Interrupted Aortic Arch (IAA)

Jeremy L. Herrmann, MD, John W. Brown, MD

Key Points

  • What is IAA?: Interrupted Aortic Arch (IAA) is a rare birth defect where the aorta is not fully connected, stopping blood flow from the heart to the body. It is classified into three types based on where the interruption occurs.
  • Commonly Associated Defects: Most patients with IAA also have a hole between the heart’s ventricles (VSD) and may have other heart or genetic problems, such as DiGeorge syndrome.
  • How It Presents and Is Diagnosed: Babies with IAA rely on a vessel called the ductus arteriosus for blood flow. When this closes after birth, they can quickly become very sick. Echocardiography is the main tool for diagnosis.
  • Prognosis Without Treatment: Without surgery, IAA is almost always fatal within the first weeks of life. Early medical and surgical advances have greatly improved survival.
  • Surgical Repair: Surgery reconnects the aorta and fixes any other heart defects. There are different surgical approaches, and the choice depends on the patient’s anatomy and associated conditions.

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