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Repair of this anomaly is usually performed through a left fourth interspace thoracotomy. In rare cases, when the ductus is located in the right chest, a right fourth interspace incision is used. Division of the isolated ductus rather than ligation is used, except in premature infants or before cardiopulmonary bypass when working through a median sternotomy. Division is safer and should avoid ductal fracture or incomplete closure, which are risks of ligation.

Vascular ring operations are performed through a left third or fourth interspace thoracotomy and all anatomic structures are dissected. Angiography is rarely necessary to define vascular anatomy, although magnetic resonance imaging (MRI) or computerized tomogrpahy (CT) is used by many surgeons for this purpose. Vascular sling repair is also performed through a left thoracotomy, except in cases when associated intracardiac surgery is carried out. In this circumstance, a median sternotomy approach is used. Magnetic resonance imaging, CT, or angiography is helpful in defining this anomaly. An aortopexy is part of most vascular ring repairs because this pulls the pulsatile aorta away form the trachea. The aorta may continue to obstruct the trachea if left in its original position even after division of the ring.

Ectopia cortis is a dramatic anomaly even to the experienced heart surgeon. Infants born with this anomaly always have associated midline defects, including an omphalocele and sternal and/or rib deficiencies. Associated intracardiac problems are common. Initial surgery is aimed at covering the heart and abdominal wall defect with skin. Ideally, repair of intracardiac defects is delayed until a later age, as is chest wall reconstruction.