Coronary Sinus Atrial Septal Defect Research Articles

Totally Endoscopic MICS via Left Atriotomy Using a Right-Side Approach for Patch Closure of an Unroofed Coronary Sinus Atrial Septal Defect

Totally Endoscopic MICS via Left Atriotomy Using a Right-Side Approach for Patch Closure of an Unroofed Coronary Sinus Atrial Septal Defect

540 RAGHIB SYNDROME WITH VERY LATE CLINICAL PRESENTATION.

Abstract A 72-year-old woman who had been diagnosed with pulmonary emphysema and permanent atrial fibrillation was admitted to our emergency department with signs and symptoms of heart failure (NYHA class III). Transthoracic echocardiography (TTE) revealed dilatation of both right and left atria, dilatation and systolic dysfunction of the right ventricle (RV) and severe pulmonary hypertension. Combined severe mitral and tricuspid regurgitation was also noted and, after clinical stabilization, transesophageal echocardiography (TEE) was performed to better assess valvular anatomy and function. TEE revealed clear evidence of a pre-tricuspid left-to-right shunt with signs of significant RV volume overload, but with difficulties in defining the characteristics of a suspected atrial septal defect (ASD). CMR confirmed the presence of a large coronary sinus ASD (Panel A) as responsible for a significant left-to-right shunt (Qp/Qs ∼ 3,5, measured by phase-contrast imaging after correcting for the combined mitral and tricuspid regurgitation; Panel B), as well as severe RV dilatation and dysfunction. Pulmonary venous drainage was normal. Persistent left superior vena cava (SVC; Panel C) draining directly into the left atrium (LA) and a completely unroofed coronary sinus were unveiled. The extremely rare combination of an unroofed coronary sinus with abnormal systemic venous drainage from double SVC circulation (usually as a persistent left SVC) is also known as Raghib syndrome (or Raghib complex). In the ASD with completely unroofed coronary sinus there is no common wall separating the coronary sinus from the LA, making the depiction of the shunt anatomy particularly difficult to assess with echocardiography. When unrecognized in the perinatal age, clinical natural course of Raghib syndrome can vary based on the severity of RV volume overload, and late (or very late) clinical presentations have been rarely reported. CMR, with its large field-of-view capability and multiparametric approach, provides accurate definition of cardiac and great vessels anatomy and function allowing the full non-invasive depiction of most congenital abnormalities. In many cases, multimodality imaging is the key to reach a full depiction of these complex conditions, particularly when adult patients with possible comorbidities need to be evaluated.

Abstract 11183: Unusual Color Flow Leads to Diagnosis of Unroofed Coronary Atrial Septal Defect

Introduction: Unroofed coronary sinus-atrial septal defect (CS-ASD) is a very rare anomaly that may be easily missed by transthoracic echocardiography. Case: A 59 year old woman presented with chronic and progressively worsening dyspnea. TTE demonstrated preserved ejection fraction, right ventricular and atrial dilation, and elevated pulmonary artery pressures. Color doppler demonstrated a persistent diastolic flow at the interatrial septum immediately above the plane of the tricuspid valve, suspicious for atrial septal defect (Fig. 1). Such aberrant flow was not observed on multiple prior TTEs. Decision-Making: Given the uncertain nature of the abnormal color flow seen, further imaging was pursued with a gated Cardiac CT with contrast. CT imaging demonstrated a dilated coronary sinus with a communication between the roof of the terminal coronary sinus and the left atrium, consistent with an unroofed coronary sinus-atrial septal defect (CS-ASD) (Fig. 2). There was no evidence of a persistent left superior vena cava. Cardiac MRI demonstrated a significant right to left shunt with a Qp/Qs of 1.53 using phase contrast imaging. Discussion: CS-ASD is a very rare cardiac anomaly and is the most uncommon type of ASD (<1%), often associated with a persistent left superior vena cava. Given the posterior nature of the defect, it is often missed on TTE. In our case, suspicion was raised by a small abnormal color jet of flow coming from the interatrial septum. Cardiac CT is an excellent diagnostic modality for CS-ASD given its excellent visuospatial capabilities and ability to evaluate pulmonary veins. Cardiac MRI may serve as a helpful adjunct in quantification of shunt flow.

Awareness of a Rare Diagnosis: CIA of the Coronary Sinus (A Case Report)

Interatrial communication is a common congenital heart disease in adulthood. It is subdivided into four distinct groups based on location. Coronary sinus type is the rarest one and represents less than 1% of interatrial septal defects. Its echocardiographic diagnosis can be difficult. We report the case of a 27-year-old female patient, admitted to the emergency department for management of a poorly tolerated supraventricular tachycardia, referred to cardiology after cardioversion. Clinical examination revealed a SaO2 of 95%, a B2 burst at the pulmonary site. The ECG in sinus rhythm showed right bundle branch block and right atrial hypertrophy. Echocardiography showed dilated right chambers, coronary sinus atrial septal defect with a left-to-right shunt, dysplastic mitral valve, and pulmonary pression was estimated at 85mmhg on tricuspid insufficiency flow; right ventricular function was normal. The patient was treated with B-blocker and VKA with a good clinical course. She is waiting a right catheterization to decide on surgical closure of the atrial septal defect. Through this case, we wanted to underline the importance of not omitting the echocardiographic search for a defect of the roof of the interatrial septum in front of a volumetric overload of the right cavities.

Coronary sinus atrial septal defects in adults over the past 20\u2009years at new Tokyo hospital: case series

BackgroundAn isolated coronary sinus (CS) atrial septal defect (ASD) is defined as a CS unroofed in the terminal portion without a persistent left superior vena cava or other anomalies. This defect is rare and part of the wide spectrum of unroofed CS syndrome (URCS). Recently, several reports have described this finding. The database of New Tokyo Hospital was searched to determine the incidence of this defect. Additionally, to raise awareness of this condition, the findings from five patients with CS ASD who underwent surgical repair at New Tokyo Hospital are discussed.Case presentationThe patients were three women and two men with an age range of 63–77 years. All patients underwent transthoracic echocardiography and computed tomography, and one underwent magnetic resonance imaging. In two patients, the defect was found unexpectedly intraoperatively; left-to-right shunting was apparent in the other three patients preoperatively. The pulmonary-to-systemic blood flow ratio ranged from 1.42 to 3.1 following cardiac catheterization, and oxygen saturation step-up was seen on the right side of the heart. Valvular regurgitation was seen in 4/5 patients with different combinations and degrees of mitral, tricuspid, and aortic valve involvement. Right atrial and ventricular dilation were seen in 4/5 patients; three patients had left atrial dilation. Three patients experienced atrial fibrillation, and one of these also experienced paroxysmal ventricular contractions. All patients underwent surgical repair, and some underwent multiple procedures. One patient who had previously undergone kidney transplantation died approximately 1 year postoperatively; the remaining four patients are currently experiencing good activities of daily living without symptoms.ConclusionsCS ASD (Kirklin and Barratt–Boyes type IV URCS) comprised 1.3% of adult congenital heart surgeries and 0.07% of adult open-heart surgeries at New Tokyo Hospital from 1999 to 2019. At New Tokyo Hospital, cardiac surgery is performed mainly for patients with acquired cardiac disease, and CS ASD is rare. Early diagnosis is important, as well as early surgical repair in symptomatic patients, especially those with blood access shunts, which may overload the heart. The case of a poor prognosis in this series is noteworthy, as similar cases have not been reported previously.

A Surgical Case of Coronary Sinus Atrial Septal Defect

A Surgical Case of Coronary Sinus Atrial Septal Defect

Coronary Sinus Atrial Septal Defect without Persistent Left Superior Vena Cava: Single Vessel Coronary Artery Disease, Surgically Managed – A Rarest Case Report

Coronary Sinus Atrial Septal Defect without Persistent Left Superior Vena Cava: Single Vessel Coronary Artery Disease, Surgically Managed – A Rarest Case Report

Unroofed coronary sinus atrial septal defect: 4D flow cardiovascular magnetic resonance vs. cardiac computed tomography

Unroofed coronary sinus atrial septal defect: 4D flow cardiovascular magnetic resonance vs. cardiac computed tomography

Successful catheter ablation of atrioventricular reentrant tachycardia with left lateral bypass tract in a patient with unroofed coronary sinus atrial septal defect

Successful catheter ablation of atrioventricular reentrant tachycardia with left lateral bypass tract in a patient with unroofed coronary sinus atrial septal defect

Transcatheter closure of coronary sinus atrial septal defect by Amplatzer septal occluder device

Coronary Sinus Atrial Septal Defect (CS-ASD) are the rarest defects of the atrial septum, comprising <1% of the five different types of ASDs. Despite the widespread adoption of percutaneous device closure of ASD, till date surgical closure remains the treatment of choice for such defects. A very few isolated case reports of successful percutaneous closure of such defect with covered stent or amplatzer septaloccluder (ASO) device has been reported in world literature. However, uncertainty regarding safety and efficacy limits widespread application of trans-catheter intervention. Here we report a successful trans-catheter closure of a CS- ASD by ASO device in a 4year old boy.

Coronary sinus atrial septal defect in adult (RCD code: IV-2B.1)

We present a case report of a 45‑year old man, who was admitted to medical center due to dyspnea and cough. Right bundle branch block was detected on electrocardiography. Transthoracic echocardiography demonstrated coronary sinus dilatation, enlargement of right‑side chambers, moderate to severe pulmonary hypertension and atrial septal defect with left‑to‑right shunt. Coronary sinus atrial septal defect was diagnosed by transesophageal echocardiography and computed tomography. Surgical repair of the defect was performed. Atrial flutter occurred in early postoperative period and was successfully treated by electrostimulation. Coronary sinus atrial septal defect in adults is a rare congenital heart disease. Transesophageal echocardiography and computed tomography are imaging modalities of choice in such cases. Surgical treatment is needed to prevent further complications. JRCD 2017; 3 (3): 86–88

Coronary Sinus Atrial Septal Defect (Unroofed Coronary Sinus) with Total Anomalous Pulmonary Venous Connection—A Case Report

Introduction: To present a rare occurrence of coronary sinus atrial septal defect (ASD) associated with total anomalous pulmonary venous connection (TAPVC). Case Report: A 16-year-old girl was diagnosed with features of an atrial septal defect (ASD) by transthoracic echocardiography and the absence of PLSVC (persistent left superior vena cava) connection to coronary sinus was confirmed by saline contrast echocardiography (“bubble study”). Discussion: The cause of the dilated coronary sinus was due to total anomalous pulmonary venous connection. Conclusion: Coronary sinus atrial septal defect (ASD) is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava (PLSVC) and occurs in less than 1% of cases of ASDs. It is characterized by the absence of at least a portion of the common wall that separates the coronary sinus and left atrium. Isolated defects are associated with low rate of morbidity and mortality.

Coronary sinus atrial septal defect in a 65-year-old woman: Diagnosis by two- and three-dimensional echocardiography

Coronary sinus atrial septal defect in a 65-year-old woman: Diagnosis by two- and three-dimensional echocardiography

Coronary sinus atrial septal defect in a 65-year-old woman: Diagnosis by two- and three-dimensional echocardiography

Coronary sinus atrial septal defect in a 65-year-old woman: Diagnosis by two- and three-dimensional echocardiography

PP-085 Prenatal Diagnosis of Isolated Ostium Secundum Defect by Ultrasound in the Second Trimester

The heart consists of four chambers. Of these, two of them are named as atriums. These chambers at the heart's upper floor are separated by a membrane wall. The holes of various sizes and locations on this membranous wall is called as Atrial Septal Defect(ASD). ASD has four major types: A-) Secundum ASD: at the Fossa ovalis, the most common B-) Primum ASD: Partial AVSD, C) Sinus venosus ASD and D) Coronary sinus ASD. Sinus venosus ASD and coronary sinus ASD are rare. We aimed to present a secundum ASD case detected in the second-trimester detailed obstetric US examination.

Percutaneous management of coronary sinus atrial septal defect: two cases representing the spectrum for device closure and a review of the literature

Coronary sinus atrial septal defects are the rarest defects of the atrial septum comprising <1% of the five different types of atrial septal defects. Despite the widespread adoption of percutaneous device closure of secundum atrial septal defects, the published experience with percutaneous device closure of coronary sinus atrial septal defects is limited to only a few isolated case reports because of uncertainty regarding safety and efficacy. Open-heart surgical repair remains the treatment of choice for coronary sinus atrial septal defects, although this may not be the only treatment option in selected cases. Herein we describe our own experience with two patients with different clinical presentations and our method of successful percutaneous coronary sinus atrial septal defect closure in each. We then present a review of the anatomic spectrum of coronary sinus atrial septal defects along with a review of contemporary surgical and percutaneous device treatment.

Persistent left superior vena cava draining into left atrium, unroofed coronary sinus, and coronary sinus atrial septal defect as rare cause for severe right heart dilatation: diagnostic steps and therapy

A 44-year-old female patient with dyspnoea New York Heart Association III was referred for diagnostics. Transthoracic echocardiography revealed dilated right ventricle (RV) and impaired right ventricular function with mild pulmonary hypertension. Subsequent contrast application from the right antecubital vein showed early contrast in right atrium (RA, Panel A and Supplementary data online, Movie S1 ) and moderate interatrial shunt. By contrast injection …

HEART's Original [症例] 冠静脈洞型心房中隔欠損症の1治験成人例

HEART's Original [症例] 冠静脈洞型心房中隔欠損症の1治験成人例

Total Anomalous Pulmonary Venous Connection to the Unroofed Coronary Sinus in a Neonate

This report describes the unique case of a newborn with total anomalous pulmonary venous connection to an unroofed coronary sinus associated with Ullrich-Turner syndrome and aortic coarctation. Total anomalous pulmonary venous connection to the unroofed coronary sinus is an extremely uncommon cardiac abnormality. This congenital heart disease is difficult to diagnose and rarely ever reported. Few symptoms are to be expected when it is not associated with other congenital heart defects. This lesion results in a "naturally" corrected total anomalous pulmonary venous return and a coronary sinus atrial septal defect presenting as a left-to-right shunting lesion. Making the diagnosis of this cardiac defect, and if possible, surgical repair is of importance because cardiac dysfunction due to significant atrial left-to-right shunting is a known long-term complication in the older adult patient.

Intraoperative transesophageal echocardiographic guidance for robotically assisted coronary sinus atrial septal defect repair

Intraoperative transesophageal echocardiographic guidance for robotically assisted coronary sinus atrial septal defect repair