UNASSIGNED: Delivery systems are crucially important for the implantation of medical devices in patients with congenital heart disease. However, very little data is available comparing the advantages and disadvantages of the various delivery systems.
UNASSIGNED: This article describes the delivery systems and methods used for delivery of atrial septal occluder devices, ventricular septal occluder devices, devices to occlude patent arterial ducts, and transcatheter pulmonary valves. Delivery systems are compared relating to prepping and loading, positioning of the delivery sheath/catheter, deployment, ability to recapture and reposition, as well as device release.
UNASSIGNED: For most ASD/VSD/PDA occluder devices, the basic delivery mechanism has changed very little over the preceding 20 years. Future modifications may focus on meaningful modifications to the cable systems that reduce stiffness and improve angulation at the connection to the device. Over the next 5-10 years, it is expected to see significant changes to delivery systems used for transcatheter pulmonary valve implantation, that result in improvements in the ability to recapture and reposition self-expandable transcatheter valves during the deployment process, combined with kink resistant sheaths that facilitate easy tracking across often complex right ventricular outflow tracts.

Leadless pacemakers have demonstrated potential as a transvenous pacing option in Adult Congenital Heart Disease patients. Aveir™ single-chamber (VR) leadless pacemakers have demonstrated safety in patients without congenital heart disease in a dual chamber approach. We present a case of dual-chamber pacing using the Aveir dual-chamber (DR) leadless pacemaker in a patient with repaired dextro-transposition of the great arteries with ventricular septal defect (VSD) surgical closure. A 26-year-old male patient with a history of transposition of the great arteries status post arterial switch and VSD repair neonatally had complicated second degree atrioventricular block and sinus node dysfunction necessitating pacemaker placement. Epicardial single-chamber ventricular pacemaker was placed neonatally, which was switched to dual-chamber pacemaker at age 17 due to malfunction. Recent fracture of pacemaker leads led to implantation of new dual chamber leadless pacemaker. Removal of previous pacemaker leads via mechanical extraction occurred and implantation of Aveir DR leadless pacemaker was performed under anesthesia via right femoral vein access without complication. Follow-up demonstrated Aveir VR threshold of 1.0V@0.2ms, R-wave of 8.9mV, impedance of 490Ω, and the Aveir AR threshold of 0.75V@0.2ms, P-wave of 3.7mV, and impedance of 400Ω. This case demonstrates safety and efficacy of dual chamber leadless pacemaker implantation in an ACHD patient.

Aim: The Amplatzer Piccolo Occluder (APO) is approved for patent ductus arteriosus (PDA) occlusion in infants weighing >700 g but could offer versatility to treat other lesions.Methods: Retrospective review of children in whom APO was utilized for defects other than PDA between January 2022 and June 2023.Results: The APO was used in nine patients; three for ventricular septal defects, four with coronary fistulas, one for a ventricular pseudoaneurysm and one where APO deployed within a fenestration of a previously placed Amplatzer Septal Occluder. All nine patients had successful occlusions without complications.Conclusion: The APO is a versatile device that can be used to treat various small diameter lesions in children besides the PDA for which it is currently approved for.
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In patients with post-myocardial infarction ventricular septal defects, temporary left ventricular support using Impella 5.5 can decrease shunting, facilitate peri-infarct tissue remodeling, and allow for assessment of myocardial recovery prior to repair. When there is inadequate cardiac recovery, implantation of a durable left ventricular assist device such as HeartMate 3 at time of repair can be safely performed. A right ventriculotomy provides multiple advantages when performing VSD repair and concomitant HeartMate 3 placement.

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BACKGROUND: Congenital heart disease (CHD) encompasses morphofunctional anomalies in the heart and circulatory system present at birth, which may not become apparent until later in life. In Ethiopia, there needs to be more understanding of the prevalence, patterns, and associated complications of CHD malformations. This study aimed to investigate the patterns and complications of CHDs among patients receiving follow-up care at a specialized university referral hospital in Ethiopia.
METHODS: A hospital-based cross-sectional study was conducted on 199 patients with CHDs to assess the patterns and complications of defects. Retrospective data were collected from 16,972 patients who had follow-ups at a cardiac clinic in 2021 using medical records, and a statistical analysis was performed with SPSS version 24.
RESULTS: The most prevalent types of CHDs in our study population were atrial septal defects (ASDs) at 41.2% (82 cases), ventricular septal defects (VSDs) at 26.6% (53 cases), and patent ductus arteriosus (PDAs) at 9.5% (19 cases). Complications related to CHDs were observed in 69.3% (138) of patients, with 30.7% (61) experiencing a single complication and 39.2% (87) experiencing multiple complications.
CONCLUSIONS: This study found a higher prevalence of CHDs in females (77.8%) compared to males, a trend consistent across various atrial and ventricular defect types. Individuals aged 15 to 25 years exhibited the highest incidence of ASD and VSD. Moreover, CHD-related anomalies were present in 69.3% of the patients studied.

BACKGROUND: Cleft in the mitral valve leaflet is a primary cause of congenital mitral regurgitation, stemming from developmental anomalies in the mitral valve and frequently associated with other congenital heart defects. Concurrent presence of cleft in mitral valve leaflet with atrial septal defect and ventricular septal defect is relatively rare. Echocardiography, especially transesophageal echocardiography, is essential in diagnosing cleft mitral valve leaflet and related congenital heart defects, providing critical, detailed imagery for accurate assessment. This study presents a young female patient whose anterior mitral cleft, along with atrial septal defect and ventricular septal defect, was revealed through three-dimensional transesophageal echocardiography.
METHODS: A 25-year-old Iranian female, experiencing progressive dyspnea and diminished physical capacity over 3 months, was referred to our hospital. Initial examination and transthoracic echocardiography indicated severe mitral regurgitation. Further evaluation with transesophageal echocardiography corroborated these findings and identified a cleft in the anterior mitral valve leaflet, coupled with mild left ventricular enlargement and significant left atrial enlargement. The complexity of the patient\'s condition was heightened by the diagnosis of cleft mitral valve leaflet in conjunction with atrial septal defect and ventricular septal defect, showing the complex nature of congenital defects.
CONCLUSIONS: This case emphasizes the critical role of transthoracic echocardiography in diagnosing cleft of mitral valve leaflet and associated cardiac anomalies, showcasing its superiority over transthoracic echocardiography for detailed visualization of cardiac structures. The identification of multiple congenital defects highlights the necessity for a comprehensive diagnostic approach to manage and treat patients with complex congenital heart diseases effectively. Future research should aim to refine diagnostic methodologies to enhance patient outcomes for cleft of mitral valve leaflets and related congenital conditions.

BACKGROUND: Chromosome 7 has regions enriched with low copy repeats (LCRs), which increase the likelihood of chromosomal microdeletion disorders. Documented microdeletion disorders on chromosome 7 include both well-known Williams syndrome and more rare cases. It is noteworthy that most cases of various microdeletions are characterized by phenotypic signs of neuropsychological developmental disorders, which, however, have a different genetic origin. The localization of the microdeletions, the genes included in the region, as well as the structural features of the sequences of these genes have a cumulative influence on the phenotypic characteristics of the individuals for each specific case and the severity of the manifestations of disorders. The consideration of these features and their detailed analysis is important for a correct and comprehensive assessment of the disease.
RESULTS: The article describes a clinical case of 7p22.3 microdeletion in a patient with congenital heart defect and neurological abnormalities - epilepsy, combined with moderate mental and motor developmental delay.
CONCLUSIONS: Through detailed genetic analyses, we are improving the clinical description of the rare 7p22.3 microdeletion and thus creating a basis for future genetic counseling and research into targeted therapies.

Among congenital cyanotic heart diseases (CHDs), situs inversus totalis with transposition of great vessels with a large ventricular septal defect (VSD) has a very low incidence of around 1 in 10,000. Hereby, we present a 16-year-old man with the aforementioned cardiac anomaly with cardiac arrhythmias, septic shock, and a history of road traffic accident-causing osteomyelitis of the left thigh requiring incision and drainage. The patient was admitted to the intensive care unit with a high-grade fever, narrow pulse pressure, and atrial fibrillation. The patient was operated on under general anesthesia with endotracheal intubation after optimization. Invasive monitoring, antiarrhythmics, and vasopressors were required intraoperatively, and surgery progressed uneventfully. Furthermore, the patient had undergone a series of debridements after 8 days, which were performed under regional anesthesia uneventfully. This case report represents a plan of action for perioperative anesthetic management and anticipates the difficulties for CHD patients in the course of surgery and subsequential prudence.

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