Outcomes Following Surgical Repair of Sinus Venosus Atrial Septal Defects: A Systematic Review and 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.

Real-World Patent Foramen Ovale (PFO) Closure in Japan　- 30-Day Clinical Outcomes From the AmplatzerTM PFO Occluder Japan Post-Marketing Surveillance Study.

BACKGROUND: The AmplatzerTM PFO Occluder was approved for marketing in Japan in May 2019, and the Amplatzer PFO Occluder Japan Post-marketing Surveillance (PFO Japan PMS) study was initiated in December 2019. This analysis presents 30-day clinical outcomes for PFO Japan PMS study patients.Methods and Results: PFO Japan PMS is a prospective single-arm non-randomized multicenter clinical study. Eligible patients were indicated for patent foramen ovale (PFO) closure and underwent an implant attempt with the AmplatzerTM PFO Occluder. Technical success was defined as successful delivery and release of the occluder; procedural success was defined as technical success with no serious adverse events (SAEs) within 1 day of the procedure. The primary safety endpoint includes predefined device- and/or procedure-related SAEs through 30 days after the procedure. From December 2019 to July 2021, 500 patients were enrolled across 53 Japanese sites. The mean (±SD) patient age was 52.7±15.4 years, and 29.8% of patients were aged >60 years. Technical and procedural success rates were both high (99.8% and 98.8%, respectively). Further, there was only one primary safety endpoint event (0.2%): an episode of asymptomatic paroxysmal atrial fibrillation that occurred 26 days after the procedure. CONCLUSIONS: In this real-world Japanese study with almost one-third of patients aged >60 years, PFO closure with the AmplatzerTM PFO Occluder was performed successfully and safely, with a low incidence of procedure-related atrial arrhythmias.

Better Late Than Never: Definitive Anatomic Repair of Dextro-Transposition of the Great Arteries.

We present a case of anatomic repair of dextro-transposition of the great arteries (d-TGA) with ventricular septal defect (VSD) in a 55-year-old man who presented with acute heart failure. This case highlights the importance of multimodal imaging and multidisciplinary involvement in developing a comprehensive surgical and medical plan for adults with congenital heart disease. We think this is the oldest reported patient undergoing anatomic surgical repair of d-TGA with VSD.

Application of Computed Tomography Angiography in Preoperative Diagnosis of Coarctation of Aorta and Evaluation of Aortic Dilatation in Infants.

OBJECTIVE: To evaluate the occurrence of aortic dilatation and its associated predictors with coarctation of the aorta (CoA) in infants using multi-slice computed tomography (MSCT). METHODS: The clinical data of 47 infantile patients with CoA diagnosed by MSCT and 28 infantile patients with simple ventricular septal defect were analyzed retrospectively. Aortic diameters were measured at six different levels, and aortic sizes were compared by z score. The coarctation site-diaphragm ratio was used to describe the degree of narrowing. Relevant clinical data were collated and analyzed. RESULTS: The dilation rate and z score of the ascending aorta in the severe CoA group were significantly higher than those in the mild CoA group (11 [52.38%] vs. 21 [80.77%], P=0.038 and 2.00 ± 0.48 vs. 2.36 ± 0.43, P=0.010). Pearson's correlation analysis found that the z score of the ascending aorta was negatively correlated with the coarctation site-diaphragm ratio value (r=-0.410, P=0.004). A logistic retrospective analysis found that an increased degree of coarctation was an independent predictor of aortic dilatation (adjusted odds ratio 0.002; 95% confidence interval 0.00-0.819; P=0.043). The z score of the ascending aorta in the severe CoA group was significantly higher than that in the ventricular septal defect group (P<0.05). CONCLUSION: Most infants with CoA can also have significant dilatation of the ascending aorta, and the degree of this dilatation is related to the degree of coarctation. Assessment of aortic diameter and related malformations by MSCT can predict the risk of aortic dilatation in infants with CoA.

Comparative evaluation of microprobe versus conventional transoesophageal echocardiography for PFO closure guidance.

BACKGROUND: Patent foramen ovale (PFO) closure is traditionally guided by transoesophageal echocardiography (TEE) under general anaesthesia, which prolongs procedure duration and increases costs and risks. A transnasal echocardiography with a microTEE-probe (microTNE) is tolerated under conscious sedation and offers an effective alternative to TEE. The aim of this study was to compare the feasibility, safety and time expenditure of PFO closure using conventional TEE versus microTNE guidance. METHODS: Consecutive patients assigned for PFO losure in Helsinki University Hospital from 2003 to 2021 were included in the study (n=336). TEE with general anaesthesia was used until November 2018 (n=167) while microTNE-guided PFO closure (n=169) under conscious sedation was the principal method thereafter. Patients were followed for 3 months after PFO closure. RESULTS: The microTNE-route success rate was 97.2% vs TEE 100% (p=0.06) and procedure success rate was 97.7% with microTNE and 96.0% with TEE-guidance (p=0.54). The procedure time was significantly shorter with microTNE 21±7 min than with TEE 30±13 min (p<0.001). At the beginning of microTNE era, nasal bleeding complication was quite frequent; however, overall complication rates were equal between the groups. However, C reactive protein (CRP) increase was significantly milder with microTNE than TEE 1.0±2.9 vs 3.0±4.0 mg/L (p<0.001). An increase in CRP was independently associated with procedure type (p=0.004) and time (p=0.003). CONCLUSIONS: MicroTNE is a feasible and safe alternative for PFO closure guidance. MicroTNE under conscious sedation shortens procedure duration and induces a milder inflammatory reaction than conventional TEE under general anaesthesia.

Innovative ultrasound-guided one-stop surgery for complex cardiovascular cases: a case report.

Elderly patients with multiple comorbidities often face complex cardiac challenges, including aortic valve issues and atrial septal defects. Traditional open-heart surgery may not be viable for this demographic. Transcatheter aortic valve implantation (TAVI) emerges as a preferred alternative. In this case, a frail patient with multiple comorbidities, atrial septal defect, and significant aortic stenosis and regurgitation underwent a one-stop procedure, combining TAVI and atrial septal defect closure, guided by advanced imaging, including three-dimensional ultrasound. Ultrasound played a pivotal role in the perioperative phase, offering precise screening and guidance. This innovative technique, minimizing surgical trauma and recovery time, significantly improved the patient's quality of life.

Application of Computed Tomography Angiography in Preoperative Diagnosis of Coarctation of Aorta and Evaluation of Aortic Dilatation in Infants

ABSTRACT Objective: To evaluate the occurrence of aortic dilatation and its associated predictors with coarctation of the aorta (CoA) in infants using multi-slice computed tomography (MSCT). Methods: The clinical data of 47 infantile patients with CoA diagnosed by MSCT and 28 infantile patients with simple ventricular septal defect were analyzed retrospectively. Aortic diameters were measured at six different levels, and aortic sizes were compared by z score. The coarctation site-diaphragm ratio was used to describe the degree of narrowing. Relevant clinical data were collated and analyzed. Results: The dilation rate and z score of the ascending aorta in the severe CoA group were significantly higher than those in the mild CoA group (11 [52.38%] vs. 21 [80.77%], P=0.038 and 2.00 ± 0.48 vs. 2.36 ± 0.43, P=0.010). Pearson's correlation analysis found that the z score of the ascending aorta was negatively correlated with the coarctation site-diaphragm ratio value (r=-0.410, P=0.004). A logistic retrospective analysis found that an increased degree of coarctation was an independent predictor of aortic dilatation (adjusted odds ratio 0.002; 95% confidence interval 0.00-0.819; P=0.043). The z score of the ascending aorta in the severe CoA group was significantly higher than that in the ventricular septal defect group (P<0.05). Conclusion: Most infants with CoA can also have significant dilatation of the ascending aorta, and the degree of this dilatation is related to the degree of coarctation. Assessment of aortic diameter and related malformations by MSCT can predict the risk of aortic dilatation in infants with CoA.

Epidemiología del diagnóstico prenatal de defectos congénitos en la provincia de Holguín / Epidemiology of prenatal diagnosis of congenital defects in Holguín province

Introducción: En Cuba, los defectos congénitos constituyen la segunda causa de muerte en niños menores de un año, por lo cual ocupan un lugar prioritario en los programas médicos sociales del país. Objetivo: Evaluar el comportamiento epidemiológico del diagnóstico prenatal de los defectos congénitos en Holguín, Cuba. Métodos: Se realizó un estudio descriptivo y transversal de la epidemiología de los defectos congénitos en la provincia de Holguín, Cuba, en el periodo de enero 2011- junio de 2020. Resultados: Los años con mayor número de defectos congénitos diagnosticados fueron: 2011, 2012, 2017 y 2018 con 308, 253, 290 y 236 pacientes, respectivamente. Los defectos congénitos más frecuentes fueron: cardiovasculares (comunicación interventricular, canal auriculoventricular, transposición de grandes vasos e hipoplasia de cavidades), renales (pielocaliectasia, hidronefrosis, riñones poliquísticos), y del sistema nervioso central (ventriculomegalia, hidrocefalia). El grupo de edad materna donde se realizó mayor número de diagnósticos fue entre 20-24 años, la mayoría en el segundo trimestre de la gestación; en el primer trimestre, el mayor número de defectos congénitos correspondió a los defectos de pared anterior. La tasa de mortalidad infantil por defectos congénitos se mantuvo estable en la mayoría de los años estudiados. Conclusiones: La estabilidad y perfeccionamiento del programa de diagnóstico prenatal de los defectos congénitos, y el asesoramiento genético adecuado, han tenido un resultado epidemiológico favorable en la provincia.

Introduction: congenital defects in Cuba are the second cause of death in children under one year of age that is why they occupy a priority place in the social medical programs of the country. Objective: to evaluate the epidemiological manifestation of the prenatal diagnosis of congenital defects in Holguín, Cuba. Methods: a descriptive and cross-sectional study of the epidemiology of birth defects was carried out in Holguín province, Cuba from January 2011 to June 2020. Results: the years with the highest number of diagnosed birth defects were 2011, 2012, 2017 and 2018 with 308, 253, 290 and 236 patients, respectively. The most frequent birth defects were cardiovascular (ventricular septal defect, atrioventricular canal, transposition of the great vessels and hypoplasia of cavities), renal (pyelokaliectasia, hydronephrosis and polycystic kidneys), and central nervous system (ventriculomegaly and hydrocephalus). The maternal age group in which the highest number of diagnoses was made was between 20-24 years, mostly in the second trimester of pregnancy; the largest number of congenital defects in the first trimester corresponded to anterior wall defects. The infant mortality rate due to congenital defects remained stable in most of the years studied. Conclusions: the stability and improvement of the prenatal diagnosis program for congenital defects, as well as an adequate genetic counseling, have had a favourable epidemiological result in the province.

Neglected Partial Anomalous Pulmonary Venous Return during Atrial Septal Defect Device Closure.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital heart malformation in which 1 or more pulmonary veins drain into the systemic venous circulation or directly into the right atrium instead of the left atrium. It may occur alongside other congenital heart defects, including atrial septal defect (ASD). All patients with newly diagnosed ASD must be evaluated thoroughly for the likelihood of PAPVR to select surgical or percutaneous procedures. Here, we describe a 10-year-old girl with PAPVR who underwent percutaneous device closure of her secundum ASD with her PAPVR neglected at 3 years of age. We had to correct the anomalous venous connection by removing the device during an intricate procedure. The typical connection of the pulmonary veins to the left atrium was reported on her postoperative echocardiography 1 day after surgery. The patient was discharged without complications, and her first follow-up visit 7 days after discharge was unremarkable. While the most accurate diagnostic tools for PAPVR are cardiovascular magnetic resonance imaging and computed tomographic angiography, a careful examination of the pulmonary veins during pulmonary angiography or transesophageal echocardiography in children helps identify PAPVR in patients with ASD.

Transatrial repair of a ventricular septal defect and tricuspid valve replacement after a blunt chest trauma.

In this case report, we provide insight into how we managed this case of a large traumatic ventricular septal defect and concomitant tricuspid valve insufficiency with a transatrial approach to ensure a successful repair.

Complete Transthoracic Echocardiography for the Assessment and Guidance of Percutaneous Atrial Septal Defect Closure in Adults without Balloon Sizing: An Observed Study with a 10-Year Follow-Up.

OBJECTIVES: This study aims to determine if complete transthoracic echocardiography (TTE)-guided percutaneous atrial septal defect (ASD) closure without balloon sizing could be safe and efficacious in adult patients. METHODS: A total of 551 patients with ASDs were enrolled in this study, of which 438 patients underwent percutaneous ASD closure. Patients who received TTE-guided percutaneous ASD closure were classified into group T, and those who underwent a procedure that was guided by transesophageal echocardiography (TEE) were classified into group E. The clinical characteristics and the outcomes of the patients were analyzed. RESULTS: The characteristics were comparable at baseline, except for the body mass index (BMI) (21.6 ± 5.3 vs. 23.8 ± 7.1, p < 0.001) between group T and group E. No significant difference was observed between the two groups regarding in-hospital outcomes, except for the duration of the procedure (29.8 ± 15.3 min vs. 41.5 ± 20.4 min), the length of stay in the hospital (2.1 ± 2.3 d vs. 2.9 ± 2.6 d), and hospital costs (USD 6233.3 ± 312.4 vs. USD 6673.7 ± 446.9). There were no significant differences in the incidences of long-term complications, cardiac chamber sizes, and tricuspid regurgitation severity between the patients in the two groups during the 10-year follow-up period. CONCLUSION: TTE may be as safe and efficacious as TEE for the assessment and guidance of percutaneous ASD closure without balloon sizing in adult patients with lower BMIs who are commonly found in East Asia.

Tetralogy of Fallot with an Anomalous Course of the Brachiocephalic Vein.

CLINICAL DATA: Infant, 11-month-old, male, diagnosis of Tetralogy of Fallot with retrotracheoesophageal course of the brachiocephalic vein. Usual findings of Tetralogy of Fallot on physical examination. Technical description: Chest radiography showed slightly reduced pulmonary vascular markings and no cardiomegaly. Normal preoperative electrocardiogram with postoperative right bundle branch block. Usual findings of Tetralogy of Fallot on echocardiogram. Postoperative computed tomography angiography confirmed left brachiocephalic vein with anomalous retrotracheoesophageal course, configuring a U-shaped garland vein, in addition to postoperative findings of total correction of Tetralogy of Fallot. OPERATION: Complete surgical repair was performed with pulmonary valve commissurotomy and placement of bovine pericardial patch to solve right ventricular outflow tract obstruction, pulmonary trunk enlargement, and ventricular septal defect closure. COMMENTS: Systemic venous drainage may show variations in patients with Tetralogy of Fallot. These abnormalities are usually of little clinical relevance, as they are asymptomatic. We presented a rare case of retrotracheoesophageal course of an anomalous left brachiocephalic vein with intraoperative diagnosis, confirmed by imaging during postoperative follow-up, without compromising clinical management or surgical approach.

A Knife Penetrating the Right Ventricle, Interventricular Septum, and 2 Valves: A Case Report.

Penetrating chest trauma may result in significant intracardiac injury. A traumatic ventricular septal defect is a rare complication that requires surgical management, particularly if heart failure ensues. We report a case of delayed repair of an outlet-type ventricular septal defect and perforation of the aortic and pulmonary valve leaflets following a stab wound. This report highlights diagnostic and surgical considerations and also presents an opportunity to review the conotruncal anatomy, which may be relatively unfamiliar to many adult cardiac surgeons.

Transcatheter closure of atrial septal defect in the elderly: a systematic review and 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.