Abstract

BackgroundInterrupted aortic arch (IAA) is a congenital malformation of the aortic arch which involves 3 out of 1 million live births. This congenital anomaly rarely occurs as an isolated lesion and is often associated with other intracardiac malformations, most commonly ventricular septal defect and patent ductus arteriosus (PDA). The diagnosis and surgical treatment of aortic interruption is usually challenging and may require multiple operations throughout the patient’s life.Case presentationThis case represents a neonate with interrupted aortic arch (type B) and a very long segment of descending aorta hypoplasia and complex anatomy. The patient escaped early diagnosis at birth and presented few days later by a picture that mimicked severe sepsis and shock.His aortic anatomy was very complex and he was treated with long extra-anatomical aortic interposition graft.ConclusionAortic interruption is a rare congenital anomaly and is considered an extreme form of aortic coarctation.It sometimes escapes early diagnosis due to the presence of patent ductus arteriosus and present later with shock and lactic acidosis. Sometimes the aortic anatomy is very complex and requires unusual surgical techniques for its repair.

Highlights

  • ConclusionAortic interruption is a rare congenital anomaly and is considered an extreme form of aortic coarctation

  • Interrupted aortic arch (IAA) is a congenital malformation of the aortic arch which involves 3 out of 1 million live births

  • Interrupted aortic arch (IAA) is a rare congenital anomaly, and it is considered an extreme form of aortic coarctation

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Summary

Conclusion

Aortic interruption may escape diagnosis at birth and present later in a picture that may be confusing with septic shock and lactic acidosis, high index of suspicion is mandatory for diagnosis.

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