History and Physical Examination

Symptoms of interrupted aortic arch (IAA) in the neonate include tachypnea, poor feeding, and lethargy.

Recognizing interrupted aortic arch is difficult prior to reduction in the caliber of the ductus arteriosus. The hallmark thereafter is a mottled or grey appearance to the lower body, representing poor perfusion to that portion of the circulation located distal to the arch interruption. Also consider the following physical examination findings:

A difference in systolic blood pressure between the right arm and the lower extremities may or may not be present. Frequently, a lack of discrepancy in blood pressure is due to the profound reduction in cardiac performance. If the right subclavian artery is aberrant, no disparity occurs between the systolic blood pressure in the right arm and that in the lower extremities because the right subclavian origin is distal to the arch interruption.
Although a difference in oxygen saturation between the right arm and the lower body may occur in cases without an aberrant right subclavian artery, this can be quite subtle in cases of high pulmonary blood flow. In normally connected great arteries, the oxygen saturation is higher in the right arm than in the lower body. In interrupted aortic arch with transposition of the great arteries, the reverse occurs.

Although all of the above signs may represent interrupted aortic arch, aortic thrombosis may also present with such findings.^[48]

In addition, note the following:

The first heart sound is normal; the second heart sound is usually single.
A grade 2 or grade 3 systolic ejection murmur is usually present at the base, representing increased pulmonary blood flow. The mid diastolic rumble of flow-related mitral stenosis is uncommonly heard in neonates.
A holosystolic murmur of ventricular septal defect (VSD) may be heard in infants beyond the first few days of life, following a fall in pulmonary vascular resistance.
The liver is usually normal in size, but in neonates, this is principally a reflection of intravascular volume status.
Facial dysmorphism is frequently present because approximately 50% of patients with interrupted aortic arch have DiGeorge syndrome.

Differential Diagnoses

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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.

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Interrupted Aortic Arch Clinical Presentation

History and Physical Examination

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