Pediatric Surgery for Unroofed Coronary Sinus Treatment & Management

  • Author: Samuel Weinstein, MD; Chief Editor: John Kupferschmid, MD   more...
 
Updated: Aug 16, 2011
 

Medical Therapy

While in most cases the defect is associated with a more complex congenital heart lesion excluding an interventional approach, device closure has been proposed as a possible nonsurgical option.[2]

Bernado et al reported device closure of a 9.5-year-old, 31-kg child with a coronary sinus ASD and persistent LSVC, with a catheter placed within the LSVC to assist placement.[15] Torreset al, in 2007, used a similar approach to close an unroofed coronary sinus with a covered stent in a symptomatic 7-month-old infant.[16] Consider how to handle the LSVC, when present and draining to the coronary sinus, either with or without a connection to the innominate vein. Closure of a resulting right-to-left shunt is recommended, certainly if the vein is sizeable. Depending on the drainage pattern, the LSVC can be occluded with interventional techniques, as well, but surgical management is probably the most appropriate option when the vessel is large, needs reimplanting, or there are multiple perforations in the coronary sinus.[15, 16]

Other contraindications to device closure include inadequate margins to seat the device, large defects, and infants who are too small to accommodate the delivery system.[17]

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Surgical Therapy

The goals of surgical therapy include the separation of systemic venous drainage from pulmonary venous drainage, completion of the atrial septum, and management of the left superior vena cava (LSVC), if present. This lesion can be repaired with systemic venous cannulation of the LSVC, right superior vena cava (RSVC), and inferior vena cava (IVC); right atriotomy and cardiac arrest; or with circulatory arrest if the patient is an infant or neonate.

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Intraoperative Details

Repair of a coronary sinus atrial septal defect (ASD) without a LSVC can be accomplished with a roofing procedure.[18] After cardiac arrest and right atriotomy, the defect is examined through the atrial septum. If the atrial septum is intact, the fossa ovalis is incised for exposure. When the tissues are redundant, the defect can be closed primarily.

Another approach is to use a cannula or obturator introduced into the coronary sinus toward the left atrium to stent the area while a roof of pericardial tissue is created, as depicted below.[19] The patch runs from a location between the left atrial (LA) appendage and upper pulmonary veins, continuing inferiorly toward the mitral valve, and terminating near the right atrium and right atrial orifice of the coronary sinus. Bites are placed superficially toward the right side of the atrium to avoid injury to conduction tissue.[19] The atrial septum is then repaired primarily or with a patch.

3A: A venous cannula stent opens the coronary sinu3A: A venous cannula stent opens the coronary sinus while sutures are placed. 3B: A pericardial patch is used in a roofing procedure.

If an LSVC is present, the repair can be performed in a similar manner, or with an intra-atrial baffle to help avoid narrowing of pulmonary veins or obstruction of the mitral valve (see image below).[19, 20, 21] After cardiac arrest and right atriotomy, the atrial septum is first incised for exposure if the ASD is small or nonexistent. A patch of pericardium serves as a baffle, separating the orifice of the left SVC from the pulmonary veins. This allows the pulmonary venous blood to drain toward the mitral valve, whereas the systemic venous blood drains directly to the right atrium. When a common atrium is present or when a coronary sinus is absent, landmarks for the conduction system are not available to the surgeon.[19] To remedy this and to avoid the area of conduction, de Leval has described placement of the sutures along the base of the septal leaflet of the tricuspid valve and then out onto the right atrial wall beyond the tricuspid valve annulus.

A pericardial patch is used to baffle the pulmonarA 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 to drain to the right-sided atrium.

In each case, the left SVC is addressed on its own merits. If it is small and a bridging innominate vein is present, the left SVC can be ligated. Others have recommended ligation when the occlusion pressure is less than 16 mm Hg.[21] If it is large and is the sole source of venous drainage for the upper extremities and head, an intraatrial baffle can be considered. Still, others have described extracardial techniques (ie, reimplantation of the LSVC into the right atrium)[22, 19] or RSVC when a bridging vein is absent.[23] Bidirectional left superior cavopulmonary anastomosis is also described as a surgical option for patients with low pulmonary pressures.[22]

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Postoperative Details

Complications are similar to other atrial level repairs. Risks of residual left-to-right or right to left shunting and risk of heart block or sinus node dysfunction are most commonly encountered. However, these ASDs are usually seen with other lesions, which may have more severe complications that take precedence.

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Follow-up

For patient education resources, see the Heart Center, as well as Ventricular Septal Defect and Tetralogy of Fallot.

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Complications

Complications are similar to other atrial level repairs. Risks of residual left-to-right shunting and risk of heart block or sinus node dysfunction are most commonly encountered. However, these atrial septal defects (ASDs) are usually seen with other lesions, which may have more severe complications that take precedence.

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Outcome and Prognosis

Outcome and prognosis for an isolated coronary sinus ASD are excellent, but the more serious lesions that can be associated may alter the overall outcome for a patient.

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Future and Controversies

Results of surgical repair are closely related to the complexity of the associated lesions also being operated on. An unroofed coronary sinus should be suspected whenever a left superior vena cava (LSVC) is encountered on echocardiogram. Failure to repair these defects results in excessive blood return to the heart and a residual right-to-left shunt.

Debate on this topic is negligible because this atrial septal defect (ASD) is a very rare lesion (most centers may see 1-2 a year). A minimally invasive approach could be discussed, but these ASDs are observed in children who usually have other more complex problems; thus, a minimally invasive approach often does not apply.

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Contributor Information and Disclosures
Author

Samuel Weinstein, MD  Associate Professor, Albert Einstein College of Medicine; Director, Department of Pediatric Cardiothoracic Surgery, The Children's Hospital at Montefiore

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

Disclosure: Nothing to disclose.

Coauthor(s)

Avi A Harari  Trakya University Faculty of Medicine

Disclosure: Nothing to disclose.

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.

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, and Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Daniel S Schwartz, MD, FACS  Assistant Clinical Professor of Cardiothoracic Surgery, Mount Sinai School of Medicine; Chief of Thoracic Surgery, Huntington Hospital

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

Disclosure: Nothing to disclose.

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, and Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Daniel Rauch, MD, FAAP  Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine

Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine

Disclosure: Baxter Honoraria Consulting

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, and Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

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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.
3A: A venous cannula stent opens the coronary sinus while sutures are placed. 3B: A pericardial patch is used in a roofing procedure.
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 to drain to the right-sided atrium.
 
 
 
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