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Aortic Arch Anomalies 

What are aortic arch anomalies?

Aortic arch anomalies are a type of congenital heart defect. The aorta arises from the left ventricle and carries oxygenated blood from the heart to the body. There are many different variations of aortic arch anomalies.

Coarctation of the aorta means that a part of the aorta is narrowed, which causes the left ventricle to work harder. This heart defect causes decreased blood flow to the lower body. In mild cases, children may not show symptoms or be diagnosed until later in life, but in more severe cases, this condition can immediately cause difficulty breathing or poor feeding.

Interrupted aortic arch, a very rare defect, is when the aorta is not fully connected. Often there is a ventricular septal defect (VSD) or hole between the bottom chambers of the heart. Interrupted aortic arch is often associated with DiGeorge syndrome, a chromosomal abnormality.

Without treatment, these anomalies can be fatal within the first few days of life. But with early diagnosis and treatment, the prognosis is quite good.

How are aortic arch anomalies diagnosed and monitored during pregnancy?

Aortic arch anomalies can be difficult to diagnose during pregnancy. If your doctor suspects a fetal heart anomaly after reviewing your routine ultrasound, he or she may request a fetal echocardiogram (echo), an ultrasound of the fetus’s heart. This safe, noninvasive test shows the structure of the heart and how it is functioning, which can help us confirm the diagnosis of an aortic arch anomaly and discuss possible options for treatment after delivery. Our Fetal Heart Team will monitor the baby closely for the remainder of the pregnancy.

How does an aortic arch anomaly affect delivery?

Most babies with aortic arch anomalies can be delivered vaginally at term, unless there are obstetric indications for another mode of delivery. It is best for these babies to be delivered at a medical center where there is access to a team of pediatric cardiologists and neonatologists, along with an established Neonatal Intensive Care Unit (NICU).

After delivery, the baby will need prostaglandin (PGE), an intravenous medication that keeps the patent ductus arteriosus (a normal connection present in babies in the womb) open. The patent ductus arteriosus (PDA) will allow blood to flow beyond the coarctation. The baby will be monitored in the Neonatal Intensive Care Unit (NICU). In severe cases, surgery is usually performed in the first week of life.

What are the surgeries for aortic arch anomalies and what is the long-term prognosis?

There are a few options for treatment depending on the defect and its severity:

  • Surgery: In newborns with coarctation, as well as for interrupted aortic arch, the pediatric cardiothoracic surgeon will need to perform surgery to repair the defect. The surgeon will reconnect an interrupted arch, or remove the narrowed portion of the coarctation.
  • Cardiac Catheterization: In this surgery to repair a coarctation, the pediatric cardiologist will insert a catheter (a flexible tube) through a blood vessel in the in the umbilical cord or groin and into the heart, then stretch the narrowed area open with a small balloon, and finally insert a stent into the narrowed area to make sure it doesn’t narrow over time. This is typically reserved for older children and adults.

With early treatment, the long-term prognosis is usually good. The child will need regular follow-up visits with a cardiologist throughout their lifespan to be sure there is not recurrent narrowing or abnormal enlargement of the repaired area.