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Ask the Expert!

Ask the Expert is an ongoing Q&A series between Pediatric Views readers and Children's specialists.

Long-term effects of congenital heart disease

Question: What is the impact of congenital heart diseases on development? For example, a baby who had surgery for transposition of the great arteries—is his heart condition going to be a chronic disorder? How so? Could a speech delay be attributed to the surgery or delay in getting surgery? What other problems could these children expect? Please provide a brief summary of the current research in the area of congenital heart diseases and specific symptoms to look for over the developmental years that could be related to them.

—Rose Russo, DSW, LMSW
Parent and social worker, NYC

Answer: The impact of congenital heart disease on development is an active area of research at Children's Hospital Boston. Problems with development in children with congenital heart disease can be related to innate or genetic factors; from circulation in fetal life; from the physiology of congenital heart disease itself (e.g., chronic severe cyanosis, poor feeding); or from the procedures, such as cardiac catheterization or cardiac surgery, used to treat congenital heart disease.

Fortunately, the majority of children with congenital heart disease have development that is within the normal range. However, IQ scores, achievement testing and gross and fine motor function and coordination for the individual child with congenital heart disease might be less good than it would be in a brother or sister unaffected by heart disease. More children with congenital heart disease need special services to treat learning disabilities or problems with speech, language or behavior than children without it. The likelihood of developmental problems is related to a child's type of congenital heart disease. In general, developmental outcomes after repair of simple forms of heart disease, such as atrial septal defects, are similar to those in the normal population, whereas developmental outcomes may be more affected in children with more complicated heart problems. Children with single ventricle have the greatest likelihood of having developmental challenges.

The development of children with D-transposition of the great arteries has been especially well studied in the Boston Circulatory Arrest Study, where we have followed the development of children with transposition born in the late 1980s and early 1990s. Speech has indeed been noted to be an area of difficulty. At age 8, IQ and achievement were normal in the group as a whole. However, many children with transposition had some problems in their motor function, visual-spatial skills, working memory, hypothesis generation and testing, attention and higher-order language skills. For some children, these problem areas contributed to difficulty with executive function and the ability to organize, implement and modify plans. Some children showed aspects of a non-verbal learning disability. A follow-up evaluation at age 16 years is ongoing.

We recommend that pediatricians and pediatric cardiologists incorporate neurodevelopmental surveillance in their routine care of children with congenital heart disease so that children can undergo detailed testing and appropriate interventions can be initiated promptly as problems are detected. In addition, families should be counseled to anticipate issues that may arise in school performance.

—Jane Newburger, MD, MPH
Associate Chief for Academic Affairs, Department of Cardiology,
Children's Hospital Boston

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