Is Moderate/Large Residual Shunt After PFO Closure Justifiable for a Patient with a Prior History of Cryptogenic Stroke and Transient Ischemic Attack?

A 36-year-old woman suffered from an embolic stroke of an undetermined source documented by magnetic resonance imaging with residual right arm weakness. She underwent percutaneous patent foramen ovale (PFO) closure with an 18/25 mm device in another center. One year later, the patient suffered from a transient ischemic attack with dysarthria. She asked for a second opinion at our institution and a contrast-transthoracic (cTTE) /transesophageal echocardiography showed a large residual right-to-left shunt (RLS) through a still patent tunnel after PFO closure. Written informed consent for a redo procedure was obtained from the patient. A catheter-based closure of the residual shunt was therefore planned under local anesthesia and rotational intracardiac echo monitoring. A second equally sized disc (18/18 mm) device was successfully implanted without complications. The patient was discharged home the following day in good clinical condition. Dual antiplatelet therapy was recommended for the first 2 months and then single antiplatelet therapy up to 6 months. At the 6-month follow-up, the cTTE color Doppler showed the stable position of the two nitinol double-disc devices and the c-transcranial Doppler confirmed the abolition of the residual RLS.

Transcatheter closure of fenestrated atrial septal aneurysm in children: Feasibility and long-term results.

BACKGROUND: To date, extensive experience in transcatheter closure of fenestrated atrial septal aneurysm (ASA) in the pediatric population is limited. METHODS: To report on procedural feasibility, efficacy, and long-term outcome, we enrolled all children submitted to an attempt of transcatheter closure of fenestrated ASA at two, large volume, pediatric cardiology units (Naples and Massa, Italy) between April 2000 to May 2020. RESULTS: This retrospective study included 139 patients (median age 9 years [range 2-18] and weight 36 kg [range 10-102]); 19 (13.7%) children were ≤20 kg (range 10-20) and 14 (10.1%) were ≤5 years old. Single perforation was observed in 28 patients (20.1%), while 111 patients (79.9%) had multifenestrated ASA. The median size of the main defect was 15 mm (range 6-34) and 25 patients (18%) had a defect ≥20 mm. The procedural success rate was 99% (95% confidence interval [CI]: 94.9-99.8) using a single device in 75 (69%), two devices in 31 (28%), and three devices in 3 (3%) cases. Early minor adverse events (AEs) occurred in four patients (2.8%). Late minor AEs were recorded in one patient (0.7%) over a median follow-up of 5 years ([range 0-18 years; total 890.2 person-years, and with 30 patients (22%) followed ≥10 years). Neither mortality nor major AEs were recorded. Freedom from AEs was 99.1% at 10-15 years (95% CI: 93.5-99.8%), without any difference according to atrial septum anatomy or patient age and weight. CONCLUSION: Transcatheter closure of fenestrated ASA is technically feasible and effective in children with excellent long-term outcomes.

The Many Faces of the Interatrial Septum: A Diagnostic Dilemma and Considerations for Defect Closure Device Selection.

PATENT FORAMEN ovales (PFOs) and atrial septal defects (ASDs) are 2 examples of interatrial septal pathology.1 The presence of a PFO is a well-known risk factor for cryptogenic stroke.1,2 Newer evidence over the course of the last decade suggests percutaneous device closure of PFOs significantly reduces the subsequent risk of recurrent stroke.2 Among ASDs, the ostium secundum type is the most common pathology and, due to its anatomy, is most amenable to transcatheter closure.1 The tools that are available to percutaneously close these different pathologies vary, and choosing the correct device for the procedure can have significant impact on the clinical outcome. The authors here present a case that highlights how the differentiation of an ASD from a PFO using 2-dimensional (2D) and 3-dimensional (3D) echocardiography can affect the clinical decision-making and outcome in a challenging structural heart disease case.

Atrial Septal Aneurysms - A Clinically Relevant Enigma?

Atrial septal aneurysms (ASAs) are often seen during routine cardiac imaging, though their clinical relevance has been poorly defined. The aneurysmal, and often mobile, inter-atrial septum is frequently associated with other clinically relevant structural cardiac abnormalities, particularly patent foramen ovale (PFO). Whilst ASAs have previously been considered an incidental finding, a well-endowed atrial septum provides more than visual interest, including insights into atrial function and intra-atrial pressures, and has important clinical implications in PFO-associated stroke, migraines, and arrhythmias. This review outlines diagnostic challenges when assessing ASAs using all imaging modalities and the clinical implications of this common anatomical variant.

Association between atrial septal aneurysm and arrhythmias.

Objective. This study aimed to assess the association of atrial septal aneurysm (ASA) with cardiac arrhythmias by comparing patients with ASA with a control group with non-ASA, matched for age and gender. Methods. 641 patients with ASA who fulfilled the inclusion criteria were enrolled into the study. The control group consisted of 641 patients without ASA. Patients underwent physical, electrocardiographic and transthoracic echocardiographic examinations. Additional examinations such as transesophageal echocardiography, 24-h rhythm Holter monitoring, and electrophysiological study were performed when clinically needed. Results. There were no differences between the groups in respect to baseline demographic, clinical parameters and echocardiographic parameters except ischemic stroke and smoking status. Percentages of patients suffering from atrial premature complex (APC), ventricular premature complex (VPC), supraventricular tachycardia (SVT) and paroxysmal atrial fibrillation (AF) were higher in ASA patients compared to non-ASA patients. In addition, these parameters were independently associated with the presence of ASA in logistic regression analysis. Conclusions. Certain types of arrhythmias such as APC, VPC, SVT and paroxysmal AF have been shown to be independently associated with the presence of ASA.

Atrial septal aneurysm in pregnancy: echocardiography and obstetric outcomes.

Background Atrial septal aneurysm (ASA) is an uncommon cardiac anomaly that increases maternal morbidity during pregnancy. ASA is related to atrial arrhythmia thromboembolism and it may accompany congenital anomalies such as an atrial septal defect (ASD) or patent foramen ovale (PFO). There are no studies examining pregnancy outcomes in pregnant women with ASA. We aimed to investigate the cardiologic parameters and obstetric outcomes of pregnant women diagnosed with ASA. Methods This prospective cohort study analyzed 45 pregnant women diagnosed with ASA, who continued their follow-ups in an obstetric tertiary care center. Results A total of 45 pregnant women were recruited; seven pregnancies ended before the 20th gestational week (six spontaneous abortion, one fetal anomaly), 38 women gave birth. In total, there were 32 term births (≥37 weeks), six preterm births (<37 weeks), two extremely preterm births <28 weeks). Among 38 babies delivered, several obstetrical complications such as oligohydramnios, spontaneous preterm labor, intrauterine growth restriction (IUGR), preeclampsia and gestational diabetes mellitus (GDM) developed in 16 patients, while 22 women ended with term pregnancy without any complications. Deep venous thrombosis (DVT) developed in one patient. Conclusion ASA may have an increased risk for cardiac complications during pregnancy and may also be associated with poor pregnancy outcomes. Increased attention to these entities with more studies is needed in order to determine a potential risk for pregnant women.

A giant atrial septal aneurysm causing tricuspid valvular obstruction.

Atrial septal aneurysm (ASA) is a rare congenital malformation consisting of redundant atrial septal tissue that bulges into either the right or the left atrium. Here, we report the case of a 45-year-old man with a giant ASA prolapsing into the tricuspid orifice and leading to tricuspid valvular obstruction. He was treated successfully by resection of the ASA and repair of the resulting atrial septal defect. Our case indicates the utility of transthoracic, contrast, and transesophageal echocardiography to characterize a huge ASA causing tricuspid valvular obstruction.

Reprint of : Transcatheter closure of patent foramen ovale to prevent stroke recurrence in patients with otherwise unexplained ischaemic stroke: Expert consensus of the French Neurovascular Society and the French Society of Cardiology.

BACKGROUND: Unlike previous randomized clinical trials (RCTs), recent trials and meta-analyses have shown that transcatheter closure of patent foramen ovale (PFO) reduces stroke recurrence risk in young and middle-aged adults with an otherwise unexplained PFO-associated ischaemic stroke. AIM: To produce an expert consensus on the role of transcatheter PFO closure and antithrombotic drugs for secondary stroke prevention in patients with PFO-associated ischaemic stroke. METHODS: Five neurologists and five cardiologists with extensive experience in the relevant field were nominated by the French Neurovascular Society and the French Society of Cardiology to make recommendations based on evidence from RCTs and meta-analyses. RESULTS: The experts recommend that any decision concerning treatment of patients with PFO-associated ischaemic stroke should be taken after neurological and cardiological evaluation, bringing together the necessary neurovascular, echocardiography and interventional cardiology expertise. Transcatheter PFO closure is recommended in patients fulfilling all the following criteria: age 16-60 years; recent (≤6 months) ischaemic stroke; PFO associated with atrial septal aneurysm (>10mm) or with a right-to-left shunt>20 microbubbles or with a diameter≥2mm; PFO felt to be the most likely cause of stroke after thorough aetiological evaluation by a stroke specialist. Long-term oral anticoagulation may be considered in the event of contraindication to or patient refusal of PFO closure, in the absence of a high bleeding risk. After PFO closure, dual anti-platelet therapy with aspirin (75mg/day) and clopidogrel (75mg/day) is recommended for 3 months, followed by monotherapy with aspirin or clopidogrel for≥5 years. CONCLUSIONS: Although a big step forward that will benefit many patients has been taken with recent trials, many questions remain unanswered. Pending results from further studies, decision-making regarding management of patients with PFO-associated ischaemic stroke should be based on a close coordination between neurologists/stroke specialists and cardiologists.

Device Closure of Patent Foramen Ovale for Cryptogenic Stroke: Patient Selection and Outcomes According to New Randomized Trials.

PURPOSE OF REVIEW: This review summarizes the most recent randomized clinical trials that studied the role of device-mediated patent foramen ovale (PFO) closure in patients after an ischemic stroke presumed to have been caused by a paradoxical embolism. RECENT FINDINGS: Three major randomized trials published in 2017 studied the strategy of using PFO closure for secondary prevention in patients between the ages of 18 and 60 who presented with an index stroke having characteristics of an embolic mechanism. All patients had a PFO that potentially could have enabled paradoxical embolism and other causes of stroke were excluded by a thorough neurologic and cardiac evaluation. Patients were randomized to PFO closure versus medical therapy alone using a variety of guideline-recommended medications. After multiple years of follow-up, all three trials showed superiority in the device arm versus the medical arm with a relative risk reduction of recurrent stroke from 46 to 100% and an absolute recurrent stroke reduction from 0.49 to 1.32% per year. Complications related to the procedure and the device were infrequent and mostly transient. These results have transformed the care of these patients, lead to FDA approval of two PFO closure devices, and started the process of updating guidelines. Patient selection is critically important since the presence of a PFO may be incidental. Therefore, both a neurologist and a cardiologist, who can also perform this procedure safely and effectively, should complete the initial evaluation and discuss their findings and recommendations with the patient as part of a shared decision-making process. There are remaining questions regarding how these trial results relate to older patients, patients with overt venothrombotic disease, and those with thrombophilia.

A Proposed Etiology for Atrial Tachyarrhythmia in Neonates with Atrial Septal Aneurysms.

An atrial septal aneurysm (ASA) is an increasingly recognized entity that involves septal tissue significantly bulging into either atria instead of remaining in a relatively neutral position. ASAs may be described based on the length of the segment of atrial septal tissue involved as well as the distance and direction of excursion into the atria throughout the cardiac cycle. Complications associated with ASA include arrhythmias and thromboembolic events with the latter usually in the context of atrial shunting. While the presence of an ASA has been implicated in the development of atrial tachyarrhythmias, no clear mechanism has been elucidated to-date. In this case, we document one of the previously proposed mechanisms of atrial arrhythmia using echocardiography imaging.

Is there any association between rs1303 (Pi*M3) variant of alpha-1 antitrypsin gene and atrial septal aneurysm development?

AIM: Atrial septal aneurysm (ASA) is one of the congenital heart defects. The underlying pathophysiology of ASA has not been fully understood yet. Alpha-1 antitrypsin (A1AT) is a serine protease inhibitor glycoprotein, which is held responsible from tissue wall proteolysis if it is deficient in the body. The aim of this study was to investigate A1AT serum levels and the rs1303 (Pi*M3) variant in A1AT gene in patients with ASA. MATERIAL AND METHODS: Thirty patients (7 male and 23 female) with isolated ASA and 33 patients (11 male and 22 female) with normal atrial septum on echocardiography were included in this study. A1AT serum levels of study patients were measured quantitatively by the enzyme-linked immune sorbent assay (ELISA) method. The A1AT gene mutation rs1303 was analyzed by genotyping, which is performed on genomic DNA extracted from circulating mononuclear blood cells. Single-nucleotide polymorphism was evaluated on polymerase chain reaction using commercial kits. RESULTS: A1AT serum levels were not statistically different among patients with and without ASA (9.52 ± 4.33 µg/mL vs 9.83 ± 5.27 µg/mL, respectively, P = .80). A1AT homozygote mutation (PiM3M3) was significantly higher in the ASA group than the control group (21 vs 11, OR (95% CI): 6.68 [2.09-21.40], P = .001). A1AT serum levels were similar among patients with normal A1AT allele (PiMM), homozygote variant (PiM3M3), and heterozygote variant (PiMM3) (P = .79). CONCLUSION: This preliminary study revealed that homozygote A1AT rs1303 (PiM3M3) variant is significantly higher in patients with isolated ASA and may be associated with ASA development. Large scale comprehensive studies are needed to validate these results.

An unusual "mass" within the left atrium: Localized aneurysm of a patent foramen ovale tunnel.

Masses on the interatrial septum are usually caused by myxomas or thrombi within a patent foramen ovale. We report a 53-year-old male patient with a hollow mass within the left atrium that was incidentally found during a routine transthoracic echocardiography. Further investigation of the mass with transesophageal two- and three-dimensional echocardiography has revealed that the mass was a localized aneurysm of a patent foramen ovale tunnel. While aneurysms of interatrial septum are relatively common in normal population, to the best of our knowledge, present case is the first report of a localized aneursym of a patent foramen ovale tunnel.

Identification of candidates for PFO closure in the echocardiography laboratory.

A patent foramen ovale (PFO) is implicated in several pathologic processes, including that of cryptogenic stroke (cCVA). Recent trials identify "high-risk" PFOs in patients with cCVA as likely to benefit from percutaneous closure. The younger the patient (<60 years old) the more likely a PFO may be attributable to the cCVA. The RoPE Score index helps determine the likelihood that an existing PFO is related to a cCVA. This may help guide the clinician and patient when contemplating percutaneous PFO closure. When evaluating a patient for possible percutaneous closure, one should identify the CVA as a typical ischemic type stroke. In order to "rule-out" other causes of CVA, imaging of the intracranial arteries, cervical, and aortic arch vessels should be performed. Small vessel disease or a lacunar-type infarct should be excluded. To rule out atrial fibrillation, prolonged monitoring should be performed. An index has been developed to determine the probability that a PFO is the causative etiology and calculates the risk of recurrence. This may help guide the clinician and patient in the decision for PFO closure. In addition, one should consider a work-up for a hypercoagulable state. We will obtain an ultrasound of the lower extremities or consider deep pelvic vein thrombosis (prolonged sitting or malignancy). If the closure is to be performed, the Food and Drug Administration (FDA) has approved the Amplatzer PFO Occluder and the GORE Cardioform Septal Occluder for percutaneous closure. These devices are both approved in patients predominately between ages 18 and 60 years with a cCVA due to presumed paradoxical embolism as verified by a neurologist and cardiologist and when other causes of ischemic CVA have been excluded. "High-risk" PFOs appear to achieve the most potential benefit from percutaneous closure.

Multiperforated atrial septal "aneurysm in aneurysm": Percutaneous closure guided by real-time three-dimensional transesophageal echocardiography.

Atrial septal aneurysm (ASA) is a localized deformity of the interatrial septum, which protrudes into the right and/or left atrium. ASA with defects is a source of cardiac embolism. Although transcatheter closure is now a common procedure, it is still challenging in patients with complicated ASA. We describe a case of giant atypical multiperforated ASA with irregular mobility, which showed a rare "aneurysm in aneurysm" structure. Real-time three-dimensional transesophageal echocardiography showed vivid images, which clearly demonstrated these irregular shaped defects mimicking the atriotomy view, and played a crucial role in guiding the transcatheter closure.

Big thrombus "sitting" in an atrial septal aneurysm.

A 79 year-old-man presented three episodes of upper gastrointestinal bleeding and weight loss. Endoscopy revealed bleeding and extrinsic compression at the pyloric region. Computed tomography scan showed a pancreatic tumor, peritoneal carcinomatosis, vascular infiltration, and incidentally found a partially calcified hypodense lesion of 35 mm in the left atrium, suggesting a myxoma or a thrombus. Echocardiography revealed moderate left atrium enlargement, dilated left atrial appendage with spontaneous echo contrast, moderate dilatation and dysfunction of the left ventricle, ejection fraction was 39%, and an atrial septal aneurysm in which a piriform, mass of 35×33×25 mm, was "sitting," suggesting an organized thrombus.

[Atrial septal aneurysm: Evolution of diagnostic and clinical judgements]. / Anevrizma mezhpredserdnoi peregorodki: évoliutsiia diagnosticheskikh i klinicheskikh predstavlenii.

The article discusses anatomical and clinical judgements on atrial septal aneurysm (ASA) as a primary cardiac structural abnormality. It presents current approaches to the classification of ASA and its echocardiographic diagnosis. Special attention is focused on the clinical significance of ASA as an isolated anomaly and concurrent with other structural abnormalities of the heart, especially from the standpoint of a risk of cardioembolic stroke.

Displacement of Amplatzer septal occluder in a patient with atrial septal defects and an atrial septal aneurysm.

Transcatheter closure of atrial septal defects has become more common because of its high success rate and low morbidity; however, this treatment for patients with atrial septal aneurysms is still challenging.

Maternal hyperoxygenation improves left heart filling in fetuses with atrial septal aneurysm causing impediment to left ventricular inflow.

OBJECTIVES: Aneurysm of the atrial septum (AAS) with excessive excursion of septum primum into the left atrium is an uncommon and relatively benign fetal condition associated with impediment to left ventricular (LV) filling and the appearance of a slender, but apex-forming, LV on fetal echocardiography. Impediment to filling can be severe, creating the image of LV hypoplasia with retrograde aortic flow. We hypothesize that maternal hyperoxygenation alters atrial septal position, improves LV filling, and normalizes aortic flow in fetuses with AAS by increasing fetal pulmonary venous return. METHODS: Fetal echocardiography was performed prior to, and at 10 min of, maternal hyperoxygenation in 12 fetuses with AAS who were referred to our center because of LV hypoplasia. Atrial septal excursion (ASE), LV and right ventricular (RV) sphericity index (SI) and direction of flow in the aortic isthmus, as determined by Doppler, were measured. RESULTS: With maternal hyperoxygenation, mean ± SD ASE decreased (0.76 ± 0.17 before maternal hyperoxygenation vs 0.53 ± 0.23 after maternal hyperoxygenation; P < 0.01), consistent with increased pulmonary venous return, LV-SI increased (0.29 ± 0.06 vs 0.42 ± 0.06; P < 0.001), indicating increased LV filling, and the direction of aortic isthmus flow changed from retrograde in all cases prior to maternal hyperoxygenation to antegrade in 10 and to bidirectional in two. RV-SI remained unchanged (0.53 ± 0.13 vs 0.52 ± 0.10; P = 0.7). CONCLUSIONS: In cases of AAS, short-term maternal hyperoxygenation increases fetal pulmonary venous return, substantially alters LV geometry and promotes antegrade flow in the aortic isthmus. This demonstrates proof-of-concept that maternal hyperoxygenation can improve filling of the left side of the fetal heart in AAS.

Atrial Septal Aneurysms and Supraventricular Arrhythmias: The Role of Atrial Electromechanical Delay.

BACKGROUND: Paroxysmal supraventricular arrhythmias (SVAs) frequently occur in patients with atrial septal aneurysm (ASA). The aim of the current study was to evaluate the electrocardiographic (P-wave duration and dispersion) and echocardiographic (atrial electromechanical delay, AEMD) noninvasive indicators of atrial conduction heterogeneity in healthy ASA subjects without interatrial shunt and to assess the AEMD role in predicting the SVAs onset in this population. MATERIALS AND METHODS: One hundered ASA patients (41 males, mean age of 32.5 ± 8 years) and 100 healthy subjects used as controls, matched for age and gender, were studied for the occurrence of SVAs during a 4-year follow-up, through 30-day external loop recorder (ELR) monitoring performed every 3 months. ASAs were diagnosed by transthoracic echocardiography based on the criteria of a minimal aneurismal base of ≥15 mm and an excursion of ≥10 mm. Intra-AEMD and inter-AEMD of both atria were measured through tissue Doppler echocardiography. P-wave dispersion (PD) was carefully measured using 12-lead electrocardiogram (ECG). RESULTS: Compared to the healthy control group, the ASA group showed a statistically significant increase in inter-AEMD, intra-left AEMD, maximum P-wave duration, and PD. Dividing the ASA group into 2 subgroups (patients with or without SVAs), the inter-AEMD, intra-left AEMD, P max, and PD were significantly higher in the subgroup with SVAs compared to the subgroup without SVAs. There were significant good correlations of intra-left AEMD and inter-AEMD with PD. A cutoff value of 40.1 msec for intra-left AEMD had a sensitivity of 82% and a specificity of 83% in identifying ASA patients at risk for SVA. CONCLUSION: Our results showed that the echocardiographic AEMD indices (intra-left and inter-AEMD) and the PD were significantly increased in healthy ASA subjects without interatrial shunt. PD and AEMD represent noninvasive, inexpensive, useful, and simple parameters to assess the SVAs' risk in ASA patients.

Giant Aneurysm of the Interatrial Septum: A Multidisciplinary Approach.

A 46-year-old woman presented for a routine echocardiogram, which revealed a giant atrial septal aneurysm (ASA) with an atrial septal defect (ASD). We surgically excised the ASA and closed the ASD with a pericardial patch. ASA, although rare, poses embolic risks, thus necessitating closure when associated with shunts. Optimal stroke prevention remains uncertain.