In Augusts' edition of Circulation. the American Heart Association and the American Academy of Pediatrics published a statement about the potential for neurodevelopmental disabilities and the need for developmental screening and follow-up in children with congenital heart disease (CHD).  The conclusions and recommendations were straightforward:

"Children with CHD are at increased risk of developmental disorder or disabilities or developmental delay.  Periodic developmental surveillance, screening, evaluation, and reevaluation throughout childhood may enhance identification of significant deficits, allowing for appropriate therapies and education to enhance later academic, behavioral, psychosocial, and adaptive functioning." (AHA, 2012).

Since the 1940s, treatment of infants with CHD has been one of the major success stories of pediatrics.  Once uniformly fatal, most forms of CHD are now routinely treatable with high survival rates.  This has led some pediatricians and parents to assume that almost all children with CHD not only survive but thrive.  But clinicians providing long-term follow-up of these children have long known that many are left with significant cognitive and motor developmental delays among infants having CHD.  The breadth of delays later was reported from international centers and included abnormal auditory brainstem responses, language development and behavioral concerns.

Many factors contribute to these problems, including associated chromosomal and non-chromosomal genetic conditions, premature gestation, low birth weight, degree and direction of in utero fetal blood-flow during development (especially in the heart, lungs and brain), degree of postnatal hypoxia and acidemia, procedural complications, and operative or by-pass pump complications.  A recent Swiss report reveals problems at school-age in 117 children with CHD not having any genetic comorbidity, citing cerebral palsy in 10% and poor neuromotor performance in up to 20%. (J Thorac Cardiovasc Surg 2012)

CHD occurs in about 1% of live births.  There are about 400,000 babies born with CHD in the United States each year, about 1/3 of which will require catheter-based or surgical intervention.  In 2012, babies born with CHD often have their condition diagnosed prenatally by obstetrical ultrasound or by specific fetal echocardiography performed in a regional perinatal center or at a children's hospital.  Pregnant women with an antenatal diagnosis of CHD need careful counseling about both survival and long term outcome.  Babies born with CHD need not only excellent surgery and intensive care but also careful follow-up for neurodevelopmental problems.  Unfortunately, that does not  routinely happen.  To do it properly will take resources in a system that is already under strain.  These children need a medical home, comprehensive screening, and some will require early-intervention services.  Many of these services will need to be funded by Medicaid or SCHIP programs that are already straining to provide mandated coverage.  As with follow-up programs for NICU survivors, the system is likely to be uneven and underfunded.

From an ethical perspective, these facts raise concerns about justice and resource allocation, but also of discerning the right and good thing for pediatricians to do.  In particular, when and by whom should pregnant women who are facing a decision about pregnancy termination, perinatal palliative or comfort care, or intensive care be provided information about the likelihood of developmental problems down the road?  How comprehensively should all of the options be explained, different goals discussed, and different choices permitted?  What information is necessary, sufficient, or obligatory as we aspire to practice 'good medicine'?  How honest can we be about long-term outcomes without negatively impacting parental hopefulness?

These are questions that have not often been asked in the context of CHD.  This new statement makes them unavoidable.

References and suggested reading:

1.  Marino BS, et al.  Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management. E-pub ahead of print in Circulation 2012;126:00
2.  von Rhein M, et al.  Risk factors for neurofevelopmental impairments in school-age children after cardiac surgery with full-flow cardio-pulmonary bypass. E-pub ahead of print in J Thorac Cardiovasc Surg 2012:1-7.
3.  Lantos J.  Cruel calculus: why saving premature babies is better business than helping themHealth Affairs 2010;29(11):2114-2117.