Unroofed Coronary Sinus Syndrome Research Articles

Totally Endoscopic Robotic Repair of Unroofed Coronary Sinus Syndrome.

Totally Endoscopic Robotic Repair of Unroofed Coronary Sinus Syndrome.

The value of cardiac CT in the diagnosis of unroofed coronary sinus syndrome

BackgroundUnroofed 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.Methods9 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.ResultsOnly 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.ConclusionsCCT with various postprocessing technologies has excellent value in diagnosing and differentiating subtypes of UCSS, measuring size of coronary sinus defect, describing accompanying cardiovascular abnormalities.

Transcatheter closure of unroofed coronary sinus syndrome: a short-term result.

Transcatheter closure of unroofed coronary sinus syndrome: a short-term result.

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.

Surgical management of unroofed coronary sinus syndrome: A 20-year-single-center experience.

Unroofed coronary sinus syndrome (UCSS) is rare and often associated with the left superior vena cava (LSVC). We report our experience of the clinical features, diagnosis, associated anomalies, surgical procedures, and late outcomes of UCSS during a 20-year period. One hundred and fifty-nine patients with UCSS were treated surgically and followed up between May 1998 and May 2019. UCSS was confirmed by preoperative echocardiography or computerized tomography (CT) scan in 97 patients and by the surgeons intraoperatively in 62 cases, and 100 cases were associated with LSVC. Among these patients, eight cases underwent ligation, and the intracardiac tunnel or baffle was reconstructed in 71 cases to ensure drainage of the LSVC into the right atrium (RA), and the extracardiac procedure was to lead the LSVC into the RA in three patients. Coronary sinus opening was diverted to the RA by the atrial septal patch in three cases. Thirteen patients underwent the reroof procedures, and two patients had untreated LSVC. The associated cardiac lesions were corrected concomitantly. There were five in-hospital deaths. Follow-up of 143 patients of early survivors was done, and no deaths were reported. No serious complications were observed, with the exception of avulsion of the internal tunnel patch in one patient. UCSS is often misdiagnosed during preoperative evaluation of congenital heart diseases. Preoperative transthoracic echocardiography remains the most important method in the diagnosis of UCSS. We adopted multiple surgical approaches to manage the different types of UCSS with LSVC with good results.

Minimally invasive surgical repair for unroofed coronary sinus syndrome directed by three-dimensional transesophageal echocardiography

BackgroundThe 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 presentationA 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.ConclusionsSuccessful repair of unroofed coronary sinus syndrome was safely and effectively achieved by a minimally invasive approach supported by preoperative three-dimensional transesophageal echocardiography.

Unroofed coronary sinus syndrome: A case report.

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.

Early and midterm outcomes of a modified single-patch technique for repairing complete atrioventricular septal defect in children and adults.

The "modified single-patch" technique (Nunn's technique) has become more widely adopted, especially in small infants with common atrioventricular septal defect (CAVSD). In this study, we retrospectively reviewed our surgical experiences of the "modified single-patch" technique with CAVSD patients, including newborns and adults, to reveal the early and midterm clinical results. There were 233 cases diagnosed with CAVSD undergoing biventricular repair in our hospital from June 2009 to 2016. A total of 197 cases (84.5%) with the "modified single-patch" technique were enrolled in the final analysis, including type A in 106 cases (53.8%), type B in 13 cases (6.6%), type C in 15 cases (7.6%), and intermediate type in 63 cases (32%). Major associated cardiovascular malformations included five cases of unroofed coronary sinus syndrome with LSVC (UCS), five cases of coarctation of the aorta and one case of translocation of the great artery. The median age at operation of the studied 197 cases was 44.3 ± 103.3 months (ranging from 18 days to 58 years old), including two newborns. Detailed information on the "modified single-patch" procedure was described previously, and the other cardiac malformations were repaired simultaneously with surgical repair. There were 14 (7.1%) early deaths and 3 (1.5%) late deaths at 3 months and 1 year after the initial operation, respectively. The total mortality rate was 8.1%, and there was no significant difference in mortality among the different age groups (P = .291). Five patients (2.5%) underwent reoperation because of severe mitral insufficiency (MI), and another patient was reoperated for left ventricular outflow tract obstruction 3 years after the initial surgery. Long-term survival in the <3 months group was significantly lower than that in the group of older children (1-18 years old). Of the 180 survivors who were followed up for at least 1 year, the proportion of severe MI began to drop from 26.2% before the operation to 13.8% just 1-week postoperation and continued to decline to the lowest level, 3.4%, at 3 months postoperation. However, MI began to rebound at 3 months and reached 20.8% at 1-year postoperation. The rebound of MI was evident in the (3 months, 6 months) age group and the >18 years age group. However, compared to the preoperation situation, the proportion of severe tricuspid insufficiencies decreased each month from 24.8% to 0.0% until 3 months after the operation and remained at a relatively low level within 1 year after surgery. In our experience, age is not a limitation of the modified single-patch technique, and patients even in adults with surgical indications can apply this technique. In older children and adults, we should pay more attention to atrioventricular valve annuloplasty. Infants may suffer from severe residual left atrioventricular valve regurgitation, especially in the <3 months age group, so we should improve postoperative follow-up work during infancy.

Surgical treatment of pediatric unroofed coronary sinus syndrome

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. Key words: Child; Unroofed coronary sinus syndrome; Surgery; Persistent left superior vena cava

Left superior vena cava draining into the left atrium: Clinical entities, diagnosis and surgical treatment

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.

Evaluation of Unroofed Coronary Sinus Syndrome Using Cardiovascular CT Angiography: An Observational Study.

The purpose of this study was to determine the prevalence of unroofed coronary sinus (CS) syndrome at a tertiary hospital and analyze the clinical information, cardiovascular CT angiography (CCTA) imaging findings, associated anomalies, and surgical treatment of the identified cases. We retrospectively searched the database of a tertiary hospital for cases of unroofed CS syndrome among patients who underwent CCTA for known or suspected congenital heart disease. After the prevalence of unroofed CS syndrome was determined, CCTA findings, associated cardiovascular abnormalities, presence or absence of airway compression, clinical information, and surgical outcome were recorded. A total of 23 patients with unroofed CS syndrome were identified, with the syndrome therefore having a prevalence of 0.36% among patients with congenital heart disease who underwent CCTA. The diagnostic accuracy of CCTA for unroofed CS syndrome was 100%, whereas that of echocardiography was 69%. Type I unroofed CS syndrome was the most commonly noted type (52% of patients). All 23 patients had associated cardiovascular anomalies, including persistent left superior vena cava (65% of patients) and atrial septal defect (65%). Surgery was performed for 70% of patients because of cardiovascular anomalies. Seven patients (30%) had associated secondary airway compression but did not require surgical correction. At our institution, the prevalence of unroofed CS syndrome was 0.36% among patients with congenital heart disease who underwent CCTA. CCTA has excellent diagnostic performance, delineating different subtypes of unroofed CS syndrome and associated cardiovascular planning for treatment of unroofed CS syndrome abnormalities, improving clinical decision making, and permitting preoperative planning for treatment of unroofed CS syndrome.

Surgical treatment and early-mid follow-up results of complete atrioventricular septal

Objective: To discuss the surgical treatment and early-mid follow-up results of complete atrioventricular septal defect (CAVSD). Methods: The clinic data of 235 cases of CAVSD underwent surgical treatment at Department of Cardiac Surgery, Guangdong General Hospital from June 2009 to June 2016 was analyzed retrospectively (104 male and 131 female patients). The patients were aged 18 days to 58 years (mid age 8.9 months) at surgery, and 2 cases of newborns (0.9%), 24 cases of 1 to 3 months (10.2%), 56 cases of 4 to 6 months (23.8%), 66 cases of 7 to 12 months (28.1%), 36 cases of 1 to 3 years (15.3%), 37 cases of 4 to 18 years (15.7%), and 14 cases above 18 years (6.0%). There were 129 cases of Rastelli type A (54.89%), 16 cases of type B (6.8%), 25 cases of type C (10.6%) and 65 cases of transitional type (27.7%). The patients were combined with 7 cases of unroofed coronary sinus syndrome, 5 cases of coarctation of aorta, 4 cases of tetralogy of Fallot, 3 cases of double outlet right ventricle, 1 case of transposition of the great arteries, 1 case of multiple ventricular septal defect, 1 case of pulmonary artery sling. The CAVSD correction operations were performed in 199 cases with modified single patch technique (84.7%), 26 cases with two patch technique (11.1%), 10 cases with conventional single patch technique (4.3%), and the corrective surgeries of other anomalies were performed in the meantime. Generalized Estimated Equation was used to statistical analysis of postoperative regurgitation level of mitral valve and tricuspid valve. Results: Followed-up for 1 to 7 years, 18 cases (7.7%) died overall and 15 cases (6.4%) died within 30 days post-operation.The mortality of little infant (operation age ≤3 months) was much highest(26.9%). The mortality of different operation age had significant difference(P=0.007). There was no difference among the mortality with three techniques. Seven cases (3.0%) suffered from the re-operation for severe mitral regurgitation. A total of 217 patients survived more than 1 year after initial surgery, and there were 11 cases (5.1%) with severe mitral regurgitation and 7 cases (3.2%) with severe tricuspid valve regurgitation at 1 year post-operation.The degree of mitral regurgitation after operation decreased at 1 week to 6 months, and slightly higher after 1 year postoperatively.The ratios of severe mitral regurgitation in different operative age group was statistically significant difference(P=0.017), mainly from 3 months group and 1 to 3 years group.The degree of tricuspid regurgitation decreased significantly after operation, and decreased gradually in the follow-up period. The ratio of postoperative residual severe tricuspid regurgitation was the highest in surgical aged >18 years old, less in 3 years group, the lowest in 3 to 18 years group. Conclusions: The mortality and the ratio of patients with serious mitral regurgitation were higher in infants less 3 months than elder patients. The infants need more frequently follow-up and medicine treatment after operation. The modified one patch technique is effective for all age groups.

CT angiography for diagnosis and subcategorization of unroofed coronary sinus syndrome.

To evaluate the role of CT angiography (CTA) in the diagnosis and subcategorization of unroofed coronary sinus syndrome (URCS). 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. 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. 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.

Unroofed Coronary Sinus Syndrome: An Easily Corrected Congenital Anomaly But More Diagnostic Suspicion Is Needed

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. Thirty-six patients with URCSS who underwent surgical treatment from 1998 to 2015 in our institution were enrolled in this retrospective analysis. 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. Diagnostic suspicion can contribute to lesion disclosure. Surgical correction has good long-term outcomes and low risks.

Unroofed coronary sinus detected by 2D/3D echocardiography in a patient referred to catheter ablation for atrial fibrillation

A 67-year-old female visited our echo lab for a preoperative screening exam before radiofrequency catheter ablation for atrial fibrillation. Transthoracic echocardiography revealed abnormal color flow signal in the right atrium, and following 2D/3D transesophageal echocardiography proved partial type of unroofed coronary sinus. Absence of associated congenital abnormalities was ruled out by Doppler and contrast echo examinations. Surgical findings confirmed exactly the same anatomical abnormalities as preoperative 2D/3D echocardiography. Echocardiography plays an important role in finding unexpected underlying cardiac diseases in patients who are referred to arrhythmia clinics. In the present case with atrial fibrillation, we could accurately diagnose an unroofed coronary sinus syndrome during preoperative transthoracic and transesophageal echocardiography prior to planned radiofrequency catheter ablation.<Learning objective: Radiofrequency catheter ablation therapy for atrial fibrillation has been greatly developed in recent years. Echocardiography plays an important role in the diagnosis of underlying cardiac diseases in patients who are referred to arrhythmia clinics. We could clearly visualize partial type of unroofed coronary sinus by 2D/3D echocardiography in a patient who was planned to undergo radiofrequency catheter ablation, and then could contribute to the proper therapeutic management for the rare congenital heart disease.>

Partially Unroofed Coronary Sinus: MDCT and MRI Findings

The purpose of this study was to analyze the MDCT and MRI findings in patients with unroofed coronary sinus syndrome. This retrospective study included 11 patients with unroofed coronary sinus syndrome (10 adults, one child) without persistent left superior vena cava and one adult with communication of the left atrium and coronary sinus via an anomalous vein (unroofed coronary sinus variant). Four patients underwent contrast-enhanced ECG-gated MDCT; six, MRI; and two patients, both CT and MRI. We also measured the coronary sinus on the CT scans of 28 adults with normal cardiac anatomy and 10 adults with persistent left superior vena cava and compared the measurements with those in the patients with unroofed coronary sinus syndrome. Seven patients underwent surgical treatment of unroofed coronary sinus syndrome. At echocardiography, unroofed coronary sinus syndrome was not clearly discriminated from atrial septal defect in two patients and was not suspected in three patients. CT and MRI showed that 11 patients had a defect in which the coronary sinus communicated with the left atrium and that the other patient had atresia of the coronary sinus orifice with an anomalous vein connecting the coronary sinus and left atrium. In patients with unroofed coronary sinus syndrome, the mean standardized diameter of the coronary sinus according to the patient's body surface area was 15 ± 4 mm/m², similar to that of the control group with persistent left superior vena cava (15 ± 6 mm/m²; p = 0.97) and significantly greater than that of the group with normal cardiac anatomy (7 ± 2 mm/m²; p < 0.0001). CT and MRI facilitate definite diagnosis of unroofed coronary sinus syndrome.

Diagnosis and surgical treatment of unroofed coronary sinus syndrome

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 typeⅠa,1 with typeⅠb,1 with typeⅡa,3 with type Ⅲa,and 1 with type Ⅲb 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(Ⅰa) 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,Ⅱa) and left atrium(3 cases,Ⅲa) 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.

Clinical analysis of unroofed coronary sinus syndrome with atrioventricular canal defect

To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with unroofed coronary sinus syndrome and atrioventricular canal defect. The clinical data of 20 patients with unroofed coronary sinus syndrome from September 1999 to October 2007 were reviewed retrospectively. There were 10 male and 10 female patients. The age ranged from 6 months to 38 years old, with a mean of (11.4 +/- 11.0) years old. The body weight ranged from 6.7 to 73.0 kg, with a mean of (28.4 +/- 21.3) kg. There were 18 cases of partial atrioventricular canal defect, 2 cases of complete atrioventricular canal defect, and 12 cases of common atrium. The initial diagnosis of unroofed coronary sinus syndrome was made by the surgeon at repair of a partial or complete atrioventricular canal defect in 20 patients. Complex unroofed coronary sinus with left superior vena cava (LSVC) directly draining into the left atrium was found in 11 cases, 1 case of LSVC was ligated, 10 cases were reconstructed the intraatrial baffle or the intracardiac tunnel to drain LSVC to right atrium. The other 9 patients with simple unroofed coronary sinus were repaired with other procedures. The associated cardiac lesions were corrected concomitantly. Death occurred in 1 patient with complex congenital cardiac disease due to pulmonary infection. In the 14 early survivors, who had been followed up from 4 months to 3 years, there was no death and severe complications. When associated with a partial or complete atrioventricular canal defect, LSVC and a common atrium, unroofed coronary sinus syndrome should be considered as a possible additional finding. Repair according to the type of unroofed coronary sinus syndrome is effective.

Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment

Unroofed coronary sinus syndrome: diagnosis, classification, and surgical treatment

Uncommon presentation and surgical correction of unroofed coronary sinus syndrome

A 59-year-old man with signs and symptoms of congestive heart failure, occurring a few months after an infective episode, underwent cardiac investigations revealing severe biventricular dysfunction, persistent left superior vena cava with almost completely unroofed coronary sinus, and critical stenosis of the proximal right coronary artery. Surgical correction of the congenital malformation associated with revascularization of the right coronary allowed a prompt recovery of clinical conditions and ventricular function.