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Concept: Atrial septal defect

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Paradoxical embolism (PDE) occurs after embolic material passes from the venous to the arterial circulation through a right-to-left shunt, which is frequently a patent foramen ovale (PFO). We describe the case of a patient with deep venous thrombosis and an intracardiac thrombus straddling a PFO and who was successfully treated with an emergency surgery.

Concepts: Blood vessel, Stroke, Pulmonary embolism, Hematology, Vein, Atrial septal defect, Foramen ovale, Embolism

161

Several complications have been reported regarding the percutaneous closure of secondary atrial septal defects such as erosion, thromboembolic events, arrhythmias, and endocarditis. In this report, we describe the case of a 75-year-old woman who underwent percutaneous closure of a secondary atrial septal defect with a 12 mm Amplatzer septal occluder. Six months after the uneventful implantation of the device, we manifested an asymptomatic late embolization of the device in the abdominal aorta. The device was surgically retrieved.

Concepts: Surgery, Report, Abdominal aorta, Crystallographic defect, Ventricular septal defect, Atrial septal defect, Levo-Transposition of the great arteries, Cardiac shunt

146

A patent foramen ovale (PFO) is common and found in nearly 25% of healthy individuals. The majority of patients with PFO remain asymptomatic and they are not at increased risk for developing a stroke. The presence of PFO, however, has been found to be higher in patients with cryptogenic stroke, suggesting there may be a subset of patients with PFO who are indeed at risk for stroke. Paradoxical embolization of venous thrombi through the PFO, which then enter the arterial circulation, is hypothesized to account for this relationship. Although aerated-saline transesophageal echocardiography is the gold standard for diagnosis, aerated-saline transthoracic echocardiography and transcranial Doppler are often used as the initial diagnostic tests for detecting PFO. Patients with cryptogenic stroke and PFO are generally treated with antiplatelet therapy in the absence of another condition for which anticoagulation is necessary. Based on the findings of 3 large randomized clinical trials, current consensus guidelines do not recommend percutaneous closure, though this is an area of controversy. The following review discusses the relationship of PFO and cryptogenic stroke, focusing on the epidemiology, pathophysiological mechanisms, diagnostic tools, associated clinical/anatomic factors and treatment.

Concepts: Epidemiology, Doppler echocardiography, Echocardiography, Contrast-enhanced ultrasound, Atrial septal defect, Foramen ovale, Ostium primum, Cardiac shunt

28

Background: Atrial fibrosis or fatty deposition is known to increase the propensity for the development of atrial fibrillation (AF). Apart from the pulmonic veins, the interatrial septum (IAS) might play a role in the maintenance of AF. In contrast to left atrial anatomy and adjacent veins, the IAS cannot be visualized in detail with computed tomography. Thus, preprocedural transesophageal echocardiography (TEE) may provide important morphologic information beyond exclusion from atrial thrombi. Methods: The study comprised 108 consecutive patients (mean age 60 ± 11 years; 98 men). AF was paroxysmal in 91 (84%) and persistent in 17 (16%) patients. We investigated the morphological characteristics of the IAS by TEE in patients who underwent radiofrequency ablation of AF. Results: The IAS was structurally abnormal in 46 (43%) patients, showing the following echocardiograhic findings: atrial septal hypermobility or aneurysm (n = 27) associated with a patent foramen ovale (PFO) (n = 11) or with a small atrial septal defect (ASD) (n = 2), a septal flap associated with a PFO or an ASD (n = 8), and an abnormally thickened IAS (n = 12). A thrombus in the left atrial appendage was discovered in only 2 (2%) patients. Conclusions: A structurally abnormal IAS was diagnosed in nearly half of the patients undergoing ablation therapy for AF. The information obtained by TEE is mandatory to exclude left atrial thrombi prior the ablation procedure. Moreover, detailed knowledge of morphologic characteristics of the IAS facilitates an optimized and safe performance of the transseptal puncture using long sheaths with large diameters.

Concepts: Cardiology, Atrial fibrillation, Ventricular septal defect, Atrial septal defect, Foramen ovale, Eisenmenger's syndrome, Interatrial septum, Left atrium

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The patent foramen ovale (PFO) is a normal interatrial communication during fetal life that persists after birth in approximately 1 of every 4 adults. PFO is a potential route for embolic transit from the systemic venous circulation to the brain. Though there is compelling circumstantial evidence implicating PFO, the precise role of PFO in the pathogenesis of cryptogenic stroke is not yet established. Several randomized trials of transcatheter PFO closure versus medical management are ongoing. Results of these trials may improve our ability to select the best treatment for individual patients. Further well-designed studies are necessary to address several unresolved issues related to PFO stroke and PFO migraine pathophysiology, and to identify the patients who would most likely benefit from PFO closure. The purpose of this review is to summarize contemporary understanding, discuss current treatments, and explore some of the knowledge gaps pertaining to the clinical significance of PFO.

Concepts: Systemic circulation, Atrial septal defect, Foramen ovale, Ostium primum, Evidence, Interatrial septum

27

Background. Device closure of atrial septal defect (ASD) and patent foramen ovale (PFO) are both associated with short- and long-term complications. Our knowledge of the complication rates of ASD and PFO closure is limited. Our objective was to review the peri-procedural and long-term complications of ASD and PFO closure. Methods. Medline, EMBASE, and Scopus databases were searched between 1973 and 2012. A total of 28142 patients from 203 case series were included. Of these 203 articles, 111 were reporting ASD closure, 61 were reporting PFO closure, and 31 were reporting both. Pooled incidence rates of cardiac complications were calculated separately for peri-procedural and at follow-up. Results. Peri-procedural major complications were reported from 0% to 9.4%, with a pooled estimate rate of 1.4% (95% CI: 1.3% to 1.6%). It was 1.6% (95% CI, 1.4-1.8) in ASD group, 1.1% (95% CI, 0.9-1.3) in PFO group, and 1.3% (95% CI, 0.9-1.9) in ASD/PFO group. The most common major complication was the device embolization requiring surgery. Peri-procedural minor complications were reported with a pooled estimate rate of 1.4% (95% CI, 1.2-1.7). It was 1.6% (95% CI, 1.2-2.1) in ASD group, 1.3% (95% CI, 1.0-1.7) in PFO group, and 1.5% (95% CI, 1.1-1.2.1) in ASD/PFO group. The most frequent major complications at follow-up were cerebrovascular events (1.3% (95% CI: 1.1% to 1.6%)) and device thrombosis (1.2% (95% CI: 1.0% to 1.4%)). Both were more frequent in PFO group. Conclusion. Device closure of ASD and PFO are associated with non-negligible serious complications, both in early and long-term. © 2013 Wiley Periodicals, Inc.

Concepts: Ventricular septal defect, Atrial septal defect, Foramen ovale, Ostium primum

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This study concerns the morphological differentiation between double outlet right ventricle (DORV) and aortic dextroposition (AD) defects, namely tetralogy of Fallot and Eisenmenger anomaly. Indeed, despite the similar condition in terms of sequential ventriculo-arterial connections, DORV and AD are two distinct morphological entities. It is proposed that the borderline between these two groups of malformations is represented by the specific insertion of the infundibular septum into the left anterior cranial division of the septomarginal trabeculation (or septal band) occurring in ADs and lacking in DORV. Furthermore, the spiraliform versus straight parallel arrangement of the great arteries in the two groups of anomalies is emphasized as an additional and distinctive morphological feature. Emphasis is also given to the association of straight parallel great arteries conotruncal malformations, DORV and transposition of the great arteries, with the asplenia type of heterotaxy laterality defects. Within this context, the absence of subaortic ventricular septal defect and concomitantly of spiraliform great arteries in the asplenia group of heterotaxy anomalies, as detected by this study, further substantiates our belief of not mixing collectively the ADs with the DORV in clinico-pathological diagnosis. Anat Rec, 00:000-000, 2013. © 2013 Wiley Periodicals, Inc.

Concepts: Heart, Left ventricle, Congenital heart disease, Tetralogy of Fallot, Ventricular septal defect, Atrial septal defect, Transposition of the great vessels, Double outlet right ventricle

27

A parturient required emergency admission to intensive care following a major postpartum haemorrhage. She was hypovolaemic, hypoxic and cyanosed and was also noted to have digital clubbing. A transthoracic echo showed a large atrial septal defect (ASD) with right heart dilation and possible anomalous pulmonary venous drainage. Eisenmenger syndrome was diagnosed with cyanosis due to shunt reversal at atrial level (right atrium to left), as a consequence of an elevated pulmonary vascular resistance from chronically increased pulmonary blood flow. After a full initial recovery, she was referred to a specialist grown-up congenital heart disease unit for further investigation. Cardiac MRI confirmed an ASD but showed that the cause of the cyanosis was anatomical in origin. An elongated Eustachian valve was diverting desaturated inferior venacaval blood across the intra-atrial septum into the left atrium. Her congenital heart defect was therefore amenable to corrective surgery.

Concepts: Blood, Heart, Down syndrome, Congenital heart defect, Congenital heart disease, Tetralogy of Fallot, Total anomalous pulmonary venous connection, Atrial septal defect

26

Iatrogenic erosion of the septum primum after foramen ovale closure is an anecdotal event. We report the case of a 39-year-old woman admitted to our institution for multifocal cryptogenic cerebral ischemia and a patent foramen ovale (PFO) associated with an aneurysm of the septum primum. The patient underwent percutaneous closure of the PFO with an Amplatzer PFO Occluder device. At the 6-months follow up, the device was in the right position, but a jagged defect of the septum primum and evidence of significant left-to-right shunting was detected. The atrial septal defect was then repaired by a surgical approach. Although this event is not life-threatening, it should be considered as a therapeutic pitfall, resulting in a risk of paradoxical embolism recurrences and long-term hemodynamic impairment. © 2013 Wiley Periodicals, Inc.

Concepts: Ventricular septal defect, Atrial septal defect, Foramen ovale, Ostium primum, Cardiac shunt

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Percutaneous device closure of atrial septal defect (ASD) is now considered the choice of treatment. Numerous devices with advantages/disadvantages are currently available and under development. Cardia Ultrasept II ASD occluder has a nitinol frame covered with polyvinyl alcohol (PVA) membrane. Here, a well-documented case of early malfunction of PVA membrane detected in the first week of implantation in a 4-year-old male patient, who underwent an uneventful device closure with 20 mm Cardia Ultrasept II ASD Occluder, is presented. One week after implantation left-to-right shunt through the device was detected and the explantation of device revealed PVA membrane with multiple perforations. © 2015 Wiley Periodicals, Inc.

Concepts: Device, Ventricular septal defect, Atrial septal defect, Polyvinyl alcohol, Polyvinyl acetate, Cardiac shunt