Interrupted Aortic Arch

Excerpt

Congenital heart disease is an abnormal formation of the heart or blood vessels next to the heart. It has an incidence of 8 cases of every 1000 live birth worldwide. In the United States, congenital heart disease affects 1% of births (40,000) per year, of which 25% have critical Congenital heart disease. In the U.S. the congenital heart disease represents approximately 4.5% of all neonatal deaths. The survival rate of patients with congenital heart disease will depend on the severity, time of diagnosis, and treatment. Approximately 97% of babies born with a non-critical congenital heart disease have a life expectancy of one year of age, and approximately 95% are expected to live around 18 years of age.

A rare type of congenital heart disease is an interrupted aortic arch (IAA), which affects approximately 1.5% of congenital heart disease patients. Interrupted aortic arch is an anomaly that can be considered the most severe form of aortic coarctation. In an IAA, there is an anatomical and luminal disruption between the ascending and descending aorta. IAA is a ductus dependent lesion since this is the only way the blood flow can travel to places distal to the disruption. There is posterior malalignment of the conal septum additional to the interrupted aortic arch, producing a ventricular septal defect as an associated lesion. This lesion is present is approximately 73% of all cases. Due to this malalignment, there could be left ventricular outflow tract obstruction. Besides a ventricular septal defect, IAA can be associated with other more complicated cardiac anomalies; for example, transposition of the great arteries, truncus arteriosus, aortopulmonary window, single ventricle, aortic valve atresia, right-sided ductus, and double-outlet right ventricle.