Right atrial appendage aneurysm complicated with atrial septal defect: A rare case report

Right atrial appendage aneurysms (RAAAs) are rare heart malformations, presenting as isolated anomalies or co-existing with other structural heart diseases. We describe a rare case of RAAA complicated with an atrial septal defect (ASD). The diagnosis was established using transthoracic echocardiography and confirmed using cardiac magnetic resonance imaging. To treat the ASD and reduced right atrium volume load, ASD transcatheter closure was performed. On echocardiography performed 3 months post discharge, the RAAA was observed to have reduced in size compared to that presurgery. Six years later, she was in good condition without any adverse events.     

Beware of the LASP! A structure with thrombogenic potential

The left atrial septal pouch (LASP) is a recently identified anatomical variant of the interatrial septum. It is the result of the incomplete fusion of septum primum and septum secundum and defined as a recessus communicating with the left atrium without interatrial shunt. Such anatomical feature has been suspected representing a potential thrombogenic source, but its actual role as risk factor for cryptogenic stroke still remains unclear. In this case report, we show two distinct thrombotic masses emerging from the LASP and its related areas.     

Role of Doppler echocardiography for assessing right ventricular cardiac output in patients with atrial septal defect

Background: Although Doppler echocardiography is routinely used to assess left ventricle cardiac output, there are limited data about the feasibility of Doppler echo‐ cardiography for right ventricular (RV) cardiac output assessment in patients with left‐to‐right shunt. The purpose of the study was to determine the correlation be‐ tween Doppler‐derived and Fick‐derived RV cardiac index (CI), and the interobserver correlation in Doppler‐derived RV CI assessment.
Methods: Retrospective study of patients (age ≥18 years) with unrepaired atrial septal defect who underwent cardiac catheterization and echocardiography (within 3 days), 2004‐2017. RV CI was calculated using the hydraulic orifice formula: [.785 × (right ventricle outflow tract diameter)² × right ventricular outflow tract (RVOT) time veloc‐ ity integral × heart rate]/body surface area.
Results: A total of 128 patients (age 52 ± 17 years; female 88 [69%]) met the inclusion criteria. There was a modest correlation between Doppler‐derived and Fick‐derived RV CI (r = .57, P < .001), and the mean difference between Doppler‐derived and Fick‐ derived RV CI was −.3 (95% confidence interval of agreement, −.8 to +.9) L/min/m². There was also a modest correlation between Doppler‐derived RV CI from observer #1 and observer #2 (r = .62, P < .001), and the mean difference between Doppler‐de‐ rived RV CI from observer #1 and observer #2 was −.2 (95% confidence interval of agreement, −.9 to +.6).
Conclusions: The current study demonstrated a modest correlation between Doppler‐derived and Fick‐derived RV cardiac output, and a modest interobserver correlation in Doppler‐derived RV cardiac output assessment. Further studies are required to validate these results and to explore other potential applications such as in patients with chronic pulmonary regurgitation.     

Transcatheter closure of atrial septal defects in adults with the Amplatzer septal occluder

OBJECTIVE—To assess the efficacy and complications of device occlusion of atrial septal defects in adults, using the Amplatzer septal occluder (ASO).
DESIGN—A prospective interventional study.
SETTING—Paediatric cardiology departments in two European teaching hospitals.
PATIENTS—The first 20 patients accepted for atrial septal defect device occlusion, on the basis of transoesophageal echocardiography. Sixteen patients had larger defects with right heart dilatation, while the primary indication for closure in four was a history of early paradoxical embolism.
INTERVENTIONS—Transcatheter atrial septal defect occlusions performed under transoesophageal echocardiography and fluoroscopic guidance between December 1996 and June 1998.
OUTCOME MEASURES—Success of deployment of ASO devices, procedure and fluoroscopic times, complications, and symptoms.
RESULTS—The ASO device was successfully implanted in all 20 patients (14 female), median age 44.2 years, with no complications. Of the 16 patients with right heart dilatation, the median Qp:Qs was 2.5:1. Defects measured 11-22 mm (median 18) on transoesophageal echocardiography, with balloon sized diameter (and device size) of 13-28 mm (median 20). For all 20 patients, the procedure time ranged from 38-78 minutes (median 61), and fluoroscopy 8.4-24.7 minutes (median 15.2). There were residual shunts in three patients at the end of the procedure, which were trivial ( 1 mm) as assessed by transoesophageal echocardiography, and persisted for more than six months in only one patient. Follow up ranged from 0.1-1.5 years (median 0.7). There have been no late complications.
CONCLUSIONS—The ASO device can be used successfully to close selected oval fossa defects in adults, with minimal procedural morbidity and excellent early results.


Keywords: atrial septal defect; interventional cardiac catheterisation; Amplatzer septal occluder     

Webbed left atrial septal pouch—A new anatomical variant

Atrial septal pouch is a recently described anatomical entity. We describe webbed left atrial septal pouch (LSP), a new variant in the spectrum of fusion of septum primum and septum secundum. In the continuum of the natural history of atrial septal closure from patent foramen ovale (PFO) to complete fusion of the septum, we propose the septal anatomy in our patient to be secondary to partially developed adhesions or septa. Potential association of LSP with stroke due to in situ thrombosis and atrial fibrillation has been described. Recognition of septal anatomy is vital to avoid complications related to transseptal puncture.     

房间隔瘤并发继发孔房间隔缺损的介入治疗评价

目的:评价房间隔瘤（ASA）并发继发孔型房间隔缺损（ASD）介入治疗的临床效果。方法:全组21（男8,女13）例,年龄9～56（24士14）岁。经临床、心电图、X线及经胸超声心动图（TTE）检查诊断为ASA并发继发孔型ASD。TTE检查ASA均膨入右心房,测量ASD最大直径12～28（19±7）mm。其中单孔ASD13例,多孔ASD8例,孔间距离1～7mm者7例,12mm者1例。结果:21例均一次封堵成功,技术成功率100%。所用封堵器的直径为16～40（25±8）mm。8例多孔ASD,有7例置入1个封堵器直接封堵多个缺损孔,1例（两个缺损孔之间距离为12mm）分别用18mm和12mm两个封堵器封堵。术后即刻TTE检查显示16例获完全闭合,5例有少或微量残余分流,完全堵闭率76%（16/21）。术后3d复查TTE2例（10%）有微量残余分流。1～6个月复查19例（90%）心脏大小恢复正常,2例（10%）并发心房纤颤患者心脏不同程度的缩小,1例有微量残余分流。1年后随访12例,无封堵器移位及其他并发症。结论:介入治疗房间隔瘤（ASA）并发继发孔型ASD具有操作简便、安全、技术成功率高及封堵效果好等优点。     

Transcatheter versus surgical treatment for isolated superior sinus venosus atrial septal defect

Background

The superior sinus venosus atrial septal defect is a congenital communication between the left and right atria. Open surgical approach by patch closure has historically been the only treatment option. Recently, a transcatheter approach has been developed. This study aims to compare the efficacy and safety of surgical and transcatheter approach in treatment of sinus venosus atrial septal defect.

Methods

Between March 2010 and December 2020, 58 patients (median age: 45.4, range 14.8−73.8) underwent either surgical or transcatheter correction of superior sinus venosus atrial septal defect with partial anomalous pulmonary venous drainage.

Results

Twenty-four patients (median age: 35.4, range 14.8−66.8) underwent surgery while 34 patients (median age: 46.8, range 15.5−73.8) had a transcatheter treatment. During the catheterization era, 41 patients was considered suitable for a transcatheter closure. In 5 patients, surgery was the patient's or referring physician's choice. In 2 cases, the procedure was unsuccessful; the remaining 34 were successfully closed (94.4% of cases). Intensive care unit stay (median of 1 day, range 0.5−4, vs. 0, range 0−2, p < 0.0001) and hospital stay (median 7 days, range 2−15 vs. 2 days, range 1−12, p < 0.0001), were significantly longer in the surgery group. Total early complication rate, consisted on procedural and in-hospital complication, were higher in the surgical group (62.5% vs. 23.5%; p = 0.005). However, complications in both groups were clinically mild. At follow-up, a small residual shunt was present in 6 patients (surgery group: 2 pts; catheterization group: 4 pts; p: NS). Imaging studies showed significant improvement of right ventricular size and unobstructed pulmonary venous return in all patients. No late complications occurred at follow-up.

Conclusions

Transcatheter correction of sinus venosus atrial septal defect is effective and safe in selected patients and may be considered as a valid alternative to surgery.     

Diagnostic value of transesophageal echocardiography in atrial septal aneurysm

Transthoracic and transesophageal echocardiography was performed in 40 consecutive adult patients with an atrial septal aneurysm. In 11 (27%) of 40 patients transthoracic echocardiography failed to demonstrate the lesion and the diagnosis was established by the transesophageal approach only. Interatrial shunting, assessed by echocardiographic contrast study and/or color flow mapping, was detected in 13 (54%) of 24 patients on transthoracic imaging and in 29 (76%) of 38 patients during transesophageal echocardiography. Identification of multiple fenestrations (n=9) and thrombi within the aneurysm (n=2) could be achieved only by transesophageal ultrasound. A cerebrovascular event of suspected embolic origin occurred in 20 (50%) of 40 patients; 11 (55%) of the 20 patients had repeated cerebral events. Except for mitral valve prolapse in 2 patients and spontaneous left atrial contrast phenomenon in 1 patient no other potential cardiac source of embolism could be identified by transesophageal echocardiography. A marked thickening of the aneurysm was present in 14 (70%) of 20 patients with a cerebrovascular event versus only 4 (20%) of 20 patients without a cerebrovascular event (p<0,01). The mechanism of embolization may be both primary thrombus formation within the aneurysm and paradoxical embolization through an interatrial communication as suggested by the findings on transesophageal ultrasound in 2 patients. Although the patients of this study represent a highly selected group it may be concluded that atrial septal aneurysm is a cardiac abnormality with embolic potential. Transesophageal echocardiography has to be regarded the imaging method of choice for evaluation of this lesion.     

Right atrial thrombus migration during echocardiography: a case for urgent intervention.

A 65-year-old woman presented with increasing shortness of breath, chest pain and ST-T wave abnormalities on the electrocardiogram suggestive of unstable angina. Cross-sectional echocardiography performed to assess wall-motion abnormality and left ventricular function revealed a pedunculated right atrial thrombus prolapsing into the right ventricle which suddenly dislodged and migrated into the pulmonary circulation during the examination. A diagnosis of recurrent pulmonary thromboembolism was made, necessitating urgent pulmonary angiography with infusion of streptokinase. The patient made an uneventful recovery.     

A rare congenital anomaly: biatrial appendage aneurysm with atrial and ventricular septal defect

Atrial appendage aneurysms are extremely rare entities in cardiology practice. There are reports of solitary left and right atrial appendage aneurysms in the literature. A case of biatrial appendages aneurysms is reported here. This is the first report of such an anomaly.     

Site-selective atrial septal puncture

Atrial transseptal puncture is a technique that provides pervenous access to the left heart. The aim of this technique is to provide catheter access to the left atrium and ventricle for mapping, as well as stable electrode-endocardial contact for ablation energy application. In the present report, "site-selective" atrial septal puncture is described and illustrated using case reports.     

Giant multiperforated atrial septal aneurysm in a patient with paroxysmal atrial fibrillation.

With the widespread use of transoesophageal echocardiography, atrial septal aneurysm (ASA) is frequently and easily recognized. Diagnostic criteria, however, remain arbitrary. A meticulous search for interatrial shunting (most often patent foramen ovale (PFO)) and associated cardiac diseases is important. ASA is considered a risk factor for cardioembolism, especially in association with patent foramen ovale. We describe a patient with a giant multiperforated ASA presenting with refractory paroxysmal atrial fibrillation. Resection of the aneurysm was performed in combination with a surgical Maze procedure.     

Late tissue erosion after transcatheter closure of an atrial septal defect

We present the case of a 27‐year‐old male presenting with sudden‐onset retrosternal chest pain, dyspnea, and cardiac tamponade due to erosion of an Amplatzer Atrial Septal Occluder (ASO) through the left atrial wall 4 years after placement. Emergent surgical management of the hemopericardium, followed by surgical removal of the device, repair of the left atrial wall perforation, and patch closure of the atrial septal defect (ASD) were performed successfully. Tissue erosion leading to hemopericardium and cardiac tamponade should be suspected in subjects with a history of transcatheter ASD closure who present with acute chest pain or dyspnea and signs of hemodynamic instability © 2015 Wiley Periodicals, Inc.     

Cox-Maze procedure for treatment of atrial flutter associated with an atrial septal defect

A 52-year-old man presented with atrial flutter and was found to have an atrial septal defect (ASD). The paper describes an effective surgical approach for the treatment of this atrial arrhythmia utilizing a right sided Cox-maze III procedure concomitant with ASD closure. Review of the literature and clinical implications are discussed.     

Large thrombus at the site of primary sutured atrial septal defect associated with pulmonary embolism and treatment by thrombolysis

A 53-year-old man with a history of repair of atrial septal defect 3-months ago by primary suture was admitted to an emergency department with sudden onset chest pain and dyspnea. Transthoracic echocardiography revealed a large and mobile thrombus attached to the right side of the interatrial septum. The chest X ray and perfusion lung scan established the diagnosis of the multiple pulmonary emboli. Doppler examination of the venous system for a possible source of thrombus was unremarkable. The postoperative early appearance of thrombus and multiple pulmonary emboli defects at lung scan supported that thrombus was originated from the primary suture site. Thrombolytic treatment achieved complete resolution of the thrombus both in the pulmonary artery and at the repaired septal defect site. The patient was placed on warfarin and thrombus was not detected by transesophageal echocardiography at the 9-month follow-up.     

Amplatzer封堵器经导管治疗多孔房间隔缺损

目的 :探讨 Amplatzer封堵器经导管治疗多孔房间隔缺损的临床应用价值。方法 :全组患者 6（男 2 ,女 4）例 ,年龄 4～ 5 0岁 ,经 TTE或 TEE检查诊断为多孔房间隔缺损。均在 X线和 TTE或 TEE监测下经导管行 Amplatzer封堵器治疗。术后重复 TTE或 TEE、ECG及 X线平片检查以评价疗效。结果 :全组 6例患者 Amplatzer封堵器置入均获成功 ,术中无重要并发症发生。其中置入 1个封堵器的患者 5例 ,置入 2个封堵器的患者 1例。术后即刻TTE或 TEE检查 5例无残余分流 ,1例小缺损孔有少量残余分流 ,随访 1年中少量残余分流仍存在。1例肺动脉压中度增高患者 ,术后 3 0 min测量肺动脉压下降 >2 0 mm Hg。随访期间所有患者封堵器位置形态良好 ,未发现封堵器的变形 ,移位以及金属结构断裂等改变 ;右房 ,右室有不同程度的缩小 ,X线平片显示肺血有不同程度减少 ;术后ECG检查均未见心律失常发生。结论 :Amplatzer封堵器经导管治疗多孔房间隔缺损是安全有效的。对于相隔距离较近的多孔房间隔缺损 ,可采用单个封堵器封堵 ;而相隔距离较远的缺损 ,可采用同时置入两个封堵器     

Evaluation of left-to-right shunts in adults with atrial septal defect using first-pass radionuclide cardiography.

Non-invasive determination of left-to-right shunts at the atrial level was performed by a new procedure using first-pass radionuclide measurement of cardiac output of the right and left ventricle. In 23 patients with coronary artery disease without shunt the mean difference between the cardiac outputs of the right and left ventricle was 0.04 l.min-1, the limits of agreement -0.80 to 0.88 l.min-1 and the 95% confidence interval for the bias -0.14 to 0.22 l.min-1. Right-sided cardiac catheterization was performed to assess the severity of the arterio-venous shunt by oximetry in 18 adult patients with atrial septal defects. The range of pulmonary-to-systemic flow ratios was 1.4 to 5.0 (mean 2.7) by oximetry and 1.3 to 4.4 (mean 2.5) by the radionuclide technique. The mean difference between flow ratios measured by the two methods was 0.25, the limits of agreement, -0.85 to 1.35 and the 95% confidence intervals for the bias -0.02 to 0.52. In two patients the difference in flow ratios was more than 1. In one of these the oximetric value was more than 2, and the radionuclide value less than 2. The limits of agreement between flow ratios determined by repeated measurements were -1.05 to 0.97, and by two independent observers -0.75 to 0.77. The present findings suggest that first-pass radionuclide determination of left-to-right shunts through atrial septal defects is both reproducible and accurate when compared to the oximetric technique.