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
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
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 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
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
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: Ventricular septal rupture (VSR) leading to right ventricular (RV) wall dissection is an extremely rare and life-threatening complication of inferior myocardial infarction (MI) with posterior VSR. Its rare...Background: Ventricular septal rupture (VSR) leading to right ventricular (RV) wall dissection is an extremely rare and life-threatening complication of inferior myocardial infarction (MI) with posterior VSR. Its rare incidence and complex pathology make it difficult to select the appropriate surgical procedures to prevent fatalities. Case Presentation: A 68-year-old woman was transferred to our hospital because of a post-infarction VSR 12 days after symptom onset. Short-axis image obtained using transthoracic echocardiography demonstrated a large posterior VSR. Moreover, the VSR was continuous, with a large echo-free space in the posterior wall of the right ventricle. Color echocardiography showed blood flowing into the echo-free space through the septal defect and blood flowing out into the RV lumen. Coronary angiography revealed complete occlusion of the second segment of the right coronary artery. Thus, dissection of the posterior wall of the right ventricle that continued into the RV lumen was considered to have been caused by the posterior VSR caused by an inferior MI. The patient underwent urgent surgery to repair the VSR using the sandwich double-patch technique by making a posterior RV incision that was repaired using a third patch. No additional procedure was required to block the flow from the cavity of the RV wall dissection into the RV lumen. Postoperative echocardiography and contrast-enhanced computed tomography demonstrated that the VSR was closed securely and the RV wall dissection was almost completely thrombosed. Conclusion: In this case, a patient with a posterior VSR and RV wall dissection was successfully treated using the sandwich double-patch technique with a posterior RV incision. No additional procedure may be needed for RV wall dissection when a secure VSR repair is complete;however, close follow-up is essential to improve the long-term prognosis.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
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
Coronary fistulae and ventricular septal perforation are very rare clinically, and even less caused by cardiac leiomyosarcoma. A case is reported that a 67-year-old female had cardiac leiomyosarcoma with progressive h...Coronary fistulae and ventricular septal perforation are very rare clinically, and even less caused by cardiac leiomyosarcoma. A case is reported that a 67-year-old female had cardiac leiomyosarcoma with progressive heart failure and coronary fistulae and ventricular septal perforation. This case was special since all ante-mortem examinations and cardiac surgery failed to detect the presence of any abnormal car-diac mass. Therefore, the malignant cardiac tumors could appear in an invasive form without mass and be one of the causes of the coronary fistulae and ventricular septal perforation.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 and objectives Right ventricular apical(RVA)pacing has been reported impairing left ventricular(LV)performance.Alternative pacing sites in right ventricle(RV)has been explored to obtain better cardiac funct...Background and objectives Right ventricular apical(RVA)pacing has been reported impairing left ventricular(LV)performance.Alternative pacing sites in right ventricle(RV)has been explored to obtain better cardiac function.Our study was designed to compare the hemodynamic effects of right ventricular septal(RVS)pacing with RVA pacing.Methods Ten elderly patients with chronic atrial fibrillation(AF)and long RR interval or slow ventricular response(VR)received VVI pacing.The hemodynamic difference between RVS and RVA pacing were examined by transthoracic echocardiography(TTE).Results Pacing leads were implanted successfully at the RVA and then RVS in all patients without complication.The left ventricular(LV)parameters,measured during RVA pacing including left ventricular ejection fraction(LVEF),FS,stroke volume(SV)and peak E wave velocity(EV)were decreased significantly compared to baseline data,while during RVS pacing,they were significantly better than those during RVA pacing.However,after 3-6 weeks there was no statistical significant difference between pre-and post-RVS pacing.Conclusions The LV hemodynamic parameters during RVA pacing were significantly worse than baseline data.The short term LV hemodynamic parameters of RVS pacing were significantly better than those of RVA pacing;RVS pacing could improve the hemodynamic effect through maintaining normal ventricular activation sequence and biventricular contraction synchrony in patients with chronic AF and slow ventricular response.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
The corrected shunt flow rate (Fc ) and corrected defect orifice area (Ac) were calculated by modified equation F= 2πR2 ×(NL-VL.voT× Sinθ) in 23 patients with single membranous ventricular septal defect, i...The corrected shunt flow rate (Fc ) and corrected defect orifice area (Ac) were calculated by modified equation F= 2πR2 ×(NL-VL.voT× Sinθ) in 23 patients with single membranous ventricular septal defect, in order to correct the ef fect of left ventricular outflow on flow convergence region on the left septa1 sur-face. The results indicated that F. was closely correlated with Qp -Q5. and Qp/Q5measured by pulsed wave Doppler (r = 0. 95 and r = 0. 81 respectively, P < 0.001 ). And the correlation between A. and the diameter of defect (Dd) rneasureddirectly in two-dimensional views was better than that between uncorrected defectorifice area (A ) and the Dd (r = O- 98 and O- 69, respectively, P< O. Ool ). Theshunt flow rate calculated by ideal equation F= 2ffR2 X NL overestimated the actu-al shunt flow rate in ventricular septal defect, especialIy in mernbranous type.Our study concluded that F. can be used for a more accurate eva1uation of theshunt severity of ventricular septal defect.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
Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume andejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to useRT-3DE...Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume andejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to useRT-3DE to evaluate the changes in biventricular volume and systolic function in children with ventricular septaldefect (VSD) and moderate to severe pulmonary hypertension (PH) before surgery. Methods: In this study18 children with VSD and moderate to severe PH (VSD + PH Group) and 18 healthy children of the sameage (Control Group) were recruited. Biventricular volume and systolic function were evaluated by RT-3DE.The measurements included: left and right ventricular volume indexed to body surface area (BSA), stroke volume(SV) indexed to BSA, and ejection fraction (EF). Results: The results showed left and right ventricular volumeindexed to BSA and SV indexed to BSA were significantly increased in VSD + PH Group (VSD + PH Groupvs. Control Group), LVEDV/BSA (ml/m2): 48.67 ± 21.46 vs. 25.59 ± 6.96, RVEDV/BSA (ml/m2): 55.98 ±15.35 vs. 27.69 ± 4.37, LVSV/BSA (ml/m2): 24.08 ± 9.30 vs. 15.14 ± 4.29, RVSV/BSA (ml/m2): 26.02 ± 8.87 vs.14.11 ± 2.89, (P < 0.05). While for EF in VSD + PH Group decreased (VSD + PH Group vs. Control Group),LVEF: 50.93 ± 7.50% vs. 59.38 ± 7.24%, RVEF: 45.84 ± 7.71% vs. 51.05 ± 6.90% (P < 0.05). Conclusion: Inchildren with VSD and moderate to severe PH, increased biventricular volume and decreased systolic functionwere observed with RT-3DE, but biventricular systolic function remained within acceptable limits. The childrenin this study recovered well after surgery without serious perioperative complications, suggesting that biventricularsystolic function may help facilitate the surgical decision-making process in children with VSD and moderate-toseverePH.展开更多
Objective:To evaluate the efficacy of Amplatzer duct occluder II(ADO II)in the treatment of perimembranous ventricular septal defect(pmVSD)in children.Methods:Between June 2017 and June 2020,13 patients with pmVSD had...Objective:To evaluate the efficacy of Amplatzer duct occluder II(ADO II)in the treatment of perimembranous ventricular septal defect(pmVSD)in children.Methods:Between June 2017 and June 2020,13 patients with pmVSD had attempted transcatheter closure using ADO II,seven of patients were used antegrade approach and six of them were used retrograde approach.Results:There were 8 males and 5 females,age from 1 to 7 years,weight from 10.5 to 31.0 kg,and VSD size from 2.0 to 4.0 mm.Procedure was successful in all cases with the outer diameter of the occluders ranging from 4 to 6 mm.No aortic,tricuspid regurgitation or residual shunt was found in the immediate ultrasound assessment.No arrhythmia was observed in the Holter monitoring 3 days after the intervention.Discharge echocardiography indicated complete shunt closure.No evidence of occluder prolapse,malignant arrhythmia,or intensed valve regurgitation was seen on a median follow-up of 18 months(range,6 to 36 months).Conclusions:Based on our experience,ADO II showed good efficacy in the early and middle stages of pediatric pmVSD closures.展开更多
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.
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.
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 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.
文摘Background: Ventricular septal rupture (VSR) leading to right ventricular (RV) wall dissection is an extremely rare and life-threatening complication of inferior myocardial infarction (MI) with posterior VSR. Its rare incidence and complex pathology make it difficult to select the appropriate surgical procedures to prevent fatalities. Case Presentation: A 68-year-old woman was transferred to our hospital because of a post-infarction VSR 12 days after symptom onset. Short-axis image obtained using transthoracic echocardiography demonstrated a large posterior VSR. Moreover, the VSR was continuous, with a large echo-free space in the posterior wall of the right ventricle. Color echocardiography showed blood flowing into the echo-free space through the septal defect and blood flowing out into the RV lumen. Coronary angiography revealed complete occlusion of the second segment of the right coronary artery. Thus, dissection of the posterior wall of the right ventricle that continued into the RV lumen was considered to have been caused by the posterior VSR caused by an inferior MI. The patient underwent urgent surgery to repair the VSR using the sandwich double-patch technique by making a posterior RV incision that was repaired using a third patch. No additional procedure was required to block the flow from the cavity of the RV wall dissection into the RV lumen. Postoperative echocardiography and contrast-enhanced computed tomography demonstrated that the VSR was closed securely and the RV wall dissection was almost completely thrombosed. Conclusion: In this case, a patient with a posterior VSR and RV wall dissection was successfully treated using the sandwich double-patch technique with a posterior RV incision. No additional procedure may be needed for RV wall dissection when a secure VSR repair is complete;however, close follow-up is essential to improve the long-term prognosis.
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.
文摘Coronary fistulae and ventricular septal perforation are very rare clinically, and even less caused by cardiac leiomyosarcoma. A case is reported that a 67-year-old female had cardiac leiomyosarcoma with progressive heart failure and coronary fistulae and ventricular septal perforation. This case was special since all ante-mortem examinations and cardiac surgery failed to detect the presence of any abnormal car-diac mass. Therefore, the malignant cardiac tumors could appear in an invasive form without mass and be one of the causes of the coronary fistulae and ventricular septal perforation.
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.
文摘Background and objectives Right ventricular apical(RVA)pacing has been reported impairing left ventricular(LV)performance.Alternative pacing sites in right ventricle(RV)has been explored to obtain better cardiac function.Our study was designed to compare the hemodynamic effects of right ventricular septal(RVS)pacing with RVA pacing.Methods Ten elderly patients with chronic atrial fibrillation(AF)and long RR interval or slow ventricular response(VR)received VVI pacing.The hemodynamic difference between RVS and RVA pacing were examined by transthoracic echocardiography(TTE).Results Pacing leads were implanted successfully at the RVA and then RVS in all patients without complication.The left ventricular(LV)parameters,measured during RVA pacing including left ventricular ejection fraction(LVEF),FS,stroke volume(SV)and peak E wave velocity(EV)were decreased significantly compared to baseline data,while during RVS pacing,they were significantly better than those during RVA pacing.However,after 3-6 weeks there was no statistical significant difference between pre-and post-RVS pacing.Conclusions The LV hemodynamic parameters during RVA pacing were significantly worse than baseline data.The short term LV hemodynamic parameters of RVS pacing were significantly better than those of RVA pacing;RVS pacing could improve the hemodynamic effect through maintaining normal ventricular activation sequence and biventricular contraction synchrony in patients with chronic AF and slow ventricular response.
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The corrected shunt flow rate (Fc ) and corrected defect orifice area (Ac) were calculated by modified equation F= 2πR2 ×(NL-VL.voT× Sinθ) in 23 patients with single membranous ventricular septal defect, in order to correct the ef fect of left ventricular outflow on flow convergence region on the left septa1 sur-face. The results indicated that F. was closely correlated with Qp -Q5. and Qp/Q5measured by pulsed wave Doppler (r = 0. 95 and r = 0. 81 respectively, P < 0.001 ). And the correlation between A. and the diameter of defect (Dd) rneasureddirectly in two-dimensional views was better than that between uncorrected defectorifice area (A ) and the Dd (r = O- 98 and O- 69, respectively, P< O. Ool ). Theshunt flow rate calculated by ideal equation F= 2ffR2 X NL overestimated the actu-al shunt flow rate in ventricular septal defect, especialIy in mernbranous type.Our study concluded that F. can be used for a more accurate eva1uation of theshunt severity of ventricular septal defect.
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.
基金Wuhan Health and Family Planning Commission Grant/Award(WX16D18).
文摘Background: Real-time three-dimensional echocardiography (RT-3DE) could obtain ventricular volume andejection fraction rapidly and non-invasively without relying on ventricular morphology. This study aims to useRT-3DE to evaluate the changes in biventricular volume and systolic function in children with ventricular septaldefect (VSD) and moderate to severe pulmonary hypertension (PH) before surgery. Methods: In this study18 children with VSD and moderate to severe PH (VSD + PH Group) and 18 healthy children of the sameage (Control Group) were recruited. Biventricular volume and systolic function were evaluated by RT-3DE.The measurements included: left and right ventricular volume indexed to body surface area (BSA), stroke volume(SV) indexed to BSA, and ejection fraction (EF). Results: The results showed left and right ventricular volumeindexed to BSA and SV indexed to BSA were significantly increased in VSD + PH Group (VSD + PH Groupvs. Control Group), LVEDV/BSA (ml/m2): 48.67 ± 21.46 vs. 25.59 ± 6.96, RVEDV/BSA (ml/m2): 55.98 ±15.35 vs. 27.69 ± 4.37, LVSV/BSA (ml/m2): 24.08 ± 9.30 vs. 15.14 ± 4.29, RVSV/BSA (ml/m2): 26.02 ± 8.87 vs.14.11 ± 2.89, (P < 0.05). While for EF in VSD + PH Group decreased (VSD + PH Group vs. Control Group),LVEF: 50.93 ± 7.50% vs. 59.38 ± 7.24%, RVEF: 45.84 ± 7.71% vs. 51.05 ± 6.90% (P < 0.05). Conclusion: Inchildren with VSD and moderate to severe PH, increased biventricular volume and decreased systolic functionwere observed with RT-3DE, but biventricular systolic function remained within acceptable limits. The childrenin this study recovered well after surgery without serious perioperative complications, suggesting that biventricularsystolic function may help facilitate the surgical decision-making process in children with VSD and moderate-toseverePH.
基金The work was financially supported by the National Natural Science Foundation of China(81770316,81970249 to SLP)Shandong Taishan Scholarship(2018 to SLP)and Qingdao Science and Technology Plan(20-3-4-47-nsh).
文摘Objective:To evaluate the efficacy of Amplatzer duct occluder II(ADO II)in the treatment of perimembranous ventricular septal defect(pmVSD)in children.Methods:Between June 2017 and June 2020,13 patients with pmVSD had attempted transcatheter closure using ADO II,seven of patients were used antegrade approach and six of them were used retrograde approach.Results:There were 8 males and 5 females,age from 1 to 7 years,weight from 10.5 to 31.0 kg,and VSD size from 2.0 to 4.0 mm.Procedure was successful in all cases with the outer diameter of the occluders ranging from 4 to 6 mm.No aortic,tricuspid regurgitation or residual shunt was found in the immediate ultrasound assessment.No arrhythmia was observed in the Holter monitoring 3 days after the intervention.Discharge echocardiography indicated complete shunt closure.No evidence of occluder prolapse,malignant arrhythmia,or intensed valve regurgitation was seen on a median follow-up of 18 months(range,6 to 36 months).Conclusions:Based on our experience,ADO II showed good efficacy in the early and middle stages of pediatric pmVSD closures.