What if it Were Your Child

W ith his letter agreeing to publish our manuscript on the management of hypoplastic left heart syndrome (HLHS) in the 1990s, the editor asked me the most dreaded of questions posed to pediatric cardiologists: “What would you do if it were your child?” The easy answer, to parents at least, is that I can’t answer the question because it isn’t my child, and the answer depends upon a host of ethical, religious, social, and even economic issues that are unique to each family. I then proceed to give them my recommendation, emphasizing that it is their decision and I will support them in whatever they decide. When I began my career in pediatric cardiology in the 1970s, the major decision regarding a newborn with suspected HLHS (then and now regarded as “the worst heart disease”) was whether to confirm the diagnosis by angiography, or to rely on echocardiography. The subsequent improvements in 2-dimensional and Doppler echocardiography made noninvasive diagnosis fairly simple, but shortly thereafter cardiac transplantation and the Norwood procedure were introduced as therapeutic options and difficult decision making returned. By 1990, modest success had been achieved with each form of surgical intervention and it was common to have symposia at major cardiology and pediatric meetings at which speakers would advocate transplantation, the Norwood procedure, and compassionate care. After the discussion period, the moderator often asked the audience to indicate by a show of hands what they would do if it were their child; almost all indicated that they would opt for compassionate care and forgo surgery. I suspect that a decade later, more physicians would choose intervention because of the improved surgical results, but many would remain hesitant to subject their child and their family to the enormous risks and commitments involved in either cardiac transplantation or the Norwood-Glenn-Fontan pathway. With the development of fetal echocardiography, the decision-making process has been moved from birth to the end of the first trimester, at which time most congenital cardiac abnormalities can be diagnosed by ultrasound. In Great Britain and much of Europe, termination of pregnancy upon diagnosis of congenital heart abnormalities is quite common, not only for HLHS but for other major structural abnormalities as well. This has actually produced a measurable decrease in the incidence of HLHS. In the USA, abortion is one of the most controversial issues in medicine, religion, and politics. A question regarding whether pediatric cardiologists included termination of pregnancy as an option when counseling parents of a fetus with congenital heart disease elicited more responses than any other question posed in the history of PediHeart, an internet professional discussion forum. The issue is no more settled in the medical than in the political arena. In my own experience at an academic medical center in rural Virginia, the number of parents electing to terminate a pregnancy because of a prenatal diagnosis of HLHS is extremely small (and most have involved cardiac disease as part of an major chromosomal abnormality). My experience with neonates mirrors that described in our report of management of HLHS in the USA: a finite but gradually decreasing number of parents and physicians (10% to 15% in the past decade) elect a strategy of comfort care after birth. So, what would I do if it were my child? I don’t know. Even if I did, I wouldn’t tell my patients. It is with good reason that physicians are advised not to care for themselves or their families. Personal issues may cloud medical evidence. Physicians advising parents about intervention for serious medical conditions such as HLHS must be careful not to confuse the perceived impact of such a child on their own life and family with the best interests of the child. Many pediatric cardiologists caring for infants with HLHS have sensed an inverse relation between parental socioeconomic class and the desire for surgical intervention, a notion recently confirmed. Neurodevelopmental disabilities are common in survivors of HLHS surgery, and physicians, near the top of the socioeconomic scale, may place greater importance on such disabilities than does the population as a whole. In 1990, I thought that the morbidity and mortality associated with surgical intervention were so great that I didn’t know whether intervention or comfort care was best for the newborn with HLHS. In 2002, I think the balance has shifted to surgery and that is what I advise the parents of my patients. If donor hearts were more readily available, I would recommend transplantation; because they are not, I currently recommend a Norwood procedure.