Atrial septal defects

Abstract: Objectives This study sought to compare the safety, efficacy and clinical utility of the Amplatzer septal occluder (ASO) for closure of secundum atrial septal defect (ASD) with surgical closure. Background The clinical utility of a device such as the ASO can only be judged against the results of contemporaneous surgery. Methods A multicenter, nonrandomized concurrent study was performed in 29 pediatric cardiology centers from March 1998 to March 2000. The patients were assigned to either the device or surgical closure group according to the patients’ option. Baseline physical exams and echocardiography were performed preprocedure and at follow-up (6 and 12 months for device group, 12 months for surgical group). Results A total of 442 patients were in the group undergoing device closure, whereas 154 patients were in the surgical group. The median age was 9.8 years for the device group and 4.1 years for the surgical group (p 0.05). The complication rate was 7.2% for the device group and 24.0% for the surgical group (p Conclusions The early, primary and secondary efficacy success rates for surgical versus. device closure of ASD were not statistically different; however, the complication rate was lower and the length of hospital stay was shorter for device closure than for surgical repair. Appropriate patient selection is an important factor for successful device closure. Transcatheter closure of secundum ASD using the ASO is a safe and effective alternative to surgical repair.

Abstract: Background. Atrial septal defects have been surgically correctable for more than 30 years. The long-term survival rates among patients treated in the early era of cardiac surgery are poorly documented, but such data are of critical importance to the future medical care, employability, and insurability of these patients. Methods. To determine the natural history of surgically corrected atrial septal defects, we studied all 123 patients who underwent repair of an isolated defect (ostium secundum or sinus venosus) at the Mayo Clinic between 1956 and 1960, 27 to 32 years after the procedure. The follow-up status of all patients was determined by written questionnaires and telephone interviews. Hospital records and death certificates were obtained if interim hospitalization or death had occurred. Results. The overall 30-year actuarial survival rate among survivors of the perioperative period was 74 percent, as compared with 85 percent among controls matched for age and sex. The perioperative mortality...

Abstract: Heterozygous mutations in NKX2.5, a homeobox transcription factor, were reported to cause secundum atrial septal defects and result in atrioventricular (AV) conduction block during postnatal life. To further characterize the role of NKX2.5 in cardiac morphogenesis, we sought additional mutations in groups of probands with cardiac anomalies and first-degree AV block, idiopathic AV block, or tetralogy of Fallot. We identified 7 novel mutations by sequence analysis of the NKX2.5-coding region in 26 individuals. Associated phenotypes included AV block, which was the primary manifestation of cardiac disease in nearly a quarter of affected individuals, as well as atrial septal defect and ventricular septal defect. Ventricular septal defect was associated with tetralogy of Fallot or double-outlet right ventricle in 3 individuals. Ebstein’s anomaly and other tricuspid valve abnormalities were also present. Mutations in human NKX2.5 cause a variety of cardiac anomalies and may account for a clinically significant portion of tetralogy of Fallot and idiopathic AV block. The coinheritance of NKX2.5 mutations with various congenital heart defects suggests that this transcription factor contributes to diverse cardiac developmental pathways. J. Clin. Invest. 104:1567–1573 (1999).

Abstract: The objectives of this study were to identify possible risk factors that may lead to erosion of the Amplatzer septal occluder (ASO) and recommend ways to minimize future risk. There have been rare occurrences of adverse events with development of pericardial effusion after ASO placement. Identification of high-risk cases, early recognition, and prompt intervention may minimize the future risks of adverse events. In all patients who developed hemodynamic compromise after ASO placement, echocardiograms (pre-, intra-, and postprocedure), atrial septal defect (ASD) size (nonstretched, stretched), size of the device used, cineangiograms, and operative records were reviewed by a panel selected by AGA Medical Corporation. The findings were compared to the premarket approval data obtained from FDA-approved clinical trials that were conducted in the United States, before the device was approved. A total of 28 cases (14 in United States) of adverse events were reported to AGA Medical. All erosions occurred at the dome of the atria, near the aortic root. Deficient aortic rim was seen in 89% and the defect described as high ASD, suggesting deficient superior rim. The device to unstretched ASD ratio was significantly larger in the adverse event group when compared to the FDA trial group. The incidence of device erosion in the United States was 0.1%. The risk of device erosion with ASO is low and complications can be decreased by identifying high-risk patients and following them closely. Patients with deficient aortic rim and/or superior rim may be at higher risk for device erosion. Oversized ASO may increase the risk of erosion. The defect should not be overstretched during balloon sizing. Patients with small pericardial effusion at 24 hr should have closer follow-up.

Abstract: Patients with isolated atrial septal defects (ASDs) have benefited from important recent advances in the diagnosis, evaluation, and management of their conditions This review will focus on adolescent and adult patients with sizeable ASDs who do not have other major associated cardiac defects There are 3 major types of ASDs or interatrial communications: ostium secundum, ostium primum, and sinus venosus (Figure 1A) defects The ostium secundum is a true defect of the atrial septum and involves the region of the fossa ovalis The ostium primum defect is within the spectrum of the atrioventricular (AV) septal defects (Figure 1B; also known as AV canal defects or endocardial cushion defects), the complete form of which also includes a large ventricular septal defect and a common AV valve The sinus venosus defect is usually located at the junction of the right atrium and superior vena cava and is almost always associated with partial anomalous pulmonary venous return Two very uncommon types of ASDs may be mentioned briefly: the inferior vena cava form of the sinus venosus defect and the coronary sinus septal defect (in which a defect between the coronary sinus and the left atrium allows a left-to-right shunt to occur through an “unroofed” coronary sinus) Figure 1 A, A indicates superior sinus venosus ASD; B, secundum ASD; C, inferior sinus venosus ASD; D, ostium primum ASD or partial AV septal defect; E, secundum ASD without posterior septal rim; F, coronary sinus ASD; and IVC, inferior vena cava B, AV junction viewed from the atrial aspect Normal indicates normal heart; complete AVSD, complete AV septal defect with a common AV junction and a common, single AV valve, guarded by 5 leaflets (counterclockwise: superior bridging, left mural, inferior bridging, right inferior, and right anterosuperior); partial AVSD, partial AVSD or primum ASD with …