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Introduction and importance: Raghib syndrome is a rare congenital complication consisting of the termination of the left superior vena cava (LSVC) in the left atrium, an unroofed coronary sinus, and an atrial septal defect most often found in the posterior-inferior angle of the atrial septum. Both a right-to-left and a left-to-right intracardiac shunt exist. In most circumstances, they do not show any symptoms. Presentation of case: The patient presented with a persistent left superior vena cava draining into the left atrium, an unroofed coronary sinus, and a secondary atrial septal defect (ASD). Transthoracic echocardiography was used to diagnose the condition, and surgery was applied as the primary treatment. Clinical discussion: It was formerly believed that this complex was exclusive to Raghib syndrome; however, cases have been found in which the interatrial connection is the aperture of the unroofed coronary sinus in patients with a normal atrial septum. Conclusion: Extracardiac treatment for this illness reduces the load on the left atrial suture and may stop further arrhythmias. There is no possibility of pulmonary vein flow restriction when there are no atrial tunnels.

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Unroofed coronary sinus syndrome (URCS), also known as coronary sinus septal defect, is a rare congenital heart disease. Because of its special anatomy and the lack of typical clinical symptoms, the disease is easily missed and misdiagnosed. This case report particularly describes a middle-aged male patient with URCS misdiagnosed for more than 19 years, covered by hyperthyroid heart disease, who subsequently developed uncontrollable symptoms such as chest tightness and shortness of breath and came to our hospital. After a clear diagnosis in our hospital, the patient was successfully cured after treatment with coronary sinus repair and tricuspid valvuloplasty under extracorporeal circulation.

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BACKGROUND: Unroofed coronary sinus syndrome (UCSS) is a rare cardiovascular malformation with nonspecific clinical manifestations that easily causes misdiagnosis and missed diagnosis. The aim of this study is to present the different features of UCSS by various CCT (cardiac CT) postprocessing techniques and evaluate the diagnostic advantages of CCT. METHODS: 9 UCSS patients who were diagnosed by imaging and undergone both CCT and transthoracic echocardiography (TTE) were included in this study, and their CCT images were reviewed. The UCSS images were classified by multiplanar reformations, maximum intensity projection, volume rendering and cinematic rendering. The size of CS roof defect was also measured. RESULTS: Only 4 of 9 CCT confirmed UCSS patients were detected by TTE (4/9, 44.4%), the sensitivity of TTE was lower compared to CCT by Fisher's exact test (P < 0.05). UCSS was classified according to the Kirklin and Barratt Boyes's method, including 1 case was classified as type I, 4 cases as type II, 1 case as type III, 2 cases as type IV, 1 case as type V (variant type), and TTE was undiagnosed in all type III-V patients. Additionally, CCT showed 12 extra malformations in these patients, only 5 of them were found by TTE (5/12, 41.7%), and TTE missed all extracardiac malformations. The mean size of CS roof defect was 3.04 ± 1.57 cm. CONCLUSIONS: CCT with various postprocessing technologies has excellent value in diagnosing and differentiating subtypes of UCSS, measuring size of coronary sinus defect, describing accompanying cardiovascular abnormalities.

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A 35-year-old woman presented with dyspnea on exertion. The diagnosis was unroofed coronary sinus with persistent left superior vena cava and atrial septal defect. She underwent total correction with the intracardiac baffle technique by using an interatrial flap and autologous pericardial patch. Her clinical course was uneventful and markedly improved after the surgery.

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Background: Patients with deletions involving the long arm of chromosome 1 are rare, and the main aim of this study was to refine the genotype-phenotype correlation. Case Report: In this report, a 28-year-old pregnant woman, gravida 2 para 1, at 25+4 weeks of gestation underwent ultrasound examination in our institute. The ultrasonographic findings of the fetus were as follows: (1) fetal growth restriction; (2) cleft lip and palate; (3) bilateral renal hypoplasia; (4) lateral ventriculomegaly; (5) single umbilical artery; (6) absent stomach; (7) coronary sinus dilatation with persistent left superior vena cava, ventricular septal defect and unroofed coronary sinus syndrome. Chromosomal microarray analysis of amniotic fluid from the fetus revealed a 28.025 Mb deletion in 1q23.3q31.2, spanning from position 164,559,675 to 192,584,768 (hg19). Conclusion: Genotype-phenotype correlation might improve prenatal diagnosis of fetuses with chromosome 1q deletion. PBX1 could be a candidate gene for fetal growth restriction, renal hypoplasia and congenital heart disease. Fetal growth restriction was accompanied by decreased renal volume in the fetus. Combined with ultrasonic examination, the application of chromosomal microarray analysis will provide accurate prenatal diagnosis.

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BACKGROUND: An 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 PRESENTATION: The 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. CONCLUSIONS: CS 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.

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BACKGROUND: The recent remarkable development of cardiac imaging technology for unroofed coronary sinus syndrome has led to accurate preoperative diagnosis. We report a case of unroofed coronary sinus syndrome repaired via a minimally invasive approach, under the excellent command of three-dimensional transesophageal echocardiography. CASE PRESENTATION: A 77-year-old woman with hypertension was admitted for aggravation of bilateral leg edema and diagnosed with type III unroofed coronary sinus syndrome with Qp/Qs ratio of 1.6:1. The unroofed portion was detected at the atrial side between P2 and P3 of posterior mitral leaflet by preoperative three-dimensional transesophageal echocardiography. Right minithoracotomy was performed at the fourth intercostal space and cardiopulmonary bypass routinely established. Right atriotomy and left atriotomy incisions were made under antegrade cardioplegic arrest. The unroofed portion was revealed at the same location by preoperative transesophageal echocardiography and was clearly recognized only by endoscopy, not by direct vision. It was repaired by direct running suture under endoscopic visualization. We observed no blood cardioplegia leakage or mitral insufficiency, which was also confirmed by postoperative transesophageal echocardiography. The patient's postoperative course was uneventful and she was discharged home 14 days after surgery without any residual shunt. CONCLUSIONS: Successful repair of unroofed coronary sinus syndrome was safely and effectively achieved by a minimally invasive approach supported by preoperative three-dimensional transesophageal echocardiography.

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Unroofed coronary sinus syndrome (UCSS), also named coronary sinus septal defect, is a rare type of atrial septal defect with the incidence less than 1% of the total number of atrial septal defects. It is caused by incomplete formation of left atrial venous folds during embryonic development. Here we reported a patient with UCSS, who was treated in the Second Xiangya Hospital of Central South University. The patient was 50 years old and the main clinical manifestations were fatigue and shortness of breath after repeated exercise. Color Doppler echocardiography showed coronary sinus dilatation (17 mm×14 mm), indicating the possibility of permanent left superior vena cava. Pulmonary angiography showed that the left ventricle and coronary sinus were developed at the same time while the atrial septum was intact after the development of the left atrium, followed by the right atrium and right ventricle, indicating a partial anomalous pulmonary venous drainage (intracardiac type). Finally, the cardiac computed tomograhic angiography showed that 4 pulmonary veins and permanent left superior vena cava (PLSVC) went into the left atrium and the coronary sinus, respectively, while the coronary sinus septum was absent and the PLSVC was connected with the left atrium. The patient was later treated with the correction of non-parietal sinus syndrome in the Cardiovascular Surgery Department of our hospital.

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Persistent left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect (Raghib complex) is an uncommon anomaly of systemic venous drainage. We present a unique case of simultaneous presentation of cor triatriatum and persistent left superior vena cava draining into the left atrium in an adult female with partial AV canal and common atrium. Complex intra-atrial baffling including a procedure to redirect flow from a proximal atrial chamber was successful.

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Unroofed coronary sinus syndrome (UCSS), also named coronary sinus septal defect, is a rare type of atrial septal defect with the incidence less than 1% of the total number of atrial septal defects. It is caused by incomplete formation of left atrial venous folds during embryonic development. Here we reported a patient with UCSS, who was treated in the Second Xiangya Hospital of Central South University. The patient was 50 years old and the main clinical manifestations were fatigue and shortness of breath after repeated exercise. Color Doppler echocardiography showed coronary sinus dilatation (17 mm×14 mm), indicating the possibility of permanent left superior vena cava. Pulmonary angiography showed that the left ventricle and coronary sinus were developed at the same time while the atrial septum was intact after the development of the left atrium, followed by the right atrium and right ventricle, indicating a partial anomalous pulmonary venous drainage (intracardiac type). Finally, the cardiac computed tomograhic angiography showed that 4 pulmonary veins and permanent left superior vena cava (PLSVC) went into the left atrium and the coronary sinus, respectively, while the coronary sinus septum was absent and the PLSVC was connected with the left atrium. The patient was later treated with the correction of non-parietal sinus syndrome in the Cardiovascular Surgery Department of our hospital.

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Left superior vena cava draining into the left atrium in the absence of coronary sinus is an anomaly that can appear in heterotaxy syndrome and unroofed coronary sinus syndrome. Regardless of the origin of these syndromes, biventricular repair can be done through rerouting by intracardiac procedures or through disconnection-reconnection of the left superior vena cava to the right atrium or right superior vena cava by extracardiac procedures. Different techniques can be used for this purpose, each of which has its own advantages and limitations. Therefore, appropriate selection is necessary to obtain the best results for each patient, and many factors, such as patient anatomy, age, associated cardiomyopathies, etc., have to be considered. In this review, we focus on heterotaxy and unroofed coronary sinus syndromes, associated cardiomyopathies, the state-of-the-art in their surgical treatment and our results in a sample of 10 patients. Our experience highlights the importance of accurate diagnosis and specific selection of surgical technique for the management of biventricular repair in patients with left superior vena cava draining into the left atrium in the absence of coronary sinus.

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Objective To analyze the clinical features and surgical treatment of unroofed coronary sinus syn-drome(UCSS)in children,and to provide evidence for the diagnosis and treatment of such diseases during the perio-perative period. Methods A retrospective analysis was conducted based on the clinical data of 13 patients with UCSS, who underwent surgical treatment at Beijing Children＇s Hospital Affiliated to Capital Medical University from January 2011 to December 2017. All 13 patients were complicated with persistent left superior vena cava( PLSVC)and other cardiac malformations;7 patients were diagnosed preoperatively and 6 patients were diagnosed intraoperatively. Eleven cases were diagnosed typeⅠand 2 cases were typeⅣaccording to Kirklin＇s classification. In all of 13 cases,atrial sep-tal reconstruction was performed to drain the left superior vena cava(LSVC)to the right atrium,and other cardiac mal-formations were corrected at the same time. Results One patient died in this group,and the other 12 patients had early recovery after operation. Postoperative-ventilator-assisted time was 5-246 h( median 29 h)and hospital stay time was 8-40 d(median 16 d). The remaining 12 cases were followed up from 6 months to 7 years and 2 months. No death or complications occurred. No residual shunt or residual obstruction was found. Conclusions In the case of congenital heart disease with PLSVC,UCSS should be alerted. Atrial septal reconstruction and drainage of LSVC into right atrium in children with UCSS can achieve satisfactory surgical results.

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Objective@#To analyze the clinical features and surgical treatment of unroofed coronary sinus syndrome(UCSS) in children, and to provide evidence for the diagnosis and treatment of such diseases during the perio-perative period.@*Methods@#A retrospective analysis was conducted based on the clinical data of 13 patients with UCSS, who underwent surgical treatment at Beijing Children′s Hospital Affiliated to Capital Medical University from January 2011 to December 2017.All 13 patients were complicated with persistent left superior vena cava(PLSVC) and other cardiac malformations; 7 patients were diagnosed preoperatively and 6 patients were diagnosed intraoperatively.Eleven cases were diagnosed type Ⅰ and 2 cases were type Ⅳ according to Kirklin′s classification.In all of 13 cases, atrial septal reconstruction was performed to drain the left superior vena cava(LSVC) to the right atrium, and other cardiac malformations were corrected at the same time.@*Results@#One patient died in this group, and the other 12 patients had early recovery after operation.Postoperative-ventilator-assisted time was 5-246 h(median 29 h) and hospital stay time was 8-40 d(median 16 d). The remaining 12 cases were followed up from 6 months to 7 years and 2 months.No death or complications occurred.No residual shunt or residual obstruction was found.@*Conclusions@#In the case of congenital heart disease with PLSVC, UCSS should be alerted.Atrial septal reconstruction and drainage of LSVC into right atrium in children with UCSS can achieve satisfactory surgical results.

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BACKGROUND: Unroofed coronary sinus syndrome (URCSS) is a spectrum of cardiac anomalies in which part (partial type) or the entire common wall (complete type) between the coronary sinus (CS) and left atrium is absent. It is commonly associated with a persistent left superior vena cava (PLSVC). The PLSVC can even anomalously connect to the left atrium in complete type anomaly. URCSS has been reported to be associated with delayed diagnosis and life-threatening cerebral injury. The purpose of the present study was to review our experience with surgical correction of this often-concealed malformation and discuss methods for reducing diagnostic omission. METHODS: Thirty-six patients with URCSS who underwent surgical treatment from 1998 to 2015 in our institution were enrolled in this retrospective analysis. RESULTS: The mean age of the patients was 21.8±20.9 years. Type I anomalies were found in 15 (41.7%) patients, type II in 4 (11.1%), type III in 6 (16.7%), and type IV in 11 (30.6%). A patient presenting with URCSS without other defects was identified in six (16.7%). One patient (2.8%) had a history of paradoxical embolisation. Twenty-five patients (69.4%) were diagnosed before surgery. Ten patients (27.8%) were diagnosed with URCSS intraoperatively. One patient (2.8%) was diagnosed with URCSS by follow-up echocardiography after device occlusion for atrial septal defect. Coronary sinus ostium dilation was the most important clue leading to partial type lesion disclosure intraoperatively. The anomalous PLSVC of 15 patients was repaired using roof tunnel technique in four (26.7%) cases, baffle redirection in eight (53.3%) cases, posterior left atrial wall plicating in two (13.3%) cases, and extracardiac bypass in one (6.7%) case. Postoperative complete atrioventricular block occurred in one patient (2.8%). One patient died postoperatively (2.8%). All patients are doing well after discharge from hospital. CONCLUSIONS: Diagnostic suspicion can contribute to lesion disclosure. Surgical correction has good long-term outcomes and low risks.

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BACKGROUND: To evaluate the role of CT angiography (CTA) in the diagnosis and subcategorization of unroofed coronary sinus syndrome (URCS). METHODS: We retrospectively analyzed 46 URCS patients diagnosed by CTA. Based on the defect location and size of coronary sinus (CS), URCS was divided into four types: complete defect as type I, partial defect of proximal CS as type II, partial defect of distal CS as type III, partial defect in which a communication occurs between CS and left atrial as type IV. According to presence of left superior vena cava (LSVC), all types were divided into 2 subtypes as a and b. All 46 patients underwent echocardiography. RESULTS: According to subcategorization of URCS by CTA, type I was observed in 23 cases (Ia 7, Ib 16), type II in 10 cases (IIa 3, IIb 7), type III in 12 cases (IIIa 3, IIIb 9), and type IV in 1 case classified as IVb subtype. In these 46 cases, 21 were detected by echocardiography as URCS (46%). The sensitivity of echocardiography in detecting URCS was significantly lower compared with cardiac CTA (P<0.05). In type I patients, the mean CS diameter indexed to body surface area (CS index) was larger than other types (P<0.05). Thirty patients were successfully treated by surgery and the diagnosis of URCS was confirmed by operative findings. Among them, data were available in 22 cases for analysis; and patients with types I, II and IIIa differ significantly from those with types IIIb and IV (P<0.05) with respect to surgical repair. CONCLUSIONS: CTA and imaging reconstruction can provide excellent anatomical delineation of the heart, great vessels, and CS, and allows for precise diagnosis of URCS. This CTA classification scheme of URCS is simple and easy to use, and has important clinical implications for diagnosis and treatment.

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Unroofed coronary sinus syndrome (URCS) is a rare congenital cardiac anomaly. Recently, cardiac surgery using a minimally invasive approach has become the preferred treatment, affording better cosmetic results and a more rapid post-operative recovery than the traditional method. We report the case of a 54-year-old male in whom partial URCS was treated via a totally endoscopic repair technique featuring right mini-thoracotomy.

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Objective To analyze clinical characters,associated anomalies,diagnostic approach,and surgical procedures in patients with unroofed coronary sinus syndrome.Methods The clinical,echocardiographic,operative and follow-up data of 109 patients with unroofed coronary sinus syndrome from May 1999 to July 2016 were reviewed retrospectively(type Ⅰ51cases,type Ⅱ 17 cases,type Ⅲ 17 case and type Ⅳ 24 cases).The initial diagnosis of unroofed coronary sinus syndrome was made by echocardiography in 56 cases,while the remains were confirmed by the surgeon at repair of other congenital cardiac anomalies.69 of the 109 cases associated with left superior vena cava(LSVC),and 53 of the 69 directed drained into the left atrium(LA).Among the 109 cases,2 cases associated with complex anomalies underwent palliative operations,and other cardiac malformations were corrected simultaneously by surgical correction in the other cases.LSVC was ligated in 8 cases,and the intracardiac tunnel to drain LSVC to right atrium(RA) was reconstructed in 38 cases,intracardiac baffle in 9 cases,extracardiac repair in 2 cases.The associated cardiac lesions were corrected concomitantly.Results There were 5 hospital deaths.3 patients died of low cardiac output syndrome and 2 patients died of pulmonary infection.We followed up 87 early survivors,and there was no death and severe complications.Conclusion Unroofed coronary sinus syndrome is often missed in the preoperative evaluation of congenitally malformed hearts.Preoperative TTE is still the most important method in the diagnosis of UCSS.Strengthening the understanding of the pathological anatomy and physiological characteristics of the heart malformation may help us to improve the preoperative diagnosis of UCSS.When associated with LSVC,UCSS should be considered as a possible additional finding.We performed different surgical approaches to deal with the different kinds of LSVC with a good result.

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Objective To summarize the clinical manifestations and surgical treatment of unroofed coronary sinus syndrome(UCSS). Methods Nine patients underwent surgical repair for UCSS from Jan. 1999 to Jun. 2009 in our hospital, and the patients included 3 with type I a, 1 with type I b, 1 with type II a, 3 with type III a, and 1 with type III b UCSS. The diagnosis of UCSS was made by echocardiography in 6 patients and by the surgical exploration in 3 patients. Persistent left superior vena cava (PLSVC) was found in 7 cases; 3 cases with PLSVC directly draining into the left atrium ( I a) were reconstructed by the intra-atrial routes to guide PLSVC to the right atrium, and 4 cases with PLSVC draining into the coronary sinus and coronary sinus orifice opening to the left atrium and right atrium (1 case, II a) and left atrium (3 cases, III a) were dealt with the abnormal coronary sinus orifice. Other associated cardiac lesions were corrected concomitantly. Results Two cases had early complications and the other 7 cases had smooth recovery after operation; all the patients were cured and discharged. Eight cases were followed up for 2 months to 5 years and there was no death or late complications. Conclusion UCSS is a rare congenital cardiac anomaly and is difficult to diagnose preoperatively; so careful exploration should be done during operation. Different surgical approaches should be chosen according to the types of communication between PLSVC and cardiac atrium, the positions of abnormal sinus ostium, and other cardiac lesions. Satisfactory prognosis can be obtained if physiological correction is achieved.

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Objective To summarize the surgical procedures of unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava.Methods From 2000 to 2008,753 cases of endocardial cushion defect underwent surgical repair.Among them 15 cages(2.0%)were associated with persistent left superior vena cava and unroofed coronary sinus syndrome.The drainage of the persistent left superior vena cava and coronary vein were corrected simultaneously during the operation by reestablishing the roof of the coronary sinus.The persistent left superior vena cava were drained to right atrium by intra-atrial tunnel with pericardial patch or left atrium folding in 14 cases and it was ligated in the remain one.Most of the coronary veins were drained into right atrium after the correction of cardiac defect.Results One patient died of severe pulmonary infection early after operation.Echocardiogram indicated that the velocity of left superior vena cava increased in the site of the roof of left atrium in one patient after operation.No obstruction of vena cava and pulmonary vein and remnant shunt between the atriums were found in the other 14 cases.There were no death and other complications during3 months to 6 years of follow-up.Conclusion The surgical procedures of unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava is difficult and good result can be expected by reestablishing the roof of the coronary sinus.