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Interventional Cardiology Clinics Jul 2024Superior sinus venosus defects (SVD) are interatrial communications located above the confines of the oval fossa, where unroofing of the right upper pulmonary vein leads...
Superior sinus venosus defects (SVD) are interatrial communications located above the confines of the oval fossa, where unroofing of the right upper pulmonary vein leads to its anomalous drainage to the superior venacava. Recent emergence of transcatheter closure of these defects using covered stents is an attractive alternative option especially in adults with additional comorbidities. This article focuses on various aspects of non-surgical closure of SVD, including patient selection, appropriate hardware options, step-by-step procedural details, evolution and modifications in the techniques over the last decade, protocols for follow-up evaluation, and potential complications associated with this intervention.
Topics: Humans; Cardiac Catheterization; Heart Septal Defects, Atrial; Prosthesis Design; Stents; Vena Cava, Superior
PubMed: 38839164
DOI: 10.1016/j.iccl.2024.03.007 -
Echocardiography (Mount Kisco, N.Y.) Jun 2024Loeys-Dietz syndrome (LDS) is an autosomal-dominant connective tissue disorder associated with mutations in the transforming growth factor β receptor. It is...
Loeys-Dietz syndrome (LDS) is an autosomal-dominant connective tissue disorder associated with mutations in the transforming growth factor β receptor. It is characterized by distinctive craniofacial changes, skeletal features, and cardiovascular complications. We present a case of a 24-year-old male with development delay and a one-year history of progressively worsening dyspnea on moderate exertion and orthopnea. Echocardiography revealed right atrial and right ventricle dilation, right ventricle hypertrophy, atrial septal defect, and aneurysmal dilation of the pulmonary artery trunk. This case underscores the importance of early detection and comprehensive imaging in patients suspected of having LDS, particularly considering the potential for atypical vascular manifestations.
Topics: Humans; Male; Loeys-Dietz Syndrome; Heart Septal Defects, Atrial; Pulmonary Artery; Young Adult; Delayed Diagnosis; Echocardiography; Dilatation, Pathologic; Diagnosis, Differential
PubMed: 38837500
DOI: 10.1111/echo.15851 -
Pediatric Cardiology Jun 2024In patients born with anorectal malformations (ARM), additional congenital heart defects (CHD) can occur. We aimed to provide an overview on disease and treatment...
In patients born with anorectal malformations (ARM), additional congenital heart defects (CHD) can occur. We aimed to provide an overview on disease and treatment details of CHD identified in patients born with ARM, from a unique large cohort of a very rare disease. We performed a retrospective single-center cohort study between January 2000 and July 2023. All consecutive patients with ARM were included. Outcomes were the number of patients with CHD, and screening percentage and percentage of patients diagnosed with CHD over 3 time periods (2000-2006, 2007-2014, 2015-2023). We used uni- and multi-variable logistic regression analyses to search for associations between CHD present and baseline characteristics. In total, 281 patients were included. Some 241 (85.8%) underwent echocardiography, of whom 80 (33.2%) had CHD. Screening percentage with echocardiography increased (74.1% vs. 85.7% vs. 95.9%, p < 0.001) and percentage of patients diagnosed with CHD remained similar over time (30.2% vs. 34.5% vs. 34.0%, p = 0.836). Atrial and ventricular septal defects (n = 36, n = 29), and persistent left superior vena cava (n = 17) were most identified. The presence of VACTERL-association or a genetic syndrome was independently associated with the presence of CHD. CHD were present in 33% of patients with ARM that underwent echocardiography. Over time, the number of CHD identified through screening remained similar. Patients with the presence of VACTERL-association or a genetic syndrome had a higher risk of having CHD. Therefore, acknowledging the potential presence of CHD in patients with ARM remains important.
PubMed: 38836880
DOI: 10.1007/s00246-024-03536-3 -
JTCVS Techniques Apr 2024In select patients with borderline ventricular hypoplasia, we adopted a strategy of initial single-ventricle palliation followed by staged or direct biventricular...
OBJECTIVE
In select patients with borderline ventricular hypoplasia, we adopted a strategy of initial single-ventricle palliation followed by staged or direct biventricular conversion by 2 years of age.
METHODS
Between 2018 and 2023, 14 newborns with borderline hypoplastic heart disease deemed high risk for primary biventricular repair underwent palliative procedures as a neonate/infant, followed by staged or direct biventricular conversion.
RESULTS
Of the 14 patients, 6 had borderline left ventricles and 8 had borderline right ventricles. Index neonatal operations were performed in 12 patients and included the Norwood operation (n = 5), pulmonary artery band (n = 3), ductal stent (n = 3), and hybrid Norwood (n = 1). Five patients underwent direct biventricular conversion, and the remaining 9 patients underwent staged ventricular recruitment operations at a mean age of 6 months (range, 3-11 months). Ventricular recruitment operations included atrial septation with or without ventricular rehabilitation, atrioventricular valve repair, or outflow tract operations. At a mean duration of 8 months (range, 4-10 months) after ventricular recruitment, there was a significant increase in chamber volume, aortic valve, and mitral valve size in patients with borderline left ventricles, and a normalization of the right ventricle:left ventricle end-diastolic volume ratio in patients with borderline right ventricles. To date, 13 of 14 patients have undergone successful biventricular conversion at a mean age of 16 months (range, 4-31 months).
CONCLUSIONS
In select newborns with borderline hypoplastic heart disease, single-ventricle palliation followed by staged or direct biventricular conversion may increase infant survival while allowing for early attainment of a biventricular circulation.
PubMed: 38835569
DOI: 10.1016/j.xjtc.2024.02.006 -
Tidsskrift For Den Norske Laegeforening... Jun 2024While most cases of venous thromboembolism follow a benign course, occasionally the condition may manifest a complex clinical presentation and need a comprehensive...
BACKGROUND
While most cases of venous thromboembolism follow a benign course, occasionally the condition may manifest a complex clinical presentation and need a comprehensive diagnostic workup to identify the underlying cause and provide the patient with appropriate treatment.
CASE PRESENTATION
A woman in her late thirties presented to the emergency department with a five-day history of dyspnoea. She had recently undergone liposuction surgery after pregnancy. Upon admission, initial investigations revealed a pulmonary embolism with right heart strain, and she was treated with anticoagulants. The following day, she complained of acute-onset right flank pain without fever or other accompanying symptoms. A CT scan of the abdomen confirmed a right-side renal infarction. Further investigations revealed patent foramen ovale between the right and left atria of the heart, believed to be the source of a right-to-left shunt of arterial emboli. Although the patient had not suffered a clinical stroke, it was decided to close this defect using percutaneous technique.
INTERPRETATION
Patent foramen ovale is a common condition in adults, but in most cases it remains asymptomatic. However, patients with patent foramen ovale have an elevated risk of arterial emboli affecting multiple organs. The diagnosis depends on thorough assessment to prevent potentially fatal outcomes.
Topics: Humans; Female; Adult; Foramen Ovale, Patent; Dyspnea; Abdominoplasty; Pulmonary Embolism; Tomography, X-Ray Computed; Infarction; Postoperative Complications
PubMed: 38832610
DOI: 10.4045/tidsskr.23.0742 -
Frontiers in Cardiovascular Medicine 2024Patent Foramen Ovale (PFO) is a common congenital atrial septal defect present in 20%-35% of the general population. Although generally considered a benign anatomic... (Review)
Review
Patent Foramen Ovale (PFO) is a common congenital atrial septal defect present in 20%-35% of the general population. Although generally considered a benign anatomic variant, a PFO may facilitate passage of a thrombus from the venous to arterial circulation, thereby resulting in cryptogenic stroke or systemic embolization. A PFO is detected in nearly one half of patients presenting with cryptogenic stroke and often considered the most likely etiology when other causes have been excluded. In this review, we discuss the contemporary role of transcatheter closure of PFO in the treatment of cryptogenic stroke, including devices currently available for commercial use in the United States (Amplatzer PFO Occluder and Gore Cardioform Septal Occluder) and a novel suture-mediated device (NobleStitch EL) under clinical investigation. To provide the best care for cryptogenic stroke patients, practitioners should be familiar with the indications for PFO closure and corresponding treatment options.
PubMed: 38832314
DOI: 10.3389/fcvm.2024.1391886 -
The Application of Clinical Genetics 2024Optical Genome Mapping (OGM) technology has garnered growing interest for the identification of chromosomal structural variations (SVs), particularly complex ones that...
Optical Genome Mapping Identifies a Novel Unbalanced Translocation Between Chromosomes 4q and 6q Leading to Feeding Difficulties and Hypotonia in a Neonate: A Case Report.
Optical Genome Mapping (OGM) technology has garnered growing interest for the identification of chromosomal structural variations (SVs), particularly complex ones that are implicated in genetic diseases in humans. In this study, we performed genetic diagnostics on a neonatal patient who presented with feeding difficulties, hypotonia, and an atrial septal defect. We utilized a combination of trio-whole exome sequencing and OGM for our analysis. The results revealed an unbalanced translocation between maternal chromosomes 4 and 6 in the proband, ogm[GRch38]t(4:6)(q35.2;q25.3), resulting in a 2.8 Mb deletion at the 4q35 terminal and a 10.2 Mb duplication at the 6q25 terminal. In summary, this study highlights how OGM, in conjunction with other genetic approaches, can unveil the genetic etiology of complex clinical syndromes. Neonatal patients often exhibit low specific phenotypes, underlining the significance of SV detection.
PubMed: 38828444
DOI: 10.2147/TACG.S465244 -
Indian Journal of Thoracic and... May 2024is a well-known cause of blood culture-negative endocarditis; however, pulmonary valve involvement is rare. The case of a 40-year-old African male who presented to the...
UNLABELLED
is a well-known cause of blood culture-negative endocarditis; however, pulmonary valve involvement is rare. The case of a 40-year-old African male who presented to the Emergency Department with chest pain, cardiac failure, and a 2-week history of fever is presented. Transoesophageal echocardiography confirmed an atrial septal defect, severe pulmonary insufficiency with large vegetations, severe mitral regurgitation due to anterior leaflet prolapse, and right ventricular dysfunction. Empirical antibiotic therapy was started, and urgent surgical intervention was decided. There were vegetations on the three pulmonary valve leaflets and the mitral valve. Closure of the atrial septal defect, mitral and tricuspid valve repair, pulmonary valve replacement with a biological prosthesis, and infundibuloplasty of the right ventricle were performed. The postoperative course was uneventful. Preoperative blood cultures were negative, and was detected through 16S rRNA gene amplification and sequencing in mitral and pulmonary implants. Serology showed positive titers of 1/1260 for both and . Ceftriaxone and gentamicin were administered for 10 days, followed by oral doxycycline for 12 weeks. A one-year echocardiogram showed normal functioning of the pulmonary prosthesis and the mitral and tricuspid repair. Infection caused by is a rare cause of endocarditis with negative blood cultures, and multivalvular and pulmonary valve involvement is exceptional.
SUPPLEMENTARY INFORMATION
The online version contains supplementary material available at 10.1007/s12055-024-01727-4.
PubMed: 38827541
DOI: 10.1007/s12055-024-01727-4 -
Journal of Cardiology Cases Jun 2024Atrial tachyarrhythmias occurring after transcatheter atrial septal defect closure are not uncommon; however, those related to device stimulation are rare. Herein, a...
Refractory atrial tachycardia after transcatheter closure of an atrial septal defect, successfully treated by catheter ablation with transseptal approach via the side of the device.
UNLABELLED
Atrial tachyarrhythmias occurring after transcatheter atrial septal defect closure are not uncommon; however, those related to device stimulation are rare. Herein, a case involving a 24-year-old female, who developed drug-refractory atrial tachycardia during the early postoperative period, is reported. The results of electroanatomical mapping could eventually be obtained from both atria. They revealed a focal pattern, and the earliest site was located on the left atrial side of the superior atrial septum, between the left and right atrial discs of the device. An ablation catheter was inserted through the side of the device into the left atrial septum, and cauterization successfully achieved recovery of the sinus rhythm without device dislodgement.
LEARNING OBJECTIVE
Atrial tachyarrhythmia related to mechanical stimulation with an atrial septal defect closure device in the early perioperative period is a rare complication. Catheter ablation with transseptal approach through the side of the device might be an option, although careful attention should be paid to the risk for device dislodgement.
PubMed: 38826763
DOI: 10.1016/j.jccase.2024.02.007 -
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: Adult; Aged; Female; Humans; Male; Middle Aged; Minimally Invasive Surgical Procedures; Palliative Care; Quality of Life; Retrospective Studies; Septal Occluder Device; Tracheoesophageal Fistula; Treatment Outcome
PubMed: 38822914
DOI: 10.1007/s00423-024-03363-3