This chapter focuses on infancy; however, discussions with parents concerning their newborn with a cardiac anomaly often quickly, and appropriately, move to the length and quality of life anticipated in later childhood and adulthood. It is important that parents are accurately advised of likely and potential long-term outcomes with current therapies by cardiologists or other practitioners up-to-date with recent advances and outcome findings. In general, those with common acyanotic anomalies such as uncomplicated septal defects or pulmonary valve stenosis, and most of those with cyanotic anomalies such as simple transposition of the great arteries now can anticipate an essentially near-normal life after appropriate intervention. Even with many complicated anomalies, most can expect to survive to at least mid-adulthood, although long-term survival is highly dependent on the specific diagnosis, with the highest mortality now generally occurring in infancy (see Table 33-2). With few exceptions, a cardiac operation or catheter intervention can lengthen and improve the quality of life of a child with heart disease. Even when the nature of the long-term management is unclear, care has proceeded with the conviction that childhood survival often allows yet to be planned later interventions, resulting from future progress in the field, to provide even longer and better survival. The palliative shunt operations of 20 to 40 years ago unexpectedly produced candidates for later Fontan procedures. The central principle continues to be “where there is life, there is hope.”

With reductions in mortality, and new generations of adults and older children with repaired and palliated cardiac anomalies, it has become evident that cardiac anomalies, and procedures utilized to treat them, sometimes have late residua and sequlae, including neurologic and cognitive morbidity not evident until preschool or school
age (6,7 and 8), and arrhythmias and ventricular dysfunction developing in adolescence and adulthood. Most difficult, and not yet fully delineated have been the neurologic and cognitive morbidity. These abnormalities appear rarely with common septal and valvar malformations, and tend to be more frequent and severe with more complicated anomalies and repairs. Cardiac, neurologic and cognitive outcomes after repair of septal defects have appeared generally normal (8). Although most children with complex anomalies and procedures, such as hypoplastic left heart syndrome and operative circulatory arrest, have neuro-cognitive outcome within the normal range, on average, they have slower development, lower IQ scores, and higher rates of learning disabilities and special needs than normal. Moreover, a significant number have major impairments (8). The etiologies are complex, and include possible genetic issues, coexistent brain anomaly, potentially diminished cerebral blood flow or oxygenation in utero with some anomalies (e.g., hypoplastic left heart syndrome), pre- and/or peri-operative low cardiac output, cyanosis (See color plate), thrombo-embolism, or intracerebral hemorrhage, and operative use of hypothermic circulatory arrest (6,7 and 8). The possibility that brain injury or other injury may be acquired prenatally, pretreatment or in the process of treatment should be understood.