Surgical Considerations for Congenital Heart Disease

General Considerations

Many congenital heart defects are fatal or can lead to long-term complications or a shortened life expectancy if not corrected surgically. Due to advances in congenital heart surgery, anesthesia, critical care, and diagnostic imaging, many defects can be treated effectively with consistently improving results. Although some congenital heart defects are effectively and permanently treated with one operation, many require subsequent interventions and long-term follow-up, including the use of different imaging modalities.

Surgical Approaches

Surgery for congenital heart disease is most commonly performed via a median sternotomy with an anterior midline thoracic incision. After exposure of the sternum, a sternal saw is used to split the sternum and a retractor is placed to expose the operative field within which the pericardial sac is contained ( Fig. 69.1 ). This incision offers excellent access to all of the structures of the heart, which is useful when multiple lesions are present or if a complicated repair must be performed.

Less invasive incisions such as a partial median sternotomy or a minithoracotomy can also be utilized. In the case of a partial median sternotomy, the skin incision is kept small and only the upper or lower sternum is divided ( e-Fig. 69.2 ). Depending on the necessary exposure, thoracotomy incisions can be anterior, lateral, or posterior ( e-Fig. 69.3 ) and, in the current era, these incisions are performed by dividing the intercostal muscles and leaving the ribs intact.

The type of lesion being repaired determines the choice of surgical incision. Straightforward repair of extracardiac lesions such as aortic coarctation, vascular rings, or PDA generally can be performed through a left thoracotomy. With refined techniques, even intracardiac repairs requiring cardiopulmonary bypass (CPB) such as an atrial septal defect (ASD) or mitral, tricuspid, and aortic valve operations can be done through minimally invasive incisions. In addition, interest has developed in performing relatively straightforward operations, including some heart valve surgery, using thoracoscopic or robotic techniques. In these cases, several very small incisions and specially designed instruments are used to perform the procedure ( e-Fig. 69.4 ). Advantages touted include an improved cosmetic result and potentially shorter recovery times inside and outside the hospital.

For most complex congenital heart defects, such as repair of an AV septal defect or a cyanotic defect, a median sternotomy is used. This allows better exposure of both intracardiac and extracardiac structures that need to be repaired. For example, the repair of TOF might involve incisions in both the right atrium and the pulmonary artery to close the ventricular septal defect (VSD) and relieve the right ventricular outflow obstruction. In some cases in which a very small pulmonary annulus or significant infundibular stenosis is present, the incision might even need to be extended to the right ventricular cavity ( Fig. 69.5 ).

The choice of surgical approach also is important in the case of staged operations. Lesions that commonly require multiple operations include HLHS, TOF, and truncus arteriosus. A patient with HLHS generally will undergo three operations for complete palliation, and the need for a repeat sternotomy carries both operative and radiographic implications. Imaging of the chest can help with operative planning because redo median sternotomy incisions carry a risk of damage to the thoracic contents as a result of the formation of postoperative adhesions. For example, the aorta or other structures can be found very close to the sternum ( e-Fig. 69.6 ) and might be damaged upon entry to the chest during the sternotomy. This sometimes necessitates the emergent institution of CPB or alternative, peripheral cannulation (discussed later).

Other scenarios influencing operative planning and the choice of incision include the presence of multiple lesions or when a palliative procedure, instead of complete repair, is indicated. For example, many congenital heart defects can occur together, such as coarctation of the aorta and VSD. In this case, a surgical procedure that might have been accomplished through a thoracotomy incision for isolated coarctation ( e-Fig. 69.7 ) would necessitate a median sternotomy to address both lesions.

The incision used for surgery has a variety of clinical and radiographic consequences. Patients who have had median sternotomy incisions often will have steel wires re-approximating the sternum, although in children, sutures that are not radio-opaque often are used. Occasionally, chest wall deformities may develop in children who have sternotomy incisions as the children grow. Children who have had surgery for congenital heart disease also are at higher risk for developing scoliosis than the general population ( e-Fig. 69.8 ). Thoracotomy incisions can produce distortion of the ribs on the side that has been operated upon and also can lead to scoliosis. In the current era, ribs usually are not cut or sectioned during thoracotomy procedures, but sometimes inadvertent rib fractures can occur, which would be noted on the postoperative chest radiograph.