Imaging Studies

Echocardiography (3- or 2-dimensional) can reveal an unroofed coronary sinus (UCS) and/or persistent left superior vena cava (PLSVC). However, the diagnosis can be missed, even by those with experience, which can lead to a diagnosis made at the time of surgery.^[5]Xie et al have estimated the diagnostic accuracy of transthoracic and contrast echocardiography for UCS at only 65%.^[19]Injection of agitated saline into the left antecubital vein during echocardiography can confirm the diagnosis of a PLSVC. Bubbles are seen in the coronary sinus before appearing in the right heart.^[7]Echocardiography findings can be inconclusive because deep structures such as the coronary sinus are not always well delineated.

When the echocardiography findings are inconclusive, further imaging may be necessary. Newer generations of multidetector CT (MDCT) scanners can offer 3-dimensional reconstructions of cardiac structures and require less time, making this imaging modality a favorable choice for uncooperative patients or pediatric patients.^{[13, 20]}MDCT scanning has high spatial and temporal resolutions, multiplanar reconstruction capabilities, and a wide field of view, making it an excellent tool for the detection and characterization of defects and for the identification of associated anomalies of the heart and pulmonary vasculature.^[21]

Cardiac MRI provides high contrast of the internal cardiac structures without the need for injection of contrast media.^[11]).

Cardiac catheterization can also confirm the presence of a PLSVC associated with an UCS atrial septal defect (ASD) via selective angiography of the systemic veins, coronary sinus, or left atrium.^{[5, 22]}

Treatment & Management

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Media Gallery

Embryologic remnant resulting in persistent left superior vena cava (PLSVC). The dotted line illustrates the degenerated left anterior cardinal vein. This posterior view illustrates how the left superior vena cava drains into the coronary sinus.
The fenestration is seen draining between the left atrial appendage and the pulmonary veins.
A venous cannula stent opens the coronary sinus while sutures are placed.
A pericardial patch is used to baffle the pulmonary veins directly toward the mitral valve. This approach allows unobstructed flow from the orifice of the left superior vena cava (LSVC) to drain to the right-sided atrium.

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Author

Sergey G Toshinskiy State University of New York Upstate Medical University

Disclosure: Nothing to disclose.

Coauthor(s)

Robert E Michler, MD Samuel I Belkin Chair, Professor and Chairman, Department of Surgery, Professor and Chairman, Department of Cardiothoracic Surgery, Albert Einstein College of Medicine; Surgeon-in-Chief, Chairman, Department of Surgery and Chairman, Department of Cardiothoracic Surgery, Co-Director Montefiore-Einstein Heart Center, Montefiore Medical Center

Robert E Michler, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Medical Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, International Society for Heart and Lung Transplantation, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Samuel Weinstein, MD, MBA Executive Vice President and Chief Medical Officer, SpecialtyCare

Samuel Weinstein, MD, MBA is a member of the following medical societies: American College of Surgeons, American Heart Association, American Medical Association, Ohio State Medical Association, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John Myers, MD Director, Pediatric and Congenital Cardiovascular Surgery, Departments of Surgery and Pediatrics, Professor, Penn State Children's Hospital, Milton S Hershey Medical Center

John Myers, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, American Medical Association, Congenital Heart Surgeons Society, Pennsylvania Medical Society, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Chief Editor

John Kupferschmid, MD Director of Congenital Heart Surgery, Department of Surgery, Methodist Children's Hospital at San Antonio

John Kupferschmid, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, Society of Thoracic Surgeons, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Additional Contributors

Daniel S Schwartz, MD, MBA, FACS Medical Director of Thoracic Oncology, St Catherine of Siena Medical Center, Catholic Health Services

Daniel S Schwartz, MD, MBA, FACS is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, Society of Thoracic Surgeons, Western Thoracic Surgical Association

Disclosure: Nothing to disclose.

Acknowledgements

Suzanne Courtwright, MS, CPNP-AC Pediatric Nurse Practitioner, Department of Pediatric Cardiothoracic Surgery and Adult Congenital Heart Surgery, Montefiore Medical Center

Suzanne Courtwright, MS, CPNP-AC is a member of the following medical societies: American Academy of Pediatrics, American Nurses Association, Sigma Theta Tau International, Society of Critical Care Medicine, and Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Avi A Harari Trakya University Faculty of Medicine

Disclosure: Nothing to disclose.