Congenital heart defects are cardiac abnormalities present at birth. Approximately 8 out of every 1,000 infants are born with congenital heart disease. Some defects are mild and may not even be apparent during infancy or childhood. Others are life threatening and require surgery during infancy. The care of infants and children with congenital heart disease has been a monumental success.
With the advent of sophisticated diagnostic imaging, advanced medical therapy, catheter-based interventional procedures, and innovative surgery, over 90% of infants born with a congenital heart defect now survive well into adulthood.
The success of caring for infants and children with congenital heart disease has created new challenges. The unique anatomy, physiology, hemodynamics, and surgical procedures are unfamiliar to most physicians who care for adult patients. The nomenclature alone can be daunting and intimidating. Terms such as Pott’s shunt, Mustard procedure, restrictive and nonrestricive defects are generally unfamiliar to cardiologists who care for adult patients. In many cases patients who have undergone surgical repair of their defect are “fixed” but their hearts are not normal.
For example significant pulmonic regurgitation often follows repair of tetralogy of Fallot. This can lead to right heart dilatation, tricuspid regurgitation, arryhthmias, and shortness of breath decades after the operation. Significant mitral regurgitation may follow repair of an ostium primum atrial septal defect because of the associated cleft in the anterior leaflet of the mitral valve. In many cases the long-term sequelae following surgical or interventional catheter based repair are well recognized. In other cases the long-term sequelae are not yet characterized. Long-term follow-up studies will be needed to define the natural history after repair of both simple and complex defects. An awareness of associated defects is essential. For example, ascending aortic aneurysms...