The field of pediatric cardiology is relatively young. It was approximately 80 years ago when the first patent ductus arteriosus was ligated and a few years later when the original Blalock-Taussig operation was performed to address a severely cyanotic child with tetralogy of Fallot. With medical and surgical advances since that time, once-fatal congenital heart disease can now be addressed with neonatal surgical repair with excellent survival rates. Over the past 3 years of my fellowship, I have sat at the bedside, both prenatally and postnatally, with numerous families to deliver the news that their babies have congenital heart disease. Because of such advances in this young field, though, we have been able to provide an immediate management plan for these families.
We have certainly improved the quantity of our patients’ lives, and we must aim to improve the quality as well. As short-term survival rates have increased for our patients, research has now focused more on decreasing long-term morbidity and improving quality of life. Retrospective and prospective analyses can study prenatal, surgical, medical, and environmental risk factors associated with long-term morbidity. Families and patients can also complete a series of validated surveys to analyze quality of life in physical and psychosocial realms.
The first step in addressing this issue is to inform families upfront, to relay the evidence we have of longterm outcomes, and to be honest about uncertainties that lie ahead. In general, approximately 50% of children with congenital heart disease will experience at least a mild degree of adverse neurodevelopmental outcomes, from attention deficit disorder to learning disabilities to impaired social interactions. Counseling families from the time of diagnosis for these potential long-term issues allows them to seek appropriate resources for their children and to advocate for them if any issues do arise.
Next, we must be constantly aware of the importance of allowing our patients to lead normal lives. In addition to our physical examinations and studies examining our patients’ hearts, we must routinely ask about their school, families, and lives. Regardless of neurodevelopmental outcomes, our goal is for our patients’ disease to be just a part of their lives and not what defines them. It would be easy to wrap these children in bubble wrap to prevent any harm from befalling them. Instead, it is important to allow children to be children. They must be allowed to run and fall, to succeed and fail. There are certainly situations when restricting activity is imperative to prevent sudden cardiac death or worsening of underlying pathology, but there should always be a team approach for these decisions among the cardiology team, parents, and patient.
As the population of children and adults with congenital heart disease increases, this importance of quality of life will only continue to grow.