An interrupted aortic arch is a rare congenital abnormality, and has always been repaired in childhood. Thus, any adult patient with such a history will need lifelong surveillance by an expert in congenital heart defects. Problems may occur at the site of the original surgical repair, or may be related to other congenital heart abnormalities that the patient was born with.
A ventricular septal defect (VSD) is a very common congenital anomaly. This means that there is a hole in the part of the heart that separates the two main pumping chambers, allowing blood to flow between the heart chambers. If the hole is small, blood flows from the left heart through the right heart to the lungs, but the amount is small enough that the hole does not need to be closed. Patients with such small VSDs have two major risks. The first is of infective endocarditis, an infection of the heart that can be fatal if not properly recognized and treated. The second is leakage of the aortic valve, which may eventually require surgical repair or replacement.
Patients with larger VSDs usually have had repair as children. Some of them will have so-called “patch leaks” in which there is a small residual amount of flow across the ventricular septum. Again, the major risk has to do with endocarditis. If the patient had a large VSD and has not been repaired by adult life, they will usually have high blood pressure in the lungs (pulmonary arterial hypertension) and will have developed Eisenmenger syndrome, a condition in which the blood pressure in the lung becomes so high that blue blood starts to mix with red blood causing the patient to develop a bluish tinge to the skin and other features.
VSDs can also occur in combination with other congenital heart abnormalities, or even be part of such an abnormality as is the case in tetralogy of Fallot, where a VSD is one of the four abnormalities making up the condition.
Learn more about the Adolescent and Adult Congenital Heart Disease Program.