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Hypoplastic or Interrupted Aortic Arch

The aorta is the main conduit of blood flow from the heart to the rest of the body and is thus a very important structure in the human body. A condition/heart defect similar to coarctation of the aorta, a hypoplastic aortic arch is said to be present when there is a blockage in a certain location in the aorta. This makes alignment with the ascending aorta and descending aorta difficult. Unlike coarctation, a larger portion of the aorta is blocked and thus the condition/birth defect is more severe, and interrupted aortic arch repair requires a longer and more difficult operation to correct. Babies with this condition might have other heart conditions, including a ventricular septal defect, patent ductus arteriousus, and problems with the left subclavian artery.

Sometimes the blockage and lack of proper blood flow is so bad for the congenital heart disease that there is no flow through it at all; this is the most severe form and is thus known as “interruption.” In either case, newborn babies are affected by this immediately after birth and rarely can be discharged from the hospital without having surgery. Before the advent of safe heart surgery, almost all of these patients would die from this blockage in the newborn or infant period.

Interrupted Aortic Arch Repair/Surgery

Fortunately, all patients with congenital heart disease can now be offered surgery to help improve oxygenated blood and most go on to live normal, healthy and active lives. Surgery for this condition/heart defect, however, remains difficult and must often be performed by heart surgeons specifically trained and experienced to operate on small, newborn babies and infants. Decision-making for the exact type of heart surgery (particularly for interruption) can be difficult and require advanced imaging and experienced cardiologists. The majority of these patients also have a VSD and this also needs to be addressed at surgery. The operation requires an incision and cardiopulmonary bypass and the VSD, if present, would be patched closed in the standard fashion. The most common preferred technique for repair of a hypoplastic or interrupted arch is patch repair with tissue from a donor human being, although some other techniques are sometimes used. Sometimes, it even takes more than one operation to repair hypoplastic or interrupted aortic arch with VSD, although in the current era most patients only require one operation.

At Rady Children’s Hospital-San Diego, we have been treating hypoplastic or interrupted aortic arch for more than 30 years and have experience with all forms of treatment for the birth defect. These operations for patients can be difficult and are still considered a higher-risk operation even in today’s era. From 2009-13 alone, we have repaired 37 consecutive patients with hypoplastic or interrupted aortic arch and VSD with over a 97 percent survival. During this same time period, many more patients had hypoplastic or interrupted arches repaired as part of even more complicated and riskier operations with similar success rates. In these cases, the particular risk for you or your child’s precise condition can be more thoroughly discussed during the preoperative consultation with the surgeon at our heart center.

When patients have had surgical repairs of aortic blockages (which includes coarctation, hypoplastic or interrupted aortic arch either by themselves or as part of larger operations), sometimes they return to us with some recurrent blockage near the surgical repair site. This is relatively common and if detected early, is not usually a cause of life-threatening concern. In the current era, most patients can have this treated without even making an incision using a catheter-based treatment (balloons and stents placed through an IV). In rare cases, redo surgery may need to be discussed as an option to help improve oxygenated blood, and if this is the case, we can discuss the options and results as they pertain to you or your child’s particular condition.