Approach Considerations

Evaluation of interrupted aortic arch (IAA) as an inpatient in an intensive care setting for diagnostic testing and surgical intervention is advised.

Intravenous prostaglandin E1 (alprostadil) (0.1 mcg/kg/min) is indicated promptly preoperatively to maintain patency of the ductus arteriosus. (No special medications are required postoperatively.)

The need for an arterial line and assisted ventilation can be judged best from the initial arterial blood gas (ABG) measurement.

Consultations

Consultations with the following specialists are important in the evaluation and management of patients with interrupted aortic arch:

Cardiothoracic surgeon
Pediatric cardiologist
Geneticist

Transfer

Transfer to an institution equipped to treat neonates with congenital heart defects may be required for further diagnostic evaluation and surgical intervention.

Diet and activity

No special diet is required.

No exercise restrictions are necessary in later childhood if coexistent subaortic (and/or aortic) hypoplasia has been sufficiently relieved in earlier childhood.

Surgical Care

The arch interruption itself is usually treated with side-to-side anastomosis, rather than with conduit interposition. If the subaortic region is of good size, the ventricular septal defect is usually closed with a patch at the same occasion.

Whereas the aortic arch interruption is usually treated with side-to-side or end-to-end anastomosis, some surgeons have used the subclavian artery to create an arch-shaped aorta and reported good results.^[51]Such options should be explored in future studies.

When a malalignment-type ventricular septal defect is present, the infundibular septum is not only misplaced but is also frequently hypoplastic. Hence, significant subaortic narrowing is frequently difficult to ameliorate with mere resection of infundibular septal muscle.

Two alternative approaches have been adopted: the Ross-Konno procedure and the Norwood-Rastelli procedure. Note the following:

In the Ross-Konno procedure, the aortic outflow region is directly enlarged (Konno) and the aortic valve is replaced with a pulmonary valve autograft (Ross).^[52]The coronary arterial ostia must be relocated to the autograft, and some sort of right ventricle–to–main pulmonary artery conduit is interposed (Ross). One relative contraindication to the Ross-Konno procedure is an unfavorable coronary artery pattern because this may well limit the efficacy of the Konno procedure.
In the Norwood-Rastelli procedure, an interventricular baffle allows left ventricular blood to reach not only the aortic outflow but also the pulmonary annulus (Rastelli), and the main pulmonary artery is transected.^[53]The proximal portion is anastomosed to the ascending aorta (Norwood) while the distal portion is connected to the right ventricle via a conduit (Rastelli).

One study reported the successful use of a regional cerebral perfusion technique to correct interrupted aortic arch.^[54]

Newer surgical techniques to maintain native tissue continuity^[55]may help prevention of re-narrowing.

Following surgical reconstruction, echocardiographic and Doppler evaluation of the adequacy of the repair should be performed.

Long-Term Monitoring

During follow-up, re-intervention may be required in nearly 30% of patients.^[56]Such recurrences may be effectively treated either by balloon angioplasty or stent placement, depending upon the patient’s age and anatomy.^{[57, 58]}Because of advances in neonatal, medical, anesthetic, and surgical care of these sick babies, the prognosis has improved during the last two decades.^[59]

Medication

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Media Gallery

Interrupted Aortic Arch. Suprasternal echocardiographic identification of interrupted aortic arch Type B. Upper left: Frontal (coronal) view showing the takeoffs of the innominate artery (InnA) and left common carotid artery (LCCA). No connection is seen with the distal aorta. Upper right: Slightly more dorsal (posterior) frontal view showing the main pulmonary artery (MPA), large left-sided patent ductus arteriosus (PDA), and left-sided upper descending aorta (DescAo). The ascending aorta (AscAo) is not in discernible continuity with the descending aorta. Lower left: Left oblique view showing the LCCA takeoff and no discernible aortic arch. Lower right: sagittal view showing the origin of the left subclavian artery (LSCA) from the DescAo. Other Abbreviations: InnV= innominate vein; l=left; LPA=left pulmonary artery; p,l = posterior and leftward; s=superior; SVC=superior vena cava.
Interrupted Aortic Arch. Section A depicts a subcostal frontal echocardiogram of interrupted aortic arch (IAA) type B with transposition of the great arteries. Section B shows a high parasternal echocardiogram showing that the innominate artery (Inn A) and left common carotid artery (LCCA) arise from the ascending aorta (a ao). In section C, the left subclavian artery (LSCA) arises from the descending aorta (desc ao), which is perfused by the ductus arteriosus.
Interrupted Aortic Arch. This is the suprasternal sagittal ultrasonographic view of the patient shown in the previous image. Arch continuity has now been restored by a side-to-side anastomosis. Abbreviations are as follows: a ao = ascending aorta and desc ao = descending aorta.