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Journal of the American Heart... Jun 2024Sinus venosus atrial septal defect (SVASD) is a rare congenital cardiac anomaly comprising 5% to 10% of all atrial septal defects. Although surgical closure is the... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Sinus venosus atrial septal defect (SVASD) is a rare congenital cardiac anomaly comprising 5% to 10% of all atrial septal defects. Although surgical closure is the standard treatment for SVASD, data on outcomes have been confined to small cohorts. Thus, we conducted a systematic review of the outcomes of SVASD repair.
METHODS AND RESULTS
The primary outcome was death. Secondary outcomes encompassed atrial fibrillation, sinus node dysfunction, pacemaker insertion, cerebrovascular accident, reoperation, residual septal defect, superior vena cava obstruction, and reimplanted pulmonary vein obstruction. Pooled incidences of outcomes were calculated using a random-effects model. Forty studies involving 1320 patients who underwent SVASD repair were included. The majority were male patients (55.4%), with 88.0% presenting with associated anomalous pulmonary venous connection. The weighted mean age was 18.6±12.5 years, and the overall weighted mean follow-up period was 8.6±10.4 years. The in-hospital mortality rate was 0.24%, with a 30-day mortality rate of 0.5% reported in 780 patients. Incidences of atrial fibrillation, sinus node dysfunction, pacemaker insertion, and cerebrovascular accident over the long-term follow-up were 3.3% (2.18%-4.93%), 6.5% (5.09%-8.2%), 2.23% (1.34%-3.57%), and 2.03% (0.89%-2.46%) respectively. Reoperation occurred in 1.36% (0.68%-2.42%) of surgeries, residual septal defect in 1.34% (0.69%-2.42%), superior vena cava obstruction in 1.76% (1.02%-2.9%), and reimplanted pulmonary vein obstruction in 1.4% (0.7%-2.49%).
CONCLUSIONS
This is the first comprehensive analysis of outcomes following surgical repair of SVASD. The findings affirm the safety and effectiveness of surgery, establishing a reference point for evaluating emerging transcatheter therapies. Safety and efficacy profiles comparable to surgical repair are essential for widespread adoption of transcatheter treatments.
Topics: Humans; Heart Septal Defects, Atrial; Treatment Outcome; Cardiac Surgical Procedures; Postoperative Complications; Male; Adolescent; Young Adult; Female; Child; Hospital Mortality; Adult
PubMed: 38874063
DOI: 10.1161/JAHA.123.033686 -
Langenbeck's Archives of Surgery Jun 2024Tracheoesophageal fistula (TEF) especially malignant TEF (mTEF) is an uncommon yet critical medical condition necessitating immediate intervention. This life-threatening...
INTRODUCTION
Tracheoesophageal fistula (TEF) especially malignant TEF (mTEF) is an uncommon yet critical medical condition necessitating immediate intervention. This life-threatening condition frequently manifests in critically ill patients who are dependent on prolonged mechanical ventilation and are unsuitable candidates for thoracotomy due to their compromised health status. The Management of these mTEF patients remain a significant challenge.This study aimed to evaluate the safety and efficacy of using a cardiac septal occluder for the closure of mTEF.
METHODS
8 patients with mTEF underwent closure surgery using atrial/ventricular septal defect (ASD/VSD) septal occluders at the Respiratory Department of HuBei Yichang Central People's Hospital from 2021 to 2023. The procedure involved percutaneous placement of the occluder through the fistula to achieve closure.
RESULTS
The placement of the cardiac septal occluder was successfully achieved with ease and efficiency in all patients. The study demonstrated that the use of cardiac septal occluder therapy in patients with mTEF can alleviate symptoms, improve quality of life, and enhance survival rates, with no significant complications observed. Furthermore, the study provided comprehensive details on surgical indications, preoperative evaluation and diagnosis, selection of occluder, methods of occlusion, and postoperative care.
CONCLUSIONS
The application of cardiac septal occluder in the treatment of mTEF is a safe and effective palliative treatment. This approach may be particularly beneficial for patients with a high risk of complications and mortality associated with traditional surgical interventions.
Topics: Humans; Male; Tracheoesophageal Fistula; Palliative Care; Septal Occluder Device; Female; Middle Aged; Aged; Minimally Invasive Surgical Procedures; Treatment Outcome; Retrospective Studies; Adult; Quality of Life
PubMed: 38822914
DOI: 10.1007/s00423-024-03363-3 -
The Journal of Invasive Cardiology Apr 2024Atrial septal abnormalities are common congenital lesions that remain asymptomatic in many patients until adulthood. Adults with atrial septal defects (ASD) most...
Atrial septal abnormalities are common congenital lesions that remain asymptomatic in many patients until adulthood. Adults with atrial septal defects (ASD) most commonly have ostium secundum ASD. Transcatheter closure has become increasingly popular in recent years as a successful alternative method to surgery for treating ASD and patent foramen ovale (PFO). The overall rate of ASD transcatheter closure device embolization has been reported to be less than 1%; however, retrieving the device via surgery or by trans-catheter route can be necessary. The current manuscript describes a systematic review of the techniques used to retrieve ASD closure devices, as well as their success rates, complications, and limitations. A comprehensive search was performed covering various databases including PubMed, MEDLINE, SCOPUS, Google Scholar, and Cochrane Library from inception until April 2022 for English-published case reports, case series, and experimental studies investigating the indications, safety, and limitations of ASD closure and ASD device retrieval by trans-catheter approaches. Finally, 20 studies were included in our review. Our findings showed that most of the studies used a single snare technique; of these, all but one reported 100% success. Double tool retrieval methods (snare plus snare, snare plus bioptome, or snare plus forceps) and the gooseneck snare technique yielded 100% success. One study that used the lasso technique reported unsuccessful retrieval and the need for surgical intervention. More recently, the novel "coronary wire trap technique" was introduced, which provides a simpler method for embolized device removal by trapping the device for retrieval using coronary wire.
Topics: Adult; Humans; Foramen Ovale, Patent; Treatment Outcome; Cardiac Catheterization; Septal Occluder Device; Heart Septal Defects, Atrial; Cardiac Catheters
PubMed: 38412436
DOI: 10.25270/jic/23.00290 -
European Journal of Medical Genetics Apr 2024T-Box Transcription Factor 5 (TBX5) variants are associated with Holt-Oram syndrome. Holt-Oram syndrome display phenotypic variability, regarding upper limb defects,... (Review)
Review
T-Box Transcription Factor 5 (TBX5) variants are associated with Holt-Oram syndrome. Holt-Oram syndrome display phenotypic variability, regarding upper limb defects, congenital heart defects, and arrhythmias. To investigate the genotype-phenotype relationship between TBX5 variants and cardiac disease, we performed a systematic review of the literature. Through the systematic review we identified 108 variants in TBX5 associated with a cardiac phenotype in 277 patients. Arrhythmias were more frequent in patients with a missense variant (48% vs 30%, p = 0.009) and upper limb abnormalities were more frequent in patients with protein-truncating variants (85% vs 64%, p = 0.0008). We found clustering of missense variants in the T-box domain. Furthermore, we present a family with atrial septal defects. By whole exome sequencing, we identified a novel missense variant p.Phe232Leu in TBX5. The cardiac phenotype included atrial septal defect, arrhythmias, heart failure, and dilated cardiomyopathy. Clinical examination revealed subtle upper limb abnormalities. Thus, the family corresponds to the diagnostic criteria of Holt-Oram syndrome. We provide an overview of cardiac phenotypes associated with TBX5 variants and show an increased risk of arrhythmias associated to missense variants compared to protein-truncating variants. We report a novel missense variant in TBX5 in a family with an atypical Holt-Oram syndrome phenotype.
Topics: Humans; Abnormalities, Multiple; Heart Defects, Congenital; Heart Septal Defects, Atrial; Lower Extremity Deformities, Congenital; Phenotype; T-Box Domain Proteins; Upper Extremity Deformities, Congenital
PubMed: 38336121
DOI: 10.1016/j.ejmg.2024.104920 -
Monaldi Archives For Chest Disease =... Sep 2023CHARGE syndrome (CS) is a rare genetic disease that affects many areas of the body. The aim of the present systematic review was to evaluate the prevalence and types of...
CHARGE syndrome (CS) is a rare genetic disease that affects many areas of the body. The aim of the present systematic review was to evaluate the prevalence and types of congenital heart diseases (CHDs) in CS and their impact on clinical outcome. A systematic review from 1981 to September 2022 was conducted. Clinical studies that reported the association between CS and CHDs were identified, including a case report of a rare congenital anomaly of the aortic arch (AA) with persistent fifth aortic arch (PFAA). Demographic, clinical and outcome data were extracted and analyzed. Sixty-eight studies (44 case reports and 24 case series; n=943 CS patients) were included. The prevalence of CHDs was 76.6%, patent ductus arteriosus (PDA) 26%, ventricular (VSD) 21%, atrial septal defects (ASD) 18%, tetralogy of Fallot 11%, aortic abnormalities 24%. PFAA has not been previously reported in CS. Cardiac surgery was performed in more than half of CS patients (150/242, 62%). In-hospital mortality rate was about 9.5% (n=86/900) in case series studies and 12% (n=5/43) in case reports, including cardiovascular (CV) and non-CV causes. CHDs and feeding disorders associated with CS may have a substantial impact on prognosis. CHDs were usually associated with CS and represent important causes of morbidity and mortality. PFAA, although rare, may also be present. The prognosis is highly dependent on the presence of cardiac and non-cardiac developmental abnormalities. Further studies are needed to better identify the main causes of the long-term outcome of CS patients.
PubMed: 37675914
DOI: 10.4081/monaldi.2023.2661 -
Transcatheter closure of atrial septal defect in the elderly: a systematic review and meta-analysis.Heart (British Cardiac Society) Nov 2023Despite the establishment of transcatheter closure as the treatment of choice in adults with secundum atrial septal defects (ASDs), the effectiveness of this approach in... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Despite the establishment of transcatheter closure as the treatment of choice in adults with secundum atrial septal defects (ASDs), the effectiveness of this approach in the elderly is disputed. This systematic review and meta-analysis aims to explore the impact of transcatheter ASD closure in patients ≥60 years old.
METHODS
We systematically searched four major electronic databases (PubMed, CENTRAL (Cochrane Central Register of Controlled Trials), Scopus and Web of Science), ClinicalTrials.gov, article references and grey literature. Primary outcomes were the right ventricular end-diastolic diameter (RVEDD) and the New York Heart Association functional class change, whereas secondary outcomes included systolic pulmonary arterial pressure (sPAP), left ventricular end-diastolic diameter (LVEDD), brain natriuretic peptide (BNP), tricuspid valve regurgitation (TR) change, as well as the rate of atrial arrhythmias and all-cause mortality.
RESULTS
In total, 18 single-arm cohorts comprising 1184 patients were included. RVEDD was reduced after ASD closure (standardised mean difference (SMD) -0.9, 95% CI -1.2 to -0.7). Elderly patients had 9.5 times higher odds of being asymptomatic after ASD closure (95% CI 5.06 to 17.79). Furthermore, ASD closure improved sPAP (mean difference (MD) -10.8, 95% CI -14.6 to -7), LVEDD (SMD 0.8, 95% CI 0.7 to 1.0), TR severity (OR 0.39, 95% CI 0.25 to 0.60) and BNP (MD -68.3, 95% CI -114.4 to -22.1). There was a neutral effect of ASD closure on atrial arrhythmias.
CONCLUSIONS
Transcatheter ASD closure is beneficial for the elderly population since it improves functional capacity, biventricular dimensions, pulmonary pressures, TR severity and BNP. However, the incidence of atrial arrhythmias did not change significantly after the intervention.
PROSPERO REGISTRATION NUMBER
CRD42022378574.
Topics: Adult; Humans; Aged; Middle Aged; Atrial Fibrillation; Tricuspid Valve Insufficiency; Heart Septal Defects, Atrial; Cardiac Catheterization; Treatment Outcome
PubMed: 37380331
DOI: 10.1136/heartjnl-2023-322529 -
Frontiers in Cardiovascular Medicine 2023Transcatheter closure of atrial septal defect (ASD) and patent foramen ovale (PFO) is an established practice, and it requires monitoring and guidance. Both... (Review)
Review
BACKGROUND
Transcatheter closure of atrial septal defect (ASD) and patent foramen ovale (PFO) is an established practice, and it requires monitoring and guidance. Both transoesophageal echocardiography (TEE) and intracardiac echocardiography (ICE) can be used as guidance tools. However, the use of ICE and TEE in structural heart disease is controversial and the advantages and disadvantages of both for ASD and PFO closure need to be investigated. We did a systematic review and meta-analysis to compare the efficacy and safety of TEE and ICE for guiding transcatheter closure of ASD and PFO.
METHODS
A systematic search of Embase, PubMed, Cochrane library, Web of Science was conducted from inception to May 2022. The outcomes of this study included average time for both fluoroscopy and the procedure, complete closure, length of stay at hospital and adverse events. This study was performed using mean difference (MD), relative risk (RR) and 95% confidence interval (CI).
RESULTS
The meta-analysis was conducted with a total of 11 studies, involving 4,748 patients were included in meta-analysis, including 2,386 patients in the ICE group and 2,362 patients in the TEE group. The results of the meta-analysis showed that compared with TEE, ICE was shorter in time both fluoroscopy [MD: -3.72 (95%CI: -4.09 to -3.34) minutes, < 0.00001] and the procedure [MD: -6.43 (95%CI: -7.65 to -5.21) minutes, < 0.00001], shorter length of stay at hospital [MD = -0.95 (95% CI = -1.21 to -0.69) days, < 0.00001], lower incidence of adverse events (RR = 0.72, 95%CI: 0.62 to 0.84, < 0.0001), and the arrhythmia (RR = 0.50, 95% CI = 0.27 to 0.94, = 0.03) and vascular complications (RR = 0.52, 95%CI = 0.29 to 0.92, = 0.02) in ICE group were lower than those in TEE group. No significant difference in complete closure was found between ICE and TEE (RR = 1.00, 95% CI = 0.98 to 1.03, = 0.74).
CONCLUSION
Under the premise of ensuring successful rate of complete closure, ICE can shorten time between fluoroscopy and procedure and length of stay at hospital, and there was no increase in adverse events. However, more high-quality studies are needed to confirm the benefits of using ICE in ASD and PFO closure.
PubMed: 37215547
DOI: 10.3389/fcvm.2023.1082663 -
Pediatric Surgery International Mar 2023Cardiac anomalies occur frequently in patients with congenital duodenal obstruction (DO). However, the exact occurrence and the type of associated anomalies remain... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Cardiac anomalies occur frequently in patients with congenital duodenal obstruction (DO). However, the exact occurrence and the type of associated anomalies remain unknown. Therefore, the aim of this systematic review is to aggregate the available literatures on cardiac anomalies in patients with DO.
METHODS
In July 2022, a search was performed in PubMed and Embase.com. Studies describing cardiac anomalies in patients with congenital DO were considered eligible. Primary outcome was the pooled percentage of cardiac anomalies in patients with DO. Secondary outcomes were the pooled percentages of the types of cardiac anomalies, type of DO, and trisomy 21. A meta-analysis was performed to pool the reported data.
RESULTS
In total, 99 publications met our eligibility data, representing 6725 patients. The pooled percentage of cardiac anomalies was 29% (95% CI 0.26-0.32). The most common cardiac anomalies were persistent foramen ovale 35% (95% CI 0.20-0.54), ventricular septal defect 33% (95% CI 0.24-0.43), and atrial septal defect 33% (95% CI 0.26-0.41). The most prevalent type of obstruction was type 3 (complete atresias), with a pooled percentage of 54% (95% CI 0.48-0.60). The pooled percentage of Trisomy 21 in patients with DO was 28% (95% CI 0.26-0.31).
CONCLUSION
This review shows cardiac anomalies are found in one-third of the patients with DO regardless of the presence of trisomy 21. Therefore, we recommend that patients with DO should receive preoperative cardiac screening.
LEVEL OF EVIDENCE
II.
Topics: Humans; Child; Down Syndrome; Duodenal Obstruction; Heart Defects, Congenital
PubMed: 36967411
DOI: 10.1007/s00383-023-05449-3 -
Spectrum of congenital heart disease in Nepal from 2002-2022: A systematic review and meta-analysis.Health Science Reports Mar 2023Congenital heart diseases are recognized as public health concerns worldwide and Nepal is no exception. This study aims to study congenital heart disease in Nepal on...
BACKGROUND
Congenital heart diseases are recognized as public health concerns worldwide and Nepal is no exception. This study aims to study congenital heart disease in Nepal on grounds of burden, commonest type, common presentations, and associated noncardiac anomalies so that its spectrum can be known for prompt diagnosis and adoption of screening protocols.
METHODS
Relevant articles were searched in electronic databases using appropriate search terms and Boolean operators. Data were extracted in Excel and analyzed in Comprehensive Meta-Analysis Software. The proportion was used as an effect measure and a fixed or random-effect model was used as per the heterogeneity. Forest plots were used to give visual feedback.
RESULTS
A total of nine studies were included in the qualitative and quantitative synthesis after a rigorous screening of imported studies. The prevalence of congenital heart disease was 0.7% (Proportion: 0.007; CI: 0.001-0.035; I: 99.263%). The burden of atrial septal defect was 32.1%, ventricular septal defect was 31.1%, patent ductus arteriosus was 12.6% and Tetralogy of Fallot was 7.3%. The most common presentations were respiratory tract infection (54.7%), developmental delay (49.8%), difficulty in breathing (44.5%), failure to thrive (17.1%), and cyanosis (15.9%).
CONCLUSIONS
The prevalence of congenital heart disease in Nepal was 0.7% and the frequency of male patients was higher. The atrial septal defect was the commonest on the whole, while, Tetralogy of Fallot was the commonest among cyanotic variety. Respiratory tract infection was frequently seen at presentation and the most commonly associated noncardiac anomaly was the cleft palate.
PubMed: 36925763
DOI: 10.1002/hsr2.1147 -
Cerebrovascular Diseases (Basel,... 2023The association between a patent foramen ovale (PFO) and cryptogenic stroke (CS) is well established, and the benefits of PFO closure are clearly recognized. This study... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The association between a patent foramen ovale (PFO) and cryptogenic stroke (CS) is well established, and the benefits of PFO closure are clearly recognized. This study aimed to investigate the presence of a residual shunt in patients who have experienced cryptogenic cerebrovascular events following a PFO closure.
METHODS
Two researchers systematically searched the PubMed and Embase online database for pertinent clinical studies published between January 2000 and July 2021 concerning the recurrence of cerebrovascular events after PFO closures.
RESULTS
Upon screening an initial list of 2,342 articles, six studies were identified, involving 2,083 patients. Overall, the analysis indicated a marked difference in the recurrence of cerebrovascular events in 8.89% of residual shunt (RS) cases compared to only 2.90% of non-RS cases. The summary odds ratio was 3.484 (95% confidence interval, 2.169-5.596), which suggested that RS may be a risk factor for recurrent cerebrovascular events in patients that experienced PFO-related cerebrovascular events within 6 months after PFO closure surgery.
CONCLUSIONS
The presence of RS significantly increases the risk of recurrent cerebrovascular events in patients with clinical PFO closure.
Topics: Humans; Stroke; Foramen Ovale, Patent; Cardiac Catheterization; Treatment Outcome; Secondary Prevention; Recurrence; Risk Factors
PubMed: 36882039
DOI: 10.1159/000527457