BACKGROUND Unroofed coronary sinus syndrome(UCSS)is a rare congenital heart disease,which has variable morphologic features and is strongly associated with persistent left superior vena cava(PLSVC).However,it is often...BACKGROUND Unroofed coronary sinus syndrome(UCSS)is a rare congenital heart disease,which has variable morphologic features and is strongly associated with persistent left superior vena cava(PLSVC).However,it is often difficult to visualize the left-to-right shunt pathway through the CS by transthoracic echocardiography(TTE).CASE SUMMARY A 37-year-old female was admitted to the hepatological surgery department of a hospital with complaint of subxiphoid pain that had started 1 wk prior.Physical examination revealed a grade 3/6 systolic murmur at the left margin of the sternum,between the 2nd and 3rd intercostal cartilage.The patient underwent echocardiography and was diagnosed with ostium primum atrial septal defect(ASD);thus,she was subsequently transferred to the cardiovascular surgery department.A second TTE evaluation before surgery showed type IV UCSS with secundum ASD.Right-heart contrast echocardiography(RHCE)showed that the right atrium and right ventricle were immediately filled with microbubbles,but no microbubble was observed in the CS.Meanwhile,negative filling was observed at the right atrium orifice of the CS and right atrium side of the secundum atrial septal.RHCE identified UCSS combined with secundum ASD but without PLSVC in this patient.CONCLUSION This rare case of UCSS highlights the value of TTE combined with RHCE in confirming UCSS with ASD or PLSVC.展开更多
Ruptured sinus of Valsalva is very uncommon,and is < 1% of all congenital defects.The incidence ranges from 0.1%-3.5%.There is a male to female predominance of 4:1,with the highest incidence in the Asian population...Ruptured sinus of Valsalva is very uncommon,and is < 1% of all congenital defects.The incidence ranges from 0.1%-3.5%.There is a male to female predominance of 4:1,with the highest incidence in the Asian population.Higher incidence is also seen in patients with Marfan's syndrome and Ehlers Danlos syndrome.There is a higher association of ruptured sinus of Valsalva with ventricular septal defect(VSD),aortic stenosis,and bicuspid valve defect.While most patients with VSD often have rupture of their right coronary sinus of Valsalva into the right ventricle due to poor structural integrity,we present a rare case of a patient with VSD who had rupture of his noncoronary sinus of Valsalva into the right atrium.展开更多
Atrial septal defect (ASD)is the third commonest congenital heart disease in adults.The most common type of ASD is the ostium secundum accounting for 75%-80%of all ASDs.Less common are the ostium primum (accounting fo...Atrial septal defect (ASD)is the third commonest congenital heart disease in adults.The most common type of ASD is the ostium secundum accounting for 75%-80%of all ASDs.Less common are the ostium primum (accounting for 15%of all ASDs),the sinus venosus defect (5%-10%) and the coronary sinus defect (less than 1%).[1,2]展开更多
Background: Superior sinus venosus atrial septal defect (SV-ASD) is an interatrial defect with partial anomalous pulmonary venous connection (PAPVC) draining into the right atrium. The principle for SV-ASD surgical re...Background: Superior sinus venosus atrial septal defect (SV-ASD) is an interatrial defect with partial anomalous pulmonary venous connection (PAPVC) draining into the right atrium. The principle for SV-ASD surgical repair involves redirecting the pulmonary venous blood back to the left atrium and closure of the atrial septal defect without compromising the adjoining structures. Methods: We reviewed English literature relating to this topic via a MEDLINE and Google scholar search using the following terms: surgery for sinus venosus atrial septal defect (SV-ASD), surgery for partial anomalous pulmonary venous connection (PAPVC) and complications of the surgery. Results: A total of 910 consecutive cohort patients with different surgical options between 1984 to 2012 were analyzed. The breakdown showed that 291(32.0%) had single-patch, 275(30.2%) had Warden’s procedure, 185(20.3%) had a double-patch and 159(17.5%) had use of autologous right atrial appendage for the anastomosis. The total follow-up was between 0.008-30 years. Sinus node dysfunction (SND) was the commonest complication occurring mostly in patients who had double-patch technique 16(8.6%) followed by 15(5.5%) patients with single-patch technique, 7(4.4%) patients in whom right atrial appendage was used and 5(1.8%) patients with Warden’s procedure. Venous obstruction occurred in 22(7.7%) patients with SP, 13(5.1%) patients with Warden’s Procedure and 5(2.7%) patients with double-patch. Conclusion: The two major complications, SND and venous obstruction, assumed a see-saw approach as surgical maneuvers avoiding venous obstruction encouraged SND and vice versa. However, adopting surgical options which avoided incision across the Cavoatrial junction attenuated all the complications.展开更多
Background Sinus venosus atrial septal defect（SVASD） is a rare atrial septal defect. The right-sided pulmonary veins may drain into the right atrium directly or via vena cava. Two-dimensional transthoracic echocardi...Background Sinus venosus atrial septal defect（SVASD） is a rare atrial septal defect. The right-sided pulmonary veins may drain into the right atrium directly or via vena cava. Two-dimensional transthoracic echocardiography（2D-TTE） is certainly the initial choice of evaluation of SVASD but is unable to show its spatial relation to the surrounding structures and the configuration of the veins accurately. The purpose of this study was to evaluate the diagnostic accuracy of real-time three-dimensional transesophageal echocardiography（RT3D-TEE） for detecting SVASD and partial anomalous pulmonary venous connection（PAPVC）. Methods2D-TTE and RT3D-TEE were performed in 25 patients with the SVASD before surgical repair. Records of these patients were compared with surgical assessments. Results Twenty-three of the 25 patients with SVASD were detected by 2D-TTE. All the 23 patents were associated with PAPVC. Two of the 25 patients with SVASD were misdiagnosed as the secundum atrial septal defect by 2D-TTE. SVASD was demonstrated in all the 25 cases by RT3D-TEE, and 25 patents were associated with PAPVC. Conclusions RT3D-TEE is highly accurate, scientific method for displaying the location, size, shape of the defect and its spatial relation to the surrounding structures, and provides additional value to the surgeon and physicians for better understanding of spatial intracardiac morphology and making more accurate diagnosis and treatment.展开更多
Sinus of Valsalva aneurysm(SoVA)is a rare cardiac defect that may be congenital or acquired.It is characterized by abnormal dilatation of the aortic root due to a weakened elastic lamina at the junction of the annulus...Sinus of Valsalva aneurysm(SoVA)is a rare cardiac defect that may be congenital or acquired.It is characterized by abnormal dilatation of the aortic root due to a weakened elastic lamina at the junction of the annulus fibrosus and the aortic media.SoVAs are present in approximately 0.09%of the general population and comprise up to 3.5%of all congenital cardiac defects.It is usually found inci-dentally on cardiac imaging,with a higher incidence observed in the Western populations and a male-to-female ratio of 4:1.A transthoracic two-dimensional echocardiogram is the initial diagnostic test of choice,which may reveal the characteristic“windsock deformity”that clinches the diagnosis.Other imaging modalities,such as transesophageal echocardiography and cardiac computed tomography angiography,help provide more extensive details of the aneurysm and its adjacent structures.Management options for ruptured and unruptured SoVA include surgical repair or transcatheter closure,which serves as a game-changing development in treatment.This article aims to provide background information on the epidemiology,pathophysiology,diagnosis,and recent advan-cements over the past decade in the management of SoVAs.展开更多
Background:Partial anomalous pulmonary venous connection(PAPVC)is frequently associated with atrial septal defect(ASD),especially sinus venosus defect(SVD).Although Waggstaffe described the pathology of SVDs in 1868,t...Background:Partial anomalous pulmonary venous connection(PAPVC)is frequently associated with atrial septal defect(ASD),especially sinus venosus defect(SVD).Although Waggstaffe described the pathology of SVDs in 1868,the exact anatomic features and the nature of SVD remains controversial.SVDs with no posterior atrial rim were observed in recent years.However,no studies suggested that absence of the residual posterior atrial septal tissue might be the key feature of SVD.The aims of this study were to investigate if absence of posterior rim of atrial septum played a crucial role in patients with SVD.Methods:From January 2011 to December 2019,256 children with PAPVC combined ASD and 878 children with isolated ASD who underwent corrective cardiac surgery were consecutively enrolled.Comprehensive review of preoperative transthoracic echocardiography,computed-tomography images and surgical findings were performed by experienced pediatric cardiologists.The subtypes of PAPVC,locations and types of ASD,and presence of posterior atrial rim of associated ASD were investigated.Results:PAPVC was right-sided in 244 children,left-sided in 6 children,and bilateral in 6 children.In PAPVC cases,ASD without posterior atrial rim existed in 226 SVD cases.ASD without posterior atrial septum only existed in cases with one or more right pulmonary veins returning to right atrium(RA)or to RA-superior vena cava junction.In cases with isolated ASD,there were 3 SVD,and the other 875 cases were secundum ASD.Conclusions:ASD without posterior atrial rims was associated with one or more right pulmonary veins returning to RA or RA-superior venous cava(SVC)junction.For SVD,the key feature is that the defect is in the posterior of the interatrial septum with no posterior septal rim,rather than adjacent to the SVC or to the inferior vena cava.展开更多
文摘BACKGROUND Unroofed coronary sinus syndrome(UCSS)is a rare congenital heart disease,which has variable morphologic features and is strongly associated with persistent left superior vena cava(PLSVC).However,it is often difficult to visualize the left-to-right shunt pathway through the CS by transthoracic echocardiography(TTE).CASE SUMMARY A 37-year-old female was admitted to the hepatological surgery department of a hospital with complaint of subxiphoid pain that had started 1 wk prior.Physical examination revealed a grade 3/6 systolic murmur at the left margin of the sternum,between the 2nd and 3rd intercostal cartilage.The patient underwent echocardiography and was diagnosed with ostium primum atrial septal defect(ASD);thus,she was subsequently transferred to the cardiovascular surgery department.A second TTE evaluation before surgery showed type IV UCSS with secundum ASD.Right-heart contrast echocardiography(RHCE)showed that the right atrium and right ventricle were immediately filled with microbubbles,but no microbubble was observed in the CS.Meanwhile,negative filling was observed at the right atrium orifice of the CS and right atrium side of the secundum atrial septal.RHCE identified UCSS combined with secundum ASD but without PLSVC in this patient.CONCLUSION This rare case of UCSS highlights the value of TTE combined with RHCE in confirming UCSS with ASD or PLSVC.
文摘Ruptured sinus of Valsalva is very uncommon,and is < 1% of all congenital defects.The incidence ranges from 0.1%-3.5%.There is a male to female predominance of 4:1,with the highest incidence in the Asian population.Higher incidence is also seen in patients with Marfan's syndrome and Ehlers Danlos syndrome.There is a higher association of ruptured sinus of Valsalva with ventricular septal defect(VSD),aortic stenosis,and bicuspid valve defect.While most patients with VSD often have rupture of their right coronary sinus of Valsalva into the right ventricle due to poor structural integrity,we present a rare case of a patient with VSD who had rupture of his noncoronary sinus of Valsalva into the right atrium.
文摘Atrial septal defect (ASD)is the third commonest congenital heart disease in adults.The most common type of ASD is the ostium secundum accounting for 75%-80%of all ASDs.Less common are the ostium primum (accounting for 15%of all ASDs),the sinus venosus defect (5%-10%) and the coronary sinus defect (less than 1%).[1,2]
文摘Background: Superior sinus venosus atrial septal defect (SV-ASD) is an interatrial defect with partial anomalous pulmonary venous connection (PAPVC) draining into the right atrium. The principle for SV-ASD surgical repair involves redirecting the pulmonary venous blood back to the left atrium and closure of the atrial septal defect without compromising the adjoining structures. Methods: We reviewed English literature relating to this topic via a MEDLINE and Google scholar search using the following terms: surgery for sinus venosus atrial septal defect (SV-ASD), surgery for partial anomalous pulmonary venous connection (PAPVC) and complications of the surgery. Results: A total of 910 consecutive cohort patients with different surgical options between 1984 to 2012 were analyzed. The breakdown showed that 291(32.0%) had single-patch, 275(30.2%) had Warden’s procedure, 185(20.3%) had a double-patch and 159(17.5%) had use of autologous right atrial appendage for the anastomosis. The total follow-up was between 0.008-30 years. Sinus node dysfunction (SND) was the commonest complication occurring mostly in patients who had double-patch technique 16(8.6%) followed by 15(5.5%) patients with single-patch technique, 7(4.4%) patients in whom right atrial appendage was used and 5(1.8%) patients with Warden’s procedure. Venous obstruction occurred in 22(7.7%) patients with SP, 13(5.1%) patients with Warden’s Procedure and 5(2.7%) patients with double-patch. Conclusion: The two major complications, SND and venous obstruction, assumed a see-saw approach as surgical maneuvers avoiding venous obstruction encouraged SND and vice versa. However, adopting surgical options which avoided incision across the Cavoatrial junction attenuated all the complications.
文摘Background Sinus venosus atrial septal defect（SVASD） is a rare atrial septal defect. The right-sided pulmonary veins may drain into the right atrium directly or via vena cava. Two-dimensional transthoracic echocardiography（2D-TTE） is certainly the initial choice of evaluation of SVASD but is unable to show its spatial relation to the surrounding structures and the configuration of the veins accurately. The purpose of this study was to evaluate the diagnostic accuracy of real-time three-dimensional transesophageal echocardiography（RT3D-TEE） for detecting SVASD and partial anomalous pulmonary venous connection（PAPVC）. Methods2D-TTE and RT3D-TEE were performed in 25 patients with the SVASD before surgical repair. Records of these patients were compared with surgical assessments. Results Twenty-three of the 25 patients with SVASD were detected by 2D-TTE. All the 23 patents were associated with PAPVC. Two of the 25 patients with SVASD were misdiagnosed as the secundum atrial septal defect by 2D-TTE. SVASD was demonstrated in all the 25 cases by RT3D-TEE, and 25 patents were associated with PAPVC. Conclusions RT3D-TEE is highly accurate, scientific method for displaying the location, size, shape of the defect and its spatial relation to the surrounding structures, and provides additional value to the surgeon and physicians for better understanding of spatial intracardiac morphology and making more accurate diagnosis and treatment.
文摘Sinus of Valsalva aneurysm(SoVA)is a rare cardiac defect that may be congenital or acquired.It is characterized by abnormal dilatation of the aortic root due to a weakened elastic lamina at the junction of the annulus fibrosus and the aortic media.SoVAs are present in approximately 0.09%of the general population and comprise up to 3.5%of all congenital cardiac defects.It is usually found inci-dentally on cardiac imaging,with a higher incidence observed in the Western populations and a male-to-female ratio of 4:1.A transthoracic two-dimensional echocardiogram is the initial diagnostic test of choice,which may reveal the characteristic“windsock deformity”that clinches the diagnosis.Other imaging modalities,such as transesophageal echocardiography and cardiac computed tomography angiography,help provide more extensive details of the aneurysm and its adjacent structures.Management options for ruptured and unruptured SoVA include surgical repair or transcatheter closure,which serves as a game-changing development in treatment.This article aims to provide background information on the epidemiology,pathophysiology,diagnosis,and recent advan-cements over the past decade in the management of SoVAs.
基金The current study was supported by the National Key R&D Program of China[2018YFC1002600]Science and Technology Planning Project of Guangdong Province[Nos.2018B090944002,2019B020230003 and 2018KJY2017]+1 种基金Guangdong Peak Project of Guangdong Province[DFJH201802]the Shenzhen San-Ming Project.
文摘Background:Partial anomalous pulmonary venous connection(PAPVC)is frequently associated with atrial septal defect(ASD),especially sinus venosus defect(SVD).Although Waggstaffe described the pathology of SVDs in 1868,the exact anatomic features and the nature of SVD remains controversial.SVDs with no posterior atrial rim were observed in recent years.However,no studies suggested that absence of the residual posterior atrial septal tissue might be the key feature of SVD.The aims of this study were to investigate if absence of posterior rim of atrial septum played a crucial role in patients with SVD.Methods:From January 2011 to December 2019,256 children with PAPVC combined ASD and 878 children with isolated ASD who underwent corrective cardiac surgery were consecutively enrolled.Comprehensive review of preoperative transthoracic echocardiography,computed-tomography images and surgical findings were performed by experienced pediatric cardiologists.The subtypes of PAPVC,locations and types of ASD,and presence of posterior atrial rim of associated ASD were investigated.Results:PAPVC was right-sided in 244 children,left-sided in 6 children,and bilateral in 6 children.In PAPVC cases,ASD without posterior atrial rim existed in 226 SVD cases.ASD without posterior atrial septum only existed in cases with one or more right pulmonary veins returning to right atrium(RA)or to RA-superior vena cava junction.In cases with isolated ASD,there were 3 SVD,and the other 875 cases were secundum ASD.Conclusions:ASD without posterior atrial rims was associated with one or more right pulmonary veins returning to RA or RA-superior venous cava(SVC)junction.For SVD,the key feature is that the defect is in the posterior of the interatrial septum with no posterior septal rim,rather than adjacent to the SVC or to the inferior vena cava.
