Abstract
Introduction: To present a rare occurrence of coronary sinus atrial septal defect (ASD) associated with total anomalous pulmonary venous connection (TAPVC). Case Report: A 16-year-old girl was diagnosed with features of an atrial septal defect (ASD) by transthoracic echocardiography and the absence of PLSVC (persistent left superior vena cava) connection to coronary sinus was confirmed by saline contrast echocardiography (“bubble study”). Discussion: The cause of the dilated coronary sinus was due to total anomalous pulmonary venous connection. Conclusion: Coronary sinus atrial septal defect (ASD) is a rare interatrial shunt that is commonly associated with a persistent left superior vena cava (PLSVC) and occurs in less than 1% of cases of ASDs. It is characterized by the absence of at least a portion of the common wall that separates the coronary sinus and left atrium. Isolated defects are associated with low rate of morbidity and mortality.
Highlights
IntroductionCoronary sinus is usually not dilated on echocardiography. Anomalous venous drainage into the coronary sinus either directly or through a persistent left superior vena cava (PLSVC) is one of the etiologies for a dilated coronary sinus [1]
To present a rare occurrence of coronary sinus atrial septal defect (ASD) associated with total anomalous pulmonary venous connection (TAPVC)
Case Report: A 16-year-old girl was diagnosed with features of an atrial septal defect (ASD) by transthoracic echocardiography and the absence of persistent left superior vena cava (PLSVC) connection to coronary sinus was confirmed by saline contrast echocardiography (“bubble study”)
Summary
Coronary sinus is usually not dilated on echocardiography. Anomalous venous drainage into the coronary sinus either directly or through a persistent left superior vena cava (PLSVC) is one of the etiologies for a dilated coronary sinus [1]. Unroofed coronary sinus was first described by Raghib and colleagues in 1965 with the findings of left SVC drains into the left atrium, an ASD (which lies in the postero-inferior angle of the atrial septum and above the postero-medial commissure of the mitral valve and separated from the mitral valve by a small amount of septal tissue—the defect is considered as a true defect of a specific tissue) and absent coronary sinus which are collectively termed as “Raghib complex or syndrome” [5]. Total anomalous pulmonary venous connection (TAPVC), a very unusual malformation of the heart was published by Philosophical Transactions of the Royal Society in London in 1798 [6] It represents 2% of all congenital heart defects and 100 cases of anomalous pulmonary venous connections were reported in 1942 [7]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
