Pediatric Surgery for Unroofed Coronary Sinus Treatment & Management

Updated: Jan 04, 2016
  • Author: Sergey G Toshinskiy; Chief Editor: John Kupferschmid, MD  more...
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Treatment

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 recently as a possible nonsurgical option. [5]

Bernado et al reported device closure of a 9.5-year-old, 31-kg child with a coronary sinus atrial septal defect (ASD) and persistent left superior vena cava (PLSVC), through a catheter placed within the left superior vena cava (LSVC). [23] Torreset et al (2007) used a similar approach to close an unroofed coronary sinus (UCS) with a covered stent in a symptomatic 7-month-old infant. [24] Santoro et al (2013) reported transcatheter closure of a partial UCS, where the defect was distal in the coronary sinus, via implantation of a standard occluding device in an 8-year-old child. [25]

Depending on systemic venous drainage patterns, the LSVC can be occluded with an interventional technique or can remain draining systemic venous blood to the left atrium. In this situation, as well as when there are multiple fenestrations, surgical management may be more appropriate. [23, 24] 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. [26]

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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 PLSVC, if present. This lesion can be repaired with standard bypass techniques, and systemic venous cannulation of the LSVC may be possible if the patient is not an infant or a neonate. As well, the PLSVC can be temporarily occluded if the period is brief and will aid visualization of the repair.

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Intraoperative Details

Repair of a coronary sinus ASD without a PLSVC can be accomplished with a roofing procedure. [27] After cardiac arrest and right atriotomy, the defect is examined. If the atrial septum is intact, the fossa ovalis can be incised for increased exposure. Intraoperatively, a probe can be passed through the coronary sinus and visualized through an ASD to confirm the presence of an UCS. [2] The coronary sinus is unroofed into the left atrium, and a patch is placed to close the atrial septum and coronary sinus to the left side (see the image below).

A venous cannula stent opens the coronary sinus wh A venous cannula stent opens the coronary sinus while sutures are placed.

When the tissues are redundant, the defect can be closed primarily. When a PLSVC is not present, the amount of cyanosis from coronary blood return is incidental. After closure of the ASD, the presence of a residual leak can be determined by ventilating the lung and looking for evidence of pulmonary venous return from the coronary sinus. [2]

In each case of a PLSVC, the vessel is addressed on its own merits. If it is small and a bridging innominate vein is present, the PLSVC can be ligated. Others have recommended ligation when the occlusion pressure is less than 16 mm Hg. [28] If it is large and is the sole source of venous drainage for the upper extremities and head, an intra-atrial baffle can be considered (see the image below). Still, others have described extracardiac techniques (ie, reimplantation of the LSVC into the right atrium) [29, 30] or right superior vena cava (RSVC) when a bridging vein is absent. [31, 32]

A pericardial patch is used to baffle the pulmonar 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.

An intra-atrial baffle, however, can potentially result in pulmonary venous obstruction or mitral valve inflow obstruction. [2, 28, 29, 33] Bidirectional left superior cavopulmonary anastomosis is also described as a surgical option for patients with low pulmonary pressures. [30]

When a common atrium is present or when a coronary sinus is absent, landmarks for the conduction system are not available to the surgeon. [29] To remedy this and to avoid the area of conduction, de Leval has described the placement of 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. [29]

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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 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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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 the patient.

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Future and Controversies

Results of surgical repair are closely related to the complexity of the associated lesions. A UCS should be suspected whenever a PLSVC is encountered on echocardiogram.

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