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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.
Subject(s)
Humans , Middle Aged , Coronary Sinus , Heart Atria , Heart Septal Defects, Atrial , Vena Cava, SuperiorABSTRACT
A 15-year-old boy with coronary sinus type atrial septal defect (CS-ASD) was surgically treated with 3-port totally endoscopic technique. The patient was set in a left semi-lateral position. A 3 cm skin incision retracted by a small wound protector, a trocar for the endoscope, and a trocar for left-handed instruments were placed in the right antero-lateral chest. Cardio-pulmonary bypass was established via groin cannulation. After cardioplegic arrest, the CS-ASD was favorably exposed through the left atriotomy, and closed using a bovine pericardial patch. The total operation time was 112 min. The post-operative course was uneventful. Instead of the traditional median sternotomy and right atriotomy, small right thoracotomy and left atriotomy may be a promising alternative for closure of CS-ASD.
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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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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 summarize the prenatal ultrasonographic characteristics of unroofed coronary sinus( URCS) for improving the prenatal echocardiography diagnostic accuracy of URCS . Methods The retrospective review for prenatal ultrasonogram of 7 fetuses was applied ,which were diagnosed as URCS . The prenatal ultrasonographic characteristics of 7 fetuses with URCS were reviewed and comparatively analyzed with postpartum autopsy findings or postnatal echocardiography . The relevant literatures referring to the prenatal diagnosis of URCS were reviewed . The experience of prenatal diagnosis of URCS were summarized . Results In 7 cases with URCS ,5 cases of completely URCS were associated with right atrial isomerism syndrome and confirmed by postpartum autopsy ,and 2 cases of partially URCS were only with persistent left superior vena cava and confirmed by postnatal echocardiography . The karyotype analysis of 2 cases of partially URCS on amniotic fluid was normal . Conclusions There are some characteristic signs in ultrasonic features of URCS . URCS can be diagnosed by prenatally ultrasonography . A long axial section of the coronary sinus is the most important view for prenatal ultrasonic diagnosis of U RCS .
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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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No abstract available.
Subject(s)
Coronary Sinus , Heart Defects, Congenital , Heart Septal Defects, AtrialABSTRACT
Objective To conclude the diagnostic methodology of unroofed coronary sinus (UCS) by two-dimensional echocardiography(2DE). Methods By analyzing the echocardiographic results of 16 UCS patients who were diagnosed by 2DE and confirmed by operation, the diagnostic methodology of UCS by 2DE was summarized. Results Sixteen patients with UCS were involved in this study. Among them, 12 cases were diagnosed as complete UCS,others were partial UCS(PUCS). Twelve patients were complicated with persistence of left superior vena cava (LSVC) that was connected to left atrium (LA) through UCS. All of preoperative diagnosis conducted by 2DE were finally confirmed to be consistent with the results of operation. With the analysis of acquired echo images,key points of diagnosis were concluded as follow: 1) normal coronary sinus (CS) could not be detected in the routine 2DE views referring CS. PUCS showed partial absence of CS roof,while complete UCS displayed as total absence of CS. 2)Inter-atrial shunt would definitely be found in UCS and the opening to right atrium must be coronary sinus orifice. The shunt direction was depended on the combined cardiac malformations. 3) With the occurrence of UCS,LSVC would be in junction with LA through UCS. 4) The inter-atrial shunt resulted in cardiac morphologic and hemodynamic changes. Conclusions Better understanding of the anatomic, morphological and hemodynamic characteristics of UCS would greatly contribute to accurate diagnosis on UCS.
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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.
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Unroofed coronary sinus with persistent left superior vena cava is an extremely rare congenital anomaly. It can be suspected by the presence of a markedly dilated coronary sinus and can be confirmed by injection of agitated saline into the left antecubital vein during echocardiography. Agitated saline contrast echocardiography can easily visualize the flow in the persistent left superior vena cava communicating with the unroofed coronary sinus hereby creating a shunt into the left atrium. An agitated saline contrast echocardiography may be performed with a low risk of transient complication. We demonstrate the case of a 32-year-old pregnant woman with unroofed coronary sinus and persistent left superior vena cava, confirmed by agitated saline contrast echocardiography.
Subject(s)
Adult , Female , Humans , Coronary Sinus , Dihydroergotamine , Echocardiography , Heart Atria , Pregnant Women , Veins , Vena Cava, SuperiorABSTRACT
We report a case of 51-year-old woman with pure unroofed coronary sinus without persistent left superior vena cava and other cardiac anomaly. She presented with dyspnea on exertion during several years. Her chest film showed prominent cardiomegaly and dilated hilar vessels. Cardiac rhythm was atrial fibrillation. Transthoracic echocardiography demonstrated the enlarged coronary sinus with defect toward left atrium on parasternal long axis view and significant flow from coronary sinus into right atrium on subxyphoid view, and its other findings were dilated right ventricle and right atrium, paradoxical septal motion, moderate tricuspid regurgitation and mild mitral regurgitation, which were mimicking of large secundum atrial septal defect. Radionuclide cardioangiography and cardiac catheterization showed the existence of significant shunt. There was no evidence of persistent left superior vena cava on chest CT. Closure of Coronary sinus opening was done. Thereafter her symptoms of congestive heart failure were much improved. We think that careful examination of 2-D echocardiography can be valuable tool for diagnosis of unroofed coronary sinus in adult patient.
Subject(s)
Adult , Female , Humans , Middle Aged , Atrial Fibrillation , Axis, Cervical Vertebra , Cardiac Catheterization , Cardiac Catheters , Cardiomegaly , Coronary Sinus , Diagnosis , Dyspnea , Echocardiography , Heart Atria , Heart Failure , Heart Septal Defects, Atrial , Heart Ventricles , Mitral Valve Insufficiency , Thorax , Tomography, X-Ray Computed , Tricuspid Valve Insufficiency , Vena Cava, SuperiorABSTRACT
A 47-year-old woman complained of dyspnea on exertion. Ultrasonic cardiography revealed coronary sinus type atrial septal defect. At operation, the drainage veins to the left atrium from the coronary arteries were observed but no anomalies of the vena cave or any other veins were observed. The defect was closed with a pericardial patch under cardiopulmonary bypass. The post-operative course was uneventful. Coronary arteriography performed on the 14th post operative day confirmed that the coronary veins drained individually into the corresponding atria. Unroofed coronary sinus is rare and difficult to diagnose prior to operation. Ultrasonic cardiography and coronary arteriography are considered useful for preoperative diagnosis.