Multimodality Imaging of Sinus Venosus Atrial Septal Defect: A Challenging Diagnosis in Adults.
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ABSTRACT:
SUBMITTER: Qiu JK
PROVIDER: S-EPMC9120852 | biostudies-literature | 2022 May
REPOSITORIES: biostudies-literature
Publications
Multimodality Imaging of Sinus Venosus Atrial Septal Defect: A Challenging Diagnosis in Adults.
Qiu Jessica K JK Bamira Daniel D Vainrib Alan F AF Latson Larry A LA Halpern Dan G DG Chun Anne A Saric Muhamed M
CASE (Philadelphia, Pa.) 20211231 3
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Project description:In contrast with transthoracic echocardiography, transesophageal echocardiography provides a sure way to make the diagnosis of sinus venosus atrial septal defect; on the other hand this abnormality is more complex than that seen with the secundum atrial septal defect, and inexperienced operators may fail to recognize properly the defect. In front of a high reported sensitivity using transesophageal echocardiography, specificity is difficult to assess, due to possible underreporting of diagnostic errors. We describe a false positive diagnosis of sinus venosus atrial septal defect, in the setting of enlarged right chambers of the heart because of pressure overload. Modified anatomy of the heart, together with the presence of a prominent linear structure(probably Eustachian Valve) and an incomplete examination in this case made image interpretation very prone to misinterpretation. In this anatomical setting transesophageal longitudinal "bicaval" view may be sub-optimal for examining the atrial septum, potentially showing false images that need to be known for correct image interpretation. Nonetheless, a scan plane taken more accurately at the superior level would have demonstrated/excluded the pathognomonic feature of sinus venosus atrial septal defect in the high atrial septum, between the fatty limbus and the inferior aspect of the right pulmonary artery; moreover TEE allows morphological information about the posterior structures of the heart that need to be investigated in detail for a complete diagnosis.
| S-EPMC270092 | biostudies-literature
Project description:Transcatheter correction of superior sinus venosus atrial septal defect (SVASD) is being considered as an alternative to surgery in selected patients. We present the case of a 42-year-old woman with SVASD and partial anomalous venous connection of the right upper pulmonary vein (RUPV), who underwent transcatheter correction with self-expanding aortic stent graft, following feasibility assessment by balloon occlusion. Hemodynamic parameters and angiography demonstrated successful closure of the SVASD without any residual shunt and unobstructed return of RUPV to the left atrium. She developed cardiac tamponade after a few hours despite pericardial drain and underwent emergency exploratory thoracotomy. This revealed leak from a small rent in the ascending aortic wall adjacent to superior vena cava (SVC) caused by barbs of the stent protruding from SVC, without any leak in SVC. This was repaired with suture and further Teflon was placed around the barbs in SVC to prevent further injury. We also discuss the possible reason for this complication, considering our successful previous two cases with the same stents. This case highlights the importance of assessing the relationship between SVC and aorta to decide about the cranial placement of the aortic stent either by computed tomography prior or by contrast aortogram during the procedure. <Learning objective: Transcatheter correction of superior sinus venosus atrial septal defect is becoming an appropriate alternative option for cardiac surgery. There are multiple reports of this technique in the literature - mostly with balloon expandable stents, and few with self-expanding stents placed in superior vena cava (SVC). We present an unanticipated complication of self-expanding aortic stent in this situation and its management, highlighting the need for assessing the relationship between SVC and aorta to decide about the appropriate placement of stent.>.
| S-EPMC8766320 | biostudies-literature
Project description:Dilated cardiomyopathy, heart failure and atrial septal defects are well-recognised entities in isolation, but are rarely seen together. Now that 90% of children with congenital heart disease survive into adulthood, such combinations of disease are increasingly seen in adult cardiology. While most young patients with dilated cardiomyopathy respond well to medical therapy, some do not, and require more invasive management. We describe a 32 year-old man with dilated cardiomyopathy and a sinus venosus-type atrial septal defect associated with a remarkable pulmonary to systemic flow ratio of 5:1. We propose that the atrial septal defect blunted his heart failure symptoms by serving as a 'pop-off' valve and limiting pulmonary congestion. The patient ultimately failed medical management and received a left ventricular assist device. The case is presented along with a discussion of this unique pathophysiology and a brief review of the literature in this rapidly evolving field.
| S-EPMC4039816 | biostudies-other
Project description:Sinus venosus atrial septal defect (SVASD) with partial anomalous pulmonary venous return is a rare congenital heart defect that may present in adulthood when right heart enlargement has already occurred. We describe a case of unrepaired sinus venosus atrial septal defect and partial anomalous pulmonary venous return with massive right heart enlargement and recurrent atrial arrhythmias.
| S-EPMC10774777 | biostudies-literature
Project description:BackgroundTranscatheter correction of superior sinus venosus atrial septal defect (SVASD) with partial anomalous pulmonary venous drainage (PAPVD) of the right upper and/or right middle pulmonary vein (RUPV/RMPV) has recently been described as an alternative to surgical approach in a substantial number of patients. We describe a modified technical approach for transcatheter correction of SVASD using transoesophageal echocardiography (TOE) to confirm adequate stent landing zone and apposition and evaluate its feasibility, safety, and procedural success.Case summaryFrom 2019 to 2021, three consecutive patients received a transcatheter correction of SVASD with PAPVD by redirecting the superior vena cava and RUPV/RMPV to the left atrium by implantation of a custom-made covered Cheatham platinum stent (10-zig, length: 60-80 mm). Prior to stent implantation, a balloon testing was performed in the anticipated landing zone using TOE to confirm complete defect closure and unobstructed pulmonary venous drainage. Stent deployment and flaring of the interior stent portion were performed with TOE guidance to confirm adequate landing zone and apposition and to avoid residual shunt or pulmonary vein obstruction.ConclusionTranscatheter correction of SVASD with PAPVD was performed without any complications. The follow-up period was 7.8, 13.6, and 29.8 months, respectively. During follow-up, no mortality, stent embolization, or obstruction of pulmonary venous drainage occurred. The TOE-guided modified transcatheter approach for correction of SVASD with PAPVD is safe and feasible with excellent post-procedural results and represents an alternative to surgical treatment in a pre-selected patient cohort.
| S-EPMC9883701 | biostudies-literature
Project description:This study aimed to present a case of 33-year old man who was admitted with a history of one week headache and acute diplopia. No important finding was reported in his past medical history. Brain CT-scan revealed a large mass lesion in left parieto-occipital area with prominent vasogenic edema and midline shift. Brain magnetic resonance imaging (MRI) showed a mass with size of 5*4*5 centimeter with ring enhancement. After cranial surgery and removing the mass, transthoracic and transesophageal echocardiography (TEE) were conducted to find the source of brain abscess. Right ventricular (RV) and right atrial (RA) enlargement, significant left to right shunt, normal left ventricular (LV) and RV function, bidirectional shunt in addition to moderate size superior sinus venosus type atrial septal defect (ASD) were detected. Considering that most of brain abscesses have hematogenous source, a complete cardiac evaluation including TEE with contrast study is suggested for evaluation of patients with brain abscess.
| S-EPMC6891039 | biostudies-literature
Project description:Background Lipomatous atrial septal hypertrophy (LASH) with atrial septal defect (ASD) is a rare congenital anomaly. Although LASH is a histologically benign cardiac lesion characterized by excessive fat deposition in the interatrial septum that spares the fossa ovale, it has been associated with supraventricular arrhythmias or sick sinus syndrome. Application of multimodal imaging is crucial for accurate diagnosis, appropriate treatment of LASH with ASD, and follow-up. Case summary A 68-year-old female patient presented with recurrent chest tightness and palpitation. Multimodal imaging revealed the characterizations of LASH and ASD. Two-dimensional transesophageal echocardiography showed a “dumbbell”-shaped involvement of the cephalad and caudal regions with sparing of a single secundum ASD. The septum with a brightness feature is an uncommon condition characterized by the deposition of unencapsulated fat cells in the atrial septum. Real-time four-dimensional transesophageal echocardiography reflected the lipomatous hypertrophy of the atrial septum and an oval-shaped ASD. Cardiac computer tomography angiography later confirmed this finding. The patient achieved a good clinical response with an ASD percutaneous occlusion guided by intracardiac echocardiography (ICE). Conclusion This case demonstrates a LASH combined with ASD. Multimodality imaging can provide an accurate diagnosis and may guide the procedure for precise occlusion.
| S-EPMC10482038 | biostudies-literature
Project description:A 63-year-old man with cirrhosis, hepatocellular carcinoma, and coagulopathy was diagnosed with a sinus venosus atrial septal defect (ASD) and partial anomalous pulmonary venous return (PAPVR) of the right upper pulmonary vein (RUPV). Transcatheter repair by positioning a stent graft in the superior vena cava was planned. Based on three-dimensional (3D) reconstruction of gated cardiac CTA, a 28 mm × 7 cm Endurant II® aortic extension stent graft (Medtronic, MN) was chosen. A 3D model printed from the CTA was used to simulate device deployment, demonstrating successful exclusion of the sinus venosus ASD with return of the RUPV to the left atrium (LA). Post simulation, the 3D model was used for informed consent. The patient was then taken to the hybrid operating room. On-table cone beam CT was performed and registered with the CTA images. This enabled overlay of 3D regions of interest to live 2D fluoroscopy. The stent graft was then deployed using 3D regions of interest for guidance. Hemodynamics and angiography demonstrated successful exclusion of the sinus venosus ASD and unobstructed return of RUPV to the LA. This is the first report of comprehensive use of contemporary imaging for planning, simulation, patient consent, and procedural guidance for patient-centered complex structural intervention in repair of sinus venosus ASD with PAPVR. We propose this as a process model for continued innovation in structural interventions.
| S-EPMC6175141 | biostudies-literature
Project description:BackgroundBrain abscess is a common complication in children with cyanotic congenital heart disease. The presence of an underlying acyanotic congenital heart disease is usually not suspected in an adult patient presenting with brain abscess.Case summaryA 51-year-old male patient with no known co-morbidities came with complaints of recent onset right lower limb weakness needing support while walking and on evaluation was found to have brain abscess. He underwent robotic endoscope assisted endoport excision of the brain abscess. Two-dimensional transthoracic echocardiography showed right atrial and right ventricular dilatation with mild low-pressure tricuspid regurgitation. Transoesophageal echocardiography (TOE) revealed sinus venosus atrial septal defect (ASD) with left-to-right shunt with the right upper pulmonary vein draining into superior vena cava. Contrast echocardiography revealed a small transient right-to-left shunt. He has been advised to undergo elective surgical closure of ASD with partial anomalous pulmonary venous connection repair.DiscussionRight-to-left shunting in ASDs can occur in the early systole even in the absence of raised pressures in the right side of the heart, even when the predominant shunt is left to right, but the magnitude of such a shunt is small and transient and is easily missed. Contrast echocardiography and TOE should be done as a part of evaluation of patients presenting with brain abscess.
| S-EPMC8343453 | biostudies-literature
Project description: Not available
| S-EPMC10144289 | biostudies-literature