kg(IQR,3.3–4.2),respectively.The median follow-up duration was 110.4 months(IQR,56.8–165.0).Seven patients required preoperative respiratory support,andfive had significant(≥grade 2)preoperative valvular dysfunction.One early mortality occurred due to irreversible cardiogenic shock;no late mortality was observed.One reopera-tion was due to hemodynamically significant residual VSD at 103.8 months postoperatively.The overall survival,freedom from reoperation,and freedom from MAE at 15-years were 98.0%,96.3%,and 94.4%,respectively.Pre-operative mechanical ventilation was associated with a longer duration of postoperative mechanical ventilation(p<0.001)and a longer length of intensive care unit stay(p<0.001).Conclusions:VSD closure with favorable outcomes without morbidities is feasible even in neonates.However,neonates requiring preoperative respiratory support may require careful postoperative management considering the long-term postoperative risks.Overall,surgical VSD closure might be indicated earlier in neonates with respiratory compromise.展开更多
Background:Atrial septal defect(ASD)is one of the common congenital heart diseases.The MYH6 gene has a critical role in cardiac development but the role of MYH6 promoter variants in patients with ASD has not been expl...Background:Atrial septal defect(ASD)is one of the common congenital heart diseases.The MYH6 gene has a critical role in cardiac development but the role of MYH6 promoter variants in patients with ASD has not been explored.Methods:In 613 subjects including 320 ASD patients,we investigated the MYH6 gene promoter variants and verified the effect on gene expression by using cellular functional experiments and bioinformatics analysis.Results:Eleven variants were identified in the MYH6 gene promoter,of which four variants were found only in ASD patients,and two variants(g.3434G>C and g.4524C>T)were identified for the first time.Cellular functional experiments indicated that all four variants reduced the transcriptional activity of the MYH6 gene promoter(p<0.05).Subsequent analysis through the JASPAR(A database of transcription factor binding profiles)suggests that these variants may alter transcription factor binding sites,which may in turn lead to changes in myocardin subunit expression and ASD formation.Conclusions:Our study for the first time focuses on variants in the promoter region of the MYH6 gene in Chinese patients with ASD and the discovered variants have functional significance.The study provides new insights in the role of the MYH6 gene promoter region to better understand the genetic basis of ASD formation and facilitates clinical diagnosis.展开更多
Introduction:Transcatheter closure is an alternative to ventricular septal defect(VSD)occlusion surgery.Nit-Occlud LêVSD coil is a new device yet to be evaluated.The study aimed to evaluate immediate and midterm ...Introduction:Transcatheter closure is an alternative to ventricular septal defect(VSD)occlusion surgery.Nit-Occlud LêVSD coil is a new device yet to be evaluated.The study aimed to evaluate immediate and midterm results after transcatheter closure with the Nit-Occlud LêVSD device.Methods:The retrospective analysis included 30 patients with VSD referred for closure during the period from October 2015 to December 2020.Results:At the time of intervention,the patients’mean age and body weights were 7.5±5.6 years and 29.3±19.1 kg.The majority of the defects had perimembranous location(24/30),four defects had muscular and two outlet subaortic position.The mean effective right-side diameter of the VSDs was 3.6±1.3 mm.Single ventricular fibrillation,device embolization,and hemolysis developed in different patients and were successfully treated.None of the patients had a complete atrioventricular block.The coil was successfully placed in 25/30(83.3%)patients.The majority of the devices were 10 mm×6 mm(11/25)and 12 mm×8 mm(8/25)in size.Two patients required the implantation of a second device.The follow-up period was 2.1±1.4 years.Complete VSD closure was achieved in 48%of cases immediately after the intervention,74%during 2.1±1.6 months after the procedure,and 81%over follow-up.The remaining patients had a trivial residual defect.During the follow-up,approximately one-third of patients developed trivial aortic and mitral valve regurgitation,and half of the patients acquired trace/mild tricuspid regurgitation.Standardized(z-score)left ventricular end-diastolic diameter(0.15±0.37 vs.0.92±0.82,p=0.005)and left atrium dimension(0.47±0.58 vs.1.89±1.11,p=0.005),as well as the left atrium to aortic root ratio(1.2±0.1 vs.1.4±0.2,p=0.005)showed a significant decrease over follow-up related to the period before intervention.Conclusion:Intervention with Nit-OccludLêVSD coil showed appropriate results regarding VSD closure rate,complications,and chamber remodeling.The introduction of this device into clinical practice is a significant step forward in transcatheter perimembranous VSD occlusion.展开更多
Objective: The aim is to study the trends in ventricular septal defect (VSD) mortality in children in Kazakhstan.Methods: The retrospective study was done for the period 2011–2020. Descriptive and analytical methods ...Objective: The aim is to study the trends in ventricular septal defect (VSD) mortality in children in Kazakhstan.Methods: The retrospective study was done for the period 2011–2020. Descriptive and analytical methods of epidemiologywere applied. The universally acknowledged methodology used in sanitary statistics is used to calculatethe extensive, crude, and age-specific mortality rates. Results: Kazakhstan is thought to be seeing an increase inmortality from VSDs in children. As a result, this study for the years 2011 to 2020 was conducted to retrospectivelyassess data from the central registration of the Bureau of National Statistics that was available throughoutthe nation. Age-standardized mortality data were obtained and compared between age categories. It was shownthat 507 children died from this condition throughout the time period under study. The average annual standardizedmortality rate was 1.88 per 100,000 population and tended to decrease over time. The peak of mortality wasnoted at the age of up to 1 year, namely the neonatal period. Cartogram mortality rates were calculated usingstandardized indicators. Additionally, age-sex variations were taken into account when performing all calculations.Conclusion: In recent years, the death rate from VSD has declined from 1.5 to 0.6 per 100,000 people, withthe trend remaining constant (T = 1.4%, R^(2) = 0.5825). The analysis of mortality trends related to VSD is crucial inboth theoretical and practical aspects, as it enables early detection and treatment of VSDs. The findings of thisstudy will be valuable to public health authorities in developing a strategy to treat VSDs effectively.展开更多
Background:Ventricular septal defect(VSD)is the most common congenital heart disease.Transcatheter VSD closure is an effective treatment for patients with muscular and perimembranous VSD.However,there is a limit data ...Background:Ventricular septal defect(VSD)is the most common congenital heart disease.Transcatheter VSD closure is an effective treatment for patients with muscular and perimembranous VSD.However,there is a limit data for outlet VSD,especially impact to the aortic valve leaflet after transcatheter closure.This study aims to assess the outcomes of transcatheter closure of the outlet-type ventricular septal defect(OVSD)after 1 postoperative year.Methods:A retrospective study was performed including 50 patients who underwent transcatheter(n=25)and surgical(n=25)OVSD closure during the exact time frame at two medical centres.Results:The median age and body weight of patients in the transcatheter group were significantly higher than those of patients in the surgical group(7.0 vs.2.8 years;27.0 vs.11.4 kg;p<0.01).The defect size in the surgical group was significantly larger than that in the transcatheter group(5.0 vs.3.0 mm;p<0.01).All OVSD patients have successful transcatheter closure(100%)as effective as surgical closure.Less than small residual shunt was present 20%and 8%immediately after the procedure in the transcatheter and surgical groups(p=0.50),which decreased to 12%and 4%at the 1-year follow-up(p=0.61),respectively.No incidence of complete atrioventricular block and other complications was observed in both groups,and no significant differences were noted in the new onset or worsening of the aortic regurgitation in both groups(p=1.0).Conclusions:Transcatheter treatment could be effectively and safely achieved for OVSD closure at 1-year follow-up.展开更多
<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;&...<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;" "=""> dysplasia (Ellis-Van Creveld syndrome) such as skeletal and joint abnormalities often have concomitant congenital cardiac anomalies. Presence of cardiorespiratory symptoms in children with Ellis-Van Craved syndrome warrants a thorough cardiologic evaluation to recognize and treat underlying congenital heart anomaly. <b>Aim:</b> A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and <span>accomplish successful repair of the underlying cardiac lesion to reduce the cardiac</span> related morbidity and improve the patient survival. <b>Case Presentation:</b> Ten year</span><span style="font-family:;" "="">s</span><span style="font-family:;" "=""> old boy with chondroectodermal dysplasia (dental anomalies, genu valgum and other skeletal abnormalities) presented with dyspnea and cyanosis. Cardiac evaluation by 2D echo revealed an atrioventricular (AV) canal septal defect with AV valve regurgitation and a common atrium. Angiocardiography showed a goose neck deformity of the left ventricular outflow tract. <span>The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO<sub>2</sub> of 0.7) </span>and O<sub>2</sub> saturation in the common atrium was 0.7. The pulmonary venous connections to the common atrium were anomalous. Atriotomy on cardiopulmonary bypass and on a cardioplegic arrest discerned a partial AV canal septal defect with a common bridging leaflet, clefts in septal leaflets of tricuspid and mitral vlalves, an incompletely closed interventricular communication, and a common atrium with highly anomalous pulmonary venous insertions well anterior (8</span><span style="font-family:;" "=""> </span><span style="font-family:;" "="">cm) to vena caval orifices. Intracardiac repair was performed with two patches of Goertex to partition the common atrium into the pulmonary and systemic venous chambers after repair of the partial AV canal septal defect. Patient required only a temporary afterload reduction with enalapril;otherwise patient had an uneventful postoperative course. At a 2</span><span style="font-family:;" "="">-</span><span style="font-family:;" "="">year follow-up, the child was well without AV valve regurgitation and had normal <span>biventricular function. <b>Conclusion:</b> A child with Ellis-Van-Creveld syndrome</span> with skeletal abnormalities and dental anomalies had manifested with cardio-respiratory symptoms. Preoperative cardiac and intraoperative evaluation showed a common atrium with severely anomalous pulmonary venous connection and partial AV canal septal defect. Successful biventricular repair was accomplished by repairing the partial AV canal septal defect and partitioning <span>the common atrium into left and right atrium by a complex atrial routing tech<span>nique with two patches of Gore-Tex. On a follow-up at 2 years</span></span></span><span style="font-family:;" "="">, </span><span style="font-family:;" "="">the patient had</span><span style="font-family:;" "=""> adequate biventricular function without AV valve regurgitation.</span> <div class="__kindeditor_paste__" style="position:absolute;width:1px;height:1px;overflow:hidden;left:-1981px;top:0px;white-space:nowrap;"> <table width="100%" border="0" cellpadding="0" cellspacing="1" bgcolor="#cacfd2" style="border:0px solid #CCCCCC;line-height:25px;width:1041px;color:#000000;font-family:宋体, Arial, sans-serif;"> <tbody> <tr style="background-color:#FAFBFD;"> <td style="text-align:center;font-size:14px;vertical-align:middle;"> <div align="center"> 114264<strong></strong> </div> </td> </tr> </tbody> </table> </div>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
Objective:This study was designed to determine the long-term safety and efficacy of using the Amplatzer Duct Occluder II(ADO II)for the closure of various ventricular septal defects(VSDs).Methods:From January 2011 to ...Objective:This study was designed to determine the long-term safety and efficacy of using the Amplatzer Duct Occluder II(ADO II)for the closure of various ventricular septal defects(VSDs).Methods:From January 2011 to December 2019,selected VSD patients were treated through transcatheter intervention using ADO II occluders.The closure results and complications from 188 patients,involving 167 perimembranous ventricular septal defects(pmVSDs),9 intracristal VSDs,11 post surgery residual shunts and 1 post closure residual shunt with the mean outlet diameter3.1±0.8 mm under angiography,were enrolled in this study.Results:The success rate was 98.9%for all procedures.During the median 77-month follow-up period,no cases of complete atrioventricular block(cAVB),infective endocarditis or death occurred.One major adverse event(0.5%)was recorded:cerebrovascular accident occurred 1 day after the procedure in one patient who was transferred to the neurology department.The residual shunt rate was 44.6%,which was the most common minor adverse event.The cardiac conduction block rate was 4.3%.Specifically,one pmVSD patient developed intermittent LBBB during the 28-month follow-up.There were 3 patients(1.6%)with new-onset mild tricuspid insufficiency,and the insufficiency degree was stable during follow-up.There was no new-onset aortic insufficiency that occurred.Conclusions:Transcatheter closure of pmVSDs,some intracristal VSDs,some postsurgery or postclosure residual shunts using ADO II occluders were both safe and effective and yielded excellent long-term results in selected patients.展开更多
Background: Interventricular septal hematoma is a rare complication after congenital cardiac repair. The managementvaries according to the literature. We present our experience with this rare complication. Methods:Ech...Background: Interventricular septal hematoma is a rare complication after congenital cardiac repair. The managementvaries according to the literature. We present our experience with this rare complication. Methods:Echocardiography database were reviewed with the term ‘‘hematoma’’ or “hypoechoic mass” for patients whounderwent congenital heart surgery from January 2018 to December 2021 at our institution to identify potentialinterventricular septal hematoma cases. Relevant data of the patients identified were collected. Focus was put onthe presentation, management, outcomes according to patent medical charts and serial echocardiographic reportdata. Results: In total, there were 5 patients included. The mean age and weight at surgery were 5.5 ± 3.6 monthsand 5.5 ± 1.4 kg, respectively. Four patients were diagnosed with ventricular septal defect and the other one beingdouble outlet of the right ventricle. While all patients had intraoperative transesophageal echocardiography, 80%(4 of 5) of Interventricular septal hematoma were revealed intraoperatively. Only one patient received hematomadrainage intraoperatively while the other 3 identified in the operating room were only closely observed. One afterventricular septal defect repair presented continuous dysfunction of the left ventricle at the last follow-up, whilethe others were doing well. All hematomas resolved completely with a mean time to interventricular septal hematomaresolution of 35.8 ± 16.9 days. Conclusion: Infants seem to be at a higher risk for Interventricular septalhematoma following congenital heart surgery. While the majority of interventricular septal hematoma has abenign postoperative course, some may result in ventricular dysfunction. Management strategies may be chosenon a case-by-case basis.展开更多
In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old pa...In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old patient with Down syndrome(DS)and congenital heart disease(CHD)associated with pulmonary arterial hypertension.In this distinct population,the Authors underscore the need for early diagnosis and management as well as the need of a multidisciplinary approach for decision making.It seems that the occurrence of CHD in patients with DS adds layers of complexity to their clinical management.This editorial aims to provide a comprehensive overview of the intricate interplay between DS and congenital heart disorders,offering insights into the nuanced diagnostic and therapeutic considerations for physicians.展开更多
Congenital ventricular septal defect (VSD) spontaneous close induced by transcatheter treatment is rare and has not yet been reported. We report on one case of VSD spontaneous close induced by transcatheter treatmen...Congenital ventricular septal defect (VSD) spontaneous close induced by transcatheter treatment is rare and has not yet been reported. We report on one case of VSD spontaneous close induced by transcatheter treatment in a 10 years old girl.展开更多
Objectives To evaluate the therapeutic effect of transcatheter Amplatzer device on the closure of ventricular septal defect (VSD). Methods Among 143 patients with VSD, 135 patients with perimembrane VSD and 2 wit...Objectives To evaluate the therapeutic effect of transcatheter Amplatzer device on the closure of ventricular septal defect (VSD). Methods Among 143 patients with VSD, 135 patients with perimembrane VSD and 2 with muscular VSD aged 2.5 -28 years old, were successfully closed with Amplatzer oeeluder device by the pereutaneous guidwire through femoral artery-VSD-femoral vein route under the guidance of fluoroscopy, ventrieulography and transthoracic echocardiography (TIE). The diameters of the VSDs were 2.3-15.7 (6.90±2.76)mm by left ventriculography. Results The success rate of transcatheter closure of VSD with Amplatzer devices was 96%(137/143). Minimal residual amount of shunts were found in one patient, although the shunts was decreased one month after the procedure. There were one patient who had respiratory arrest during the procedure, 7 patients(5%)had conduction disturbance, 3 patients had complete left bundle branch block, 2 patients had complete right bundle branch block, 1 patient had Ⅰ degree atrial-ventricular block and 1 patient had Ⅲ degree atr/al-ventricular block during hospitalization. The diameters of the occluder ranged from 4 to 23 (9.13±3.31)mm and were symmetrical in 122 patients and asymmetrical in 15 patients. Conclusions Transcatheter closure of the perimembranous ventrieular septal defect using Amplatzer VSD occluder device is an efficient method for patients with the perimembranous VSD. The operation is simple with a high success rate and a good effect.展开更多
Objective To evaluate the efficacy of minimally invasive perventricular device closure of ventricular septal defect(VSD). Methods Between September 2011 and February 2013, we collected 40 patients who underwent perven...Objective To evaluate the efficacy of minimally invasive perventricular device closure of ventricular septal defect(VSD). Methods Between September 2011 and February 2013, we collected 40 patients who underwent perventricular closure via a small lower sternal incision(minimally invasive group), aged 15.5±3.5 years(12 months to 32 years) with a body weight of 24.2±7.5 kg(10.8-58.0 kg). The mean size of VSD was 5.6±0.5 mm(2-14 mm). Another 40 patients were included as the surgical group, receiving the conventional surgical repair of VSD. The device of the minimally invasive group was released under the guidance of transesophageal echocardiography. Success rate, cardiac indicators, and clinical outcomes of the 2 groups were compared. Results The patients in the surgical group and those in the minimally invasive group showed similar results in success rate(both 97.5%). The procedure time, intensive care unit stay, hospital stay, and postoperative recovery time in the minimally invasive group were significantly shorter than those in the surgical group(58±21 minutes versus 145±26 minutes, 2±1 days versus 8±3 days, 5±1 days versus 16±6 days, 3±1 days versus 90±20 days, all P<0.05). The minimally invasive group had a higher incidence of conduction anomalies(17.5% versus 2.5%, P<0.05). In the follow-up period of 3-12 months, there was no new residual shunt, noticeable aortic regurgitation, significant arrhythmias, or device failure except for new complications in the surgical group. Conclusions The success rate of minimally invasive perventricular device closure of VSD under transesophageal echocardiography guidance is similar to that of conventional surgical repair, but the short-term outcomes of the minimally invasive approach is much better. Long-term follow-up is necessary to confirm the effectiveness of this technique.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
The aim of this study was to examine how somatic mutations of the GATA4 gene contributed to the genesis of ventricular septal defect (VSD). The coding and intron-exon boundary regions of GATA4 were sequenced of DNA ...The aim of this study was to examine how somatic mutations of the GATA4 gene contributed to the genesis of ventricular septal defect (VSD). The coding and intron-exon boundary regions of GATA4 were sequenced of DNA samples from peripheral blood cells and cardiac tissues of twenty surgically treated probands with VSD. Seven novel heterozygous variants were detected in cardiac tissues from VSD patients, but they were not detected in the peripheral blood cells of VSD patients or in 500 healthy control samples. We replicated 14 single nucleotide polymorphisms (SNPs) reported in NCBI. Bioinformatics analysis was performed to analyze the possible mechanism by which mutations were linked to VSD. Among those variants, c. 1004C〉A (p.S335X) occurred in the highly conserved domain of GATA4 and generated a termination codon, which led to the production of truncated GATA4. The seven novel heterozygous GATA4 mutations were only identified in cardiac tissues with VSD, suggesting that they are of somatic origin. A higher mutation rate in cardiac tissues than in peripheral blood cells implies that the genetic contribution to VSD may have been underestimated.展开更多
Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary ar...Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary artery. Through right atrial approach, the large ventricular septal defects were closed by pericardial patch. The smaller ventricular septal defects were closed directly. Results: Out of 33 patients, 20 patients were multiple ventricular septal defects, 5 patients were atrioventricular canal defect with multiple ventricular septal defects and 8 patients were transposition of great arteries with multiple ventricular septal defects. All the patients had severe pulmonary arterial hypertension. The mean age and body weight at repair was 4.51 months and 5.41 kg respectively. Failure to locate additional ventricular septal defects happened in 1 patient where the pulmonary artery banding was done. Six patients had residual ventricular septal defect after surgery, and no significant left to right shunted. One patient had permanent pacemaker implanted in the postoperative period due to the heart block. There was no early and late mortality. Conclusion: Management of multiple ventricular septal defects is quite complex. Right atrial approach of ventricular septal defects closure is safe, simple and effective in closure of multiple ventricular septal defects.展开更多
Background: There is a need for data on epidemiological, clinical and therapeutic aspects of ventricular septal defect among children in?Sub-Saharan Africa. Objective: The aim of this study was to determine the preval...Background: There is a need for data on epidemiological, clinical and therapeutic aspects of ventricular septal defect among children in?Sub-Saharan Africa. Objective: The aim of this study was to determine the prevalence, epidemioclinical, echocardiographic, therapeutic and evolutionary aspects of ventricular septal defects (VSD) in the pediatric department of the University Hospital Center (CHUSS) of Bobo-Dioulasso. Methods: This study was a descriptive cross-sectional study, conducted from November 2013 to December 2016. All children aged 1 to 179 months seen at the pediatric consultation in CHUSS were included. CIV was confirmed with Doppler echocardiography. Results: Out of 36,240 children who received consultation in the pediatric ward of CHUSS during the study period, one hundred (100) cases of them had congenital heart disease representing a hospital prevalence of 2.76%. This was diagnosed with Doppler echocardiography. Of these, 88% were VSD isolated or associated with other cardiac malformations. Isolated form was reported in 54.3% of cases. The average?age at diagnosis was 39.6 months. The sex ratio was 1.05. Perimembranous topography and hemodynamic type 2 were the highest, representing 56.8% and 35.2% respectively. The indication for surgical repair was recommended for 81.8% of the cases, but only 9.7% of these cases benefited from cardiac surgery. The rest were for medicalcare with a high proportion of lost to follow-up (48.9%). Conclusion: VSD is the most common congenital heart disease. Its care is mainly surgical. This cardiac surgery is non-existent in Burkina Faso. The design of multidisciplinary strategies associated with an optimization of the means of the countries of Sub-Saharan Africa could improve the management of this cardiopathy.展开更多
An 86-year-old male patient was admitted in our cardiology ward with signs of congestive biventricular heart failure. The patient presented with deteriorating dyspnea on mild exertion and at rest the last days, compat...An 86-year-old male patient was admitted in our cardiology ward with signs of congestive biventricular heart failure. The patient presented with deteriorating dyspnea on mild exertion and at rest the last days, compatible with class NYHA Ⅲ-Ⅳ heart failure, bilateral peripheral oedema, increased NT-proBNP (9198 pg/mL), mildly elevated Troponin (TnT 64 pg/mL), interstitial pulmonary oedema and bilateral pleural effusions in chest X ray.展开更多
Background:Communication between the right pulmonary artery(RPA)and left atrium(LA)is a rare cause of central cyanosis in pediatric patients.Case presentation:We describe a 3-year-old female patient with an oxygen sat...Background:Communication between the right pulmonary artery(RPA)and left atrium(LA)is a rare cause of central cyanosis in pediatric patients.Case presentation:We describe a 3-year-old female patient with an oxygen saturation of 70%at admission.The echocardiogram indicated an abnormal color flow Doppler in the LA and she underwent standard cardiac catheterization.The angiography of pulmonary artery revealed a 7.4 mm×7.6 mm fistula between the RPA and LA and achieved successful closure using ventricular septal defect occlusion.Conclusion:The fistula between pulmonary artery and left atrium is an extremely rare but treatable congenital defect.It should be considered in differential diagnosis of cyanosis in children.展开更多
文摘Introduction: Ventricular septal defect (VSD) is the most common congenital heart disease of all congenital heart defects. The aim of this study was to investigate the echographic, therapeutic and evolutionary aspects of ventricular septal defects (VSD) in the general cardiology department of the Hôpital National Ignace Deen. Methods: A retrospective data collection was carried out from January 2018 to December 2023 including 85 cases of isolated IVC was performed. The variables studied were epidemiological, clinical, paraclinical, therapeutic and evolutionary. Results: Of the 320 patients seen during the study period for congenital heart disease, 85 (26.556%) were isolated IVCs. Age at diagnosis ranged from 3 months to 16 years, with an average age of 3.59 years. The most represented ethnic group was the Fulani (50.58%). The 8.24% came from consanguineous marriage versus 22.35%. 91.76% of children had a history of bronchitis. The most common clinical signs found were systolic murmur (90.58%), growth retardation (51.76%). Only 4 cases (4.70%) had a malformation associated with IVC represented by DiGeorges disease (2.35%) and trisomy 21 (2.35%). Nearly half the patients had type IIb VIC (44.71%). The other half were represented by type 1 (18.82%), type IIa (20%), type III (10.59%) and type IV (5.88%). According to site more than two-thirds of VICs (71.64%) were perimembranous in location, followed by infundibular (16.47%) and muscular (11.76%) VICs. In our study 55.29% presented an indication for both surgical intervention and medical treatment, while 16.47% required only medical treatment. In contrast, 28.23% were placed under exclusive surveillance. Of the 47 patients for whom surgery was indicated, 29 (61.17%) underwent surgical repair, while 18 (38.83%) were awaiting confirmation for surgery. Conclusion: VIC is the most common congenital heart disease. An early detection strategy and the establishment of specialized centers could improve the outcome of these children.
基金This retrospective study was approved by the Seoul National University Hospital Institutional Review Board(approval number:H-2106-179-1230).The requirement for informed consent was waived.
文摘Background:The optimal surgical timing and clinical outcomes of ventricular septal defect(VSD)closure in neo-nates remain unclear.We aimed to evaluate the clinical outcomes of VSD closure in neonates(age≤30 days).Methods:We retrospectively reviewed 50 consecutive neonates who underwent VSD closure for isolated VSDs between August 2003 and June 2021.Indications for the procedure included congestive heart failure/failure to thrive and pulmonary hypertension.Major adverse events(MAEs)were defined as the composite of all-cause mortality,reoperation,persistent atrioventricular block,and significant(≥grade 2)valvular dysfunction.Results:The median age and body weight at operation were 26.0 days(interquartile range[IQR],18.8–28.3)and 3.7 kg(IQR,3.3–4.2),respectively.The median follow-up duration was 110.4 months(IQR,56.8–165.0).Seven patients required preoperative respiratory support,andfive had significant(≥grade 2)preoperative valvular dysfunction.One early mortality occurred due to irreversible cardiogenic shock;no late mortality was observed.One reopera-tion was due to hemodynamically significant residual VSD at 103.8 months postoperatively.The overall survival,freedom from reoperation,and freedom from MAE at 15-years were 98.0%,96.3%,and 94.4%,respectively.Pre-operative mechanical ventilation was associated with a longer duration of postoperative mechanical ventilation(p<0.001)and a longer length of intensive care unit stay(p<0.001).Conclusions:VSD closure with favorable outcomes without morbidities is feasible even in neonates.However,neonates requiring preoperative respiratory support may require careful postoperative management considering the long-term postoperative risks.Overall,surgical VSD closure might be indicated earlier in neonates with respiratory compromise.
基金This study involving human participants was reviewed and approved by the ethics committee of TEDA International Cardiovascular Hospital,China(No.0715-4,2021,02 August 2021)the National Natural Science Foundation of China[82170353&81870288]+4 种基金the Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences[2020-PT310-007]Tianjin Municipal and Binhai New Area Health Commissions[KJ20071&2019BWKY010]Tianjin Science and Technology Project[18PTZWHZ00060]TEDA International Cardiovascular Hospital[2021-TD-006&2021-ZX-002&2019-TD-013]Tianjin Key Medical Discipline(Specialty)Construction Project[TJYXZDXK-019A].
文摘Background:Atrial septal defect(ASD)is one of the common congenital heart diseases.The MYH6 gene has a critical role in cardiac development but the role of MYH6 promoter variants in patients with ASD has not been explored.Methods:In 613 subjects including 320 ASD patients,we investigated the MYH6 gene promoter variants and verified the effect on gene expression by using cellular functional experiments and bioinformatics analysis.Results:Eleven variants were identified in the MYH6 gene promoter,of which four variants were found only in ASD patients,and two variants(g.3434G>C and g.4524C>T)were identified for the first time.Cellular functional experiments indicated that all four variants reduced the transcriptional activity of the MYH6 gene promoter(p<0.05).Subsequent analysis through the JASPAR(A database of transcription factor binding profiles)suggests that these variants may alter transcription factor binding sites,which may in turn lead to changes in myocardin subunit expression and ASD formation.Conclusions:Our study for the first time focuses on variants in the promoter region of the MYH6 gene in Chinese patients with ASD and the discovered variants have functional significance.The study provides new insights in the role of the MYH6 gene promoter region to better understand the genetic basis of ASD formation and facilitates clinical diagnosis.
文摘Introduction:Transcatheter closure is an alternative to ventricular septal defect(VSD)occlusion surgery.Nit-Occlud LêVSD coil is a new device yet to be evaluated.The study aimed to evaluate immediate and midterm results after transcatheter closure with the Nit-Occlud LêVSD device.Methods:The retrospective analysis included 30 patients with VSD referred for closure during the period from October 2015 to December 2020.Results:At the time of intervention,the patients’mean age and body weights were 7.5±5.6 years and 29.3±19.1 kg.The majority of the defects had perimembranous location(24/30),four defects had muscular and two outlet subaortic position.The mean effective right-side diameter of the VSDs was 3.6±1.3 mm.Single ventricular fibrillation,device embolization,and hemolysis developed in different patients and were successfully treated.None of the patients had a complete atrioventricular block.The coil was successfully placed in 25/30(83.3%)patients.The majority of the devices were 10 mm×6 mm(11/25)and 12 mm×8 mm(8/25)in size.Two patients required the implantation of a second device.The follow-up period was 2.1±1.4 years.Complete VSD closure was achieved in 48%of cases immediately after the intervention,74%during 2.1±1.6 months after the procedure,and 81%over follow-up.The remaining patients had a trivial residual defect.During the follow-up,approximately one-third of patients developed trivial aortic and mitral valve regurgitation,and half of the patients acquired trace/mild tricuspid regurgitation.Standardized(z-score)left ventricular end-diastolic diameter(0.15±0.37 vs.0.92±0.82,p=0.005)and left atrium dimension(0.47±0.58 vs.1.89±1.11,p=0.005),as well as the left atrium to aortic root ratio(1.2±0.1 vs.1.4±0.2,p=0.005)showed a significant decrease over follow-up related to the period before intervention.Conclusion:Intervention with Nit-OccludLêVSD coil showed appropriate results regarding VSD closure rate,complications,and chamber remodeling.The introduction of this device into clinical practice is a significant step forward in transcatheter perimembranous VSD occlusion.
文摘Objective: The aim is to study the trends in ventricular septal defect (VSD) mortality in children in Kazakhstan.Methods: The retrospective study was done for the period 2011–2020. Descriptive and analytical methods of epidemiologywere applied. The universally acknowledged methodology used in sanitary statistics is used to calculatethe extensive, crude, and age-specific mortality rates. Results: Kazakhstan is thought to be seeing an increase inmortality from VSDs in children. As a result, this study for the years 2011 to 2020 was conducted to retrospectivelyassess data from the central registration of the Bureau of National Statistics that was available throughoutthe nation. Age-standardized mortality data were obtained and compared between age categories. It was shownthat 507 children died from this condition throughout the time period under study. The average annual standardizedmortality rate was 1.88 per 100,000 population and tended to decrease over time. The peak of mortality wasnoted at the age of up to 1 year, namely the neonatal period. Cartogram mortality rates were calculated usingstandardized indicators. Additionally, age-sex variations were taken into account when performing all calculations.Conclusion: In recent years, the death rate from VSD has declined from 1.5 to 0.6 per 100,000 people, withthe trend remaining constant (T = 1.4%, R^(2) = 0.5825). The analysis of mortality trends related to VSD is crucial inboth theoretical and practical aspects, as it enables early detection and treatment of VSDs. The findings of thisstudy will be valuable to public health authorities in developing a strategy to treat VSDs effectively.
文摘Background:Ventricular septal defect(VSD)is the most common congenital heart disease.Transcatheter VSD closure is an effective treatment for patients with muscular and perimembranous VSD.However,there is a limit data for outlet VSD,especially impact to the aortic valve leaflet after transcatheter closure.This study aims to assess the outcomes of transcatheter closure of the outlet-type ventricular septal defect(OVSD)after 1 postoperative year.Methods:A retrospective study was performed including 50 patients who underwent transcatheter(n=25)and surgical(n=25)OVSD closure during the exact time frame at two medical centres.Results:The median age and body weight of patients in the transcatheter group were significantly higher than those of patients in the surgical group(7.0 vs.2.8 years;27.0 vs.11.4 kg;p<0.01).The defect size in the surgical group was significantly larger than that in the transcatheter group(5.0 vs.3.0 mm;p<0.01).All OVSD patients have successful transcatheter closure(100%)as effective as surgical closure.Less than small residual shunt was present 20%and 8%immediately after the procedure in the transcatheter and surgical groups(p=0.50),which decreased to 12%and 4%at the 1-year follow-up(p=0.61),respectively.No incidence of complete atrioventricular block and other complications was observed in both groups,and no significant differences were noted in the new onset or worsening of the aortic regurgitation in both groups(p=1.0).Conclusions:Transcatheter treatment could be effectively and safely achieved for OVSD closure at 1-year follow-up.
文摘<strong>Background:</strong> <span style="font-family:;" "="">Children presenting with physical features of chondro-ectodermal</span><span style="font-family:;" "=""> dysplasia (Ellis-Van Creveld syndrome) such as skeletal and joint abnormalities often have concomitant congenital cardiac anomalies. Presence of cardiorespiratory symptoms in children with Ellis-Van Craved syndrome warrants a thorough cardiologic evaluation to recognize and treat underlying congenital heart anomaly. <b>Aim:</b> A child with physical stigmata of Ellis-Van-Creveld syndrome is evaluated to detect an associated congenital cardiac anomaly and <span>accomplish successful repair of the underlying cardiac lesion to reduce the cardiac</span> related morbidity and improve the patient survival. <b>Case Presentation:</b> Ten year</span><span style="font-family:;" "="">s</span><span style="font-family:;" "=""> old boy with chondroectodermal dysplasia (dental anomalies, genu valgum and other skeletal abnormalities) presented with dyspnea and cyanosis. Cardiac evaluation by 2D echo revealed an atrioventricular (AV) canal septal defect with AV valve regurgitation and a common atrium. Angiocardiography showed a goose neck deformity of the left ventricular outflow tract. <span>The Qp/Qs was 3.4: 1, with systemic arterial oxygen desaturation (SaO<sub>2</sub> of 0.7) </span>and O<sub>2</sub> saturation in the common atrium was 0.7. The pulmonary venous connections to the common atrium were anomalous. Atriotomy on cardiopulmonary bypass and on a cardioplegic arrest discerned a partial AV canal septal defect with a common bridging leaflet, clefts in septal leaflets of tricuspid and mitral vlalves, an incompletely closed interventricular communication, and a common atrium with highly anomalous pulmonary venous insertions well anterior (8</span><span style="font-family:;" "=""> </span><span style="font-family:;" "="">cm) to vena caval orifices. Intracardiac repair was performed with two patches of Goertex to partition the common atrium into the pulmonary and systemic venous chambers after repair of the partial AV canal septal defect. Patient required only a temporary afterload reduction with enalapril;otherwise patient had an uneventful postoperative course. At a 2</span><span style="font-family:;" "="">-</span><span style="font-family:;" "="">year follow-up, the child was well without AV valve regurgitation and had normal <span>biventricular function. <b>Conclusion:</b> A child with Ellis-Van-Creveld syndrome</span> with skeletal abnormalities and dental anomalies had manifested with cardio-respiratory symptoms. Preoperative cardiac and intraoperative evaluation showed a common atrium with severely anomalous pulmonary venous connection and partial AV canal septal defect. Successful biventricular repair was accomplished by repairing the partial AV canal septal defect and partitioning <span>the common atrium into left and right atrium by a complex atrial routing tech<span>nique with two patches of Gore-Tex. On a follow-up at 2 years</span></span></span><span style="font-family:;" "="">, </span><span style="font-family:;" "="">the patient had</span><span style="font-family:;" "=""> adequate biventricular function without AV valve regurgitation.</span> <div class="__kindeditor_paste__" style="position:absolute;width:1px;height:1px;overflow:hidden;left:-1981px;top:0px;white-space:nowrap;"> <table width="100%" border="0" cellpadding="0" cellspacing="1" bgcolor="#cacfd2" style="border:0px solid #CCCCCC;line-height:25px;width:1041px;color:#000000;font-family:宋体, Arial, sans-serif;"> <tbody> <tr style="background-color:#FAFBFD;"> <td style="text-align:center;font-size:14px;vertical-align:middle;"> <div align="center"> 114264<strong></strong> </div> </td> </tr> </tbody> </table> </div>
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.
基金This study was supported by grant from Doctoral Start-Up Foundation of Liaoning Province of China(2019-BS-266).
文摘Objective:This study was designed to determine the long-term safety and efficacy of using the Amplatzer Duct Occluder II(ADO II)for the closure of various ventricular septal defects(VSDs).Methods:From January 2011 to December 2019,selected VSD patients were treated through transcatheter intervention using ADO II occluders.The closure results and complications from 188 patients,involving 167 perimembranous ventricular septal defects(pmVSDs),9 intracristal VSDs,11 post surgery residual shunts and 1 post closure residual shunt with the mean outlet diameter3.1±0.8 mm under angiography,were enrolled in this study.Results:The success rate was 98.9%for all procedures.During the median 77-month follow-up period,no cases of complete atrioventricular block(cAVB),infective endocarditis or death occurred.One major adverse event(0.5%)was recorded:cerebrovascular accident occurred 1 day after the procedure in one patient who was transferred to the neurology department.The residual shunt rate was 44.6%,which was the most common minor adverse event.The cardiac conduction block rate was 4.3%.Specifically,one pmVSD patient developed intermittent LBBB during the 28-month follow-up.There were 3 patients(1.6%)with new-onset mild tricuspid insufficiency,and the insufficiency degree was stable during follow-up.There was no new-onset aortic insufficiency that occurred.Conclusions:Transcatheter closure of pmVSDs,some intracristal VSDs,some postsurgery or postclosure residual shunts using ADO II occluders were both safe and effective and yielded excellent long-term results in selected patients.
基金the Natural Science Foundation of Hunan Province,China(Grant No.2019JJ80106).
文摘Background: Interventricular septal hematoma is a rare complication after congenital cardiac repair. The managementvaries according to the literature. We present our experience with this rare complication. Methods:Echocardiography database were reviewed with the term ‘‘hematoma’’ or “hypoechoic mass” for patients whounderwent congenital heart surgery from January 2018 to December 2021 at our institution to identify potentialinterventricular septal hematoma cases. Relevant data of the patients identified were collected. Focus was put onthe presentation, management, outcomes according to patent medical charts and serial echocardiographic reportdata. Results: In total, there were 5 patients included. The mean age and weight at surgery were 5.5 ± 3.6 monthsand 5.5 ± 1.4 kg, respectively. Four patients were diagnosed with ventricular septal defect and the other one beingdouble outlet of the right ventricle. While all patients had intraoperative transesophageal echocardiography, 80%(4 of 5) of Interventricular septal hematoma were revealed intraoperatively. Only one patient received hematomadrainage intraoperatively while the other 3 identified in the operating room were only closely observed. One afterventricular septal defect repair presented continuous dysfunction of the left ventricle at the last follow-up, whilethe others were doing well. All hematomas resolved completely with a mean time to interventricular septal hematomaresolution of 35.8 ± 16.9 days. Conclusion: Infants seem to be at a higher risk for Interventricular septalhematoma following congenital heart surgery. While the majority of interventricular septal hematoma has abenign postoperative course, some may result in ventricular dysfunction. Management strategies may be chosenon a case-by-case basis.
文摘In this editorial,we comment on the article by Kong et al published in the recent issue of the World Journal of Cardiology.In this interesting case,the authors present the challenges faced in managing a 13-year-old patient with Down syndrome(DS)and congenital heart disease(CHD)associated with pulmonary arterial hypertension.In this distinct population,the Authors underscore the need for early diagnosis and management as well as the need of a multidisciplinary approach for decision making.It seems that the occurrence of CHD in patients with DS adds layers of complexity to their clinical management.This editorial aims to provide a comprehensive overview of the intricate interplay between DS and congenital heart disorders,offering insights into the nuanced diagnostic and therapeutic considerations for physicians.
文摘Congenital ventricular septal defect (VSD) spontaneous close induced by transcatheter treatment is rare and has not yet been reported. We report on one case of VSD spontaneous close induced by transcatheter treatment in a 10 years old girl.
文摘Objectives To evaluate the therapeutic effect of transcatheter Amplatzer device on the closure of ventricular septal defect (VSD). Methods Among 143 patients with VSD, 135 patients with perimembrane VSD and 2 with muscular VSD aged 2.5 -28 years old, were successfully closed with Amplatzer oeeluder device by the pereutaneous guidwire through femoral artery-VSD-femoral vein route under the guidance of fluoroscopy, ventrieulography and transthoracic echocardiography (TIE). The diameters of the VSDs were 2.3-15.7 (6.90±2.76)mm by left ventriculography. Results The success rate of transcatheter closure of VSD with Amplatzer devices was 96%(137/143). Minimal residual amount of shunts were found in one patient, although the shunts was decreased one month after the procedure. There were one patient who had respiratory arrest during the procedure, 7 patients(5%)had conduction disturbance, 3 patients had complete left bundle branch block, 2 patients had complete right bundle branch block, 1 patient had Ⅰ degree atrial-ventricular block and 1 patient had Ⅲ degree atr/al-ventricular block during hospitalization. The diameters of the occluder ranged from 4 to 23 (9.13±3.31)mm and were symmetrical in 122 patients and asymmetrical in 15 patients. Conclusions Transcatheter closure of the perimembranous ventrieular septal defect using Amplatzer VSD occluder device is an efficient method for patients with the perimembranous VSD. The operation is simple with a high success rate and a good effect.
文摘Objective To evaluate the efficacy of minimally invasive perventricular device closure of ventricular septal defect(VSD). Methods Between September 2011 and February 2013, we collected 40 patients who underwent perventricular closure via a small lower sternal incision(minimally invasive group), aged 15.5±3.5 years(12 months to 32 years) with a body weight of 24.2±7.5 kg(10.8-58.0 kg). The mean size of VSD was 5.6±0.5 mm(2-14 mm). Another 40 patients were included as the surgical group, receiving the conventional surgical repair of VSD. The device of the minimally invasive group was released under the guidance of transesophageal echocardiography. Success rate, cardiac indicators, and clinical outcomes of the 2 groups were compared. Results The patients in the surgical group and those in the minimally invasive group showed similar results in success rate(both 97.5%). The procedure time, intensive care unit stay, hospital stay, and postoperative recovery time in the minimally invasive group were significantly shorter than those in the surgical group(58±21 minutes versus 145±26 minutes, 2±1 days versus 8±3 days, 5±1 days versus 16±6 days, 3±1 days versus 90±20 days, all P<0.05). The minimally invasive group had a higher incidence of conduction anomalies(17.5% versus 2.5%, P<0.05). In the follow-up period of 3-12 months, there was no new residual shunt, noticeable aortic regurgitation, significant arrhythmias, or device failure except for new complications in the surgical group. Conclusions The success rate of minimally invasive perventricular device closure of VSD under transesophageal echocardiography guidance is similar to that of conventional surgical repair, but the short-term outcomes of the minimally invasive approach is much better. Long-term follow-up is necessary to confirm the effectiveness of this technique.
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.
基金supported by National Natural Science Fund of China (No.30871079)National Science Foundation of Jiangsu province (No. BK2007232)
文摘The aim of this study was to examine how somatic mutations of the GATA4 gene contributed to the genesis of ventricular septal defect (VSD). The coding and intron-exon boundary regions of GATA4 were sequenced of DNA samples from peripheral blood cells and cardiac tissues of twenty surgically treated probands with VSD. Seven novel heterozygous variants were detected in cardiac tissues from VSD patients, but they were not detected in the peripheral blood cells of VSD patients or in 500 healthy control samples. We replicated 14 single nucleotide polymorphisms (SNPs) reported in NCBI. Bioinformatics analysis was performed to analyze the possible mechanism by which mutations were linked to VSD. Among those variants, c. 1004C〉A (p.S335X) occurred in the highly conserved domain of GATA4 and generated a termination codon, which led to the production of truncated GATA4. The seven novel heterozygous GATA4 mutations were only identified in cardiac tissues with VSD, suggesting that they are of somatic origin. A higher mutation rate in cardiac tissues than in peripheral blood cells implies that the genetic contribution to VSD may have been underestimated.
文摘Study: Retrospective study of 33 patients with multiple VSD during March 2009 to March 2014. Technique: The defects were located by injecting the cardioplegia solution into left atrium after occluding the pulmonary artery. Through right atrial approach, the large ventricular septal defects were closed by pericardial patch. The smaller ventricular septal defects were closed directly. Results: Out of 33 patients, 20 patients were multiple ventricular septal defects, 5 patients were atrioventricular canal defect with multiple ventricular septal defects and 8 patients were transposition of great arteries with multiple ventricular septal defects. All the patients had severe pulmonary arterial hypertension. The mean age and body weight at repair was 4.51 months and 5.41 kg respectively. Failure to locate additional ventricular septal defects happened in 1 patient where the pulmonary artery banding was done. Six patients had residual ventricular septal defect after surgery, and no significant left to right shunted. One patient had permanent pacemaker implanted in the postoperative period due to the heart block. There was no early and late mortality. Conclusion: Management of multiple ventricular septal defects is quite complex. Right atrial approach of ventricular septal defects closure is safe, simple and effective in closure of multiple ventricular septal defects.
文摘Background: There is a need for data on epidemiological, clinical and therapeutic aspects of ventricular septal defect among children in?Sub-Saharan Africa. Objective: The aim of this study was to determine the prevalence, epidemioclinical, echocardiographic, therapeutic and evolutionary aspects of ventricular septal defects (VSD) in the pediatric department of the University Hospital Center (CHUSS) of Bobo-Dioulasso. Methods: This study was a descriptive cross-sectional study, conducted from November 2013 to December 2016. All children aged 1 to 179 months seen at the pediatric consultation in CHUSS were included. CIV was confirmed with Doppler echocardiography. Results: Out of 36,240 children who received consultation in the pediatric ward of CHUSS during the study period, one hundred (100) cases of them had congenital heart disease representing a hospital prevalence of 2.76%. This was diagnosed with Doppler echocardiography. Of these, 88% were VSD isolated or associated with other cardiac malformations. Isolated form was reported in 54.3% of cases. The average?age at diagnosis was 39.6 months. The sex ratio was 1.05. Perimembranous topography and hemodynamic type 2 were the highest, representing 56.8% and 35.2% respectively. The indication for surgical repair was recommended for 81.8% of the cases, but only 9.7% of these cases benefited from cardiac surgery. The rest were for medicalcare with a high proportion of lost to follow-up (48.9%). Conclusion: VSD is the most common congenital heart disease. Its care is mainly surgical. This cardiac surgery is non-existent in Burkina Faso. The design of multidisciplinary strategies associated with an optimization of the means of the countries of Sub-Saharan Africa could improve the management of this cardiopathy.
文摘An 86-year-old male patient was admitted in our cardiology ward with signs of congestive biventricular heart failure. The patient presented with deteriorating dyspnea on mild exertion and at rest the last days, compatible with class NYHA Ⅲ-Ⅳ heart failure, bilateral peripheral oedema, increased NT-proBNP (9198 pg/mL), mildly elevated Troponin (TnT 64 pg/mL), interstitial pulmonary oedema and bilateral pleural effusions in chest X ray.
基金This work was supported by a grant from the National Science Foundation of China[Grant No.81700286].
文摘Background:Communication between the right pulmonary artery(RPA)and left atrium(LA)is a rare cause of central cyanosis in pediatric patients.Case presentation:We describe a 3-year-old female patient with an oxygen saturation of 70%at admission.The echocardiogram indicated an abnormal color flow Doppler in the LA and she underwent standard cardiac catheterization.The angiography of pulmonary artery revealed a 7.4 mm×7.6 mm fistula between the RPA and LA and achieved successful closure using ventricular septal defect occlusion.Conclusion:The fistula between pulmonary artery and left atrium is an extremely rare but treatable congenital defect.It should be considered in differential diagnosis of cyanosis in children.