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Passive Euthanasia for Hypoplastic Left Heart Syndrome

Am J Dis Child. 1992;146(12):1426. doi:10.1001/archpedi.1992.02160240036016.
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Passive euthanasia is one of the three options for infants newly diagnosed as having hypoplastic leftheart syndrome (HLHS). Each year in the United States, approximately 1000 infants are born with HLHS. Of these infants, two thirds have no other detectable malformation that would compromise length and quality of life. Once the diagnosis is made, usually within the first week of life, prostaglandin Ej is administered to maintain systemic blood flow while the parents decide whether their infant will receive no further treatment (discontinuation of prostaglandin) or surgical treatment: a staged repair (Norwood procedure) or a cardiac transplant. The current approach to providing surgical treatment for HLHS denies parents the information they need to make this decision, does not provide optimum care for these infants, and does not use resources efficiently.

Withdrawing life support is an appropriate option for an infant with a congenital malformation if valid data tell us that treatment has little or no possibility of prolonging life, offers little or no potential for interaction and development, and results only in pain and discomfort. If we can accurately determine that a given infant has a congenital malformation with this prognosis, we may ask the parents to consider passive euthanasia for their infant. For example, a newborn with Potter syndrome (pulmonary hypoplasia and renal agenesis) meets these criteria because we have valid data that the probability for survival and quality of life approaches zero and we can make the diagnosis with an accuracy approaching 100%. We do not, however, have valid data on survival and quality of life for infants with HLHS after either staged repair or transplant against which to evaluate the benefits of passive euthanasia. For newborns with HLHS, existing data suggest that the probability of survival to age 1 year with normal interaction and development ranges from 5% to 58% with staged repair and from 33% to 80% with transplantation. The wide range of reported outcomes in published reports and personal communications does not provide a rational basis for surgical and ethical decisions


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