Janvier A, Farlow B, Barrington KJ. Cardiac surgery for children with trisomies 13 and 18: Where are we now? Seminars in Perinatology. 2016. Annie and Barb and I have just published this review of the current situation regarding cardiac surgery for infants with the aneuploidies in the title. (For those who don’t know, Barb Farlow is a mother of a child, who has sadly died, who had trisomy 13.) In addition to discussing the literature we make some recommendations about how to help families making difficult decisions. The literature review is limited by the common practice of lumping together all kinds of cardiac surgery, from ASD repairs to Fontan procedures and Norwoods. Also few papers describe whether there was withholding or withdrawal of life-sustaining interventions at any time.
Nevertheless, it is clear that prolonged survival after cardiac surgery is possible.
Our hope is that we will move away from the universal denial of cardiac surgery, which still occurs in some places. On the other hand it is not appropriate, for any patient, to ignore their other problems pretending they don’t exist, infants with aneuploidies often have other difficulties, which may be serious, but which are very variable. Only by making a decision with the parents which is medically appropriate, likely to improve their life, and consistent with the families values, will we be able to truly act in their best interests.
We offer some guidance which will hopefully be helpful, this is an abbreviated version of our suggestions.
(1) Words are important, avoid these words: “vegetable,” “futile,” “lethal,” “incompatible with life,” “waste of time/resources/emotions,” “Tee 13 or 18,” “it, that,” “product of conception,” “hopeless,” “nothing we can do.”
(2) Avoid unfounded and biased predictions: do not tell parents that this child will have a negative impact on their family, their other children, or their lives.
(3) Recognize that each child and family is unique
(4) Provide balanced counseling: which includes recent medical information, as well as the experience of families who live(d) with children with these conditions.
(5) Personalize the information: all infants are different, even if they carry the same label.
(6) One step at the time: decisions about potential complex cardiac interventions need not be discussed and decided upon prenatally, but be forthright about care options for stable children.
(7) Provide parents with different possible outcomes for their fetus/child (including stillbirth): emphasize the spectrum and uniqueness of each child and the lack of control you and they have on many of these outcomes.
(8) Empower parents: recognize that this diagnosis is difficult for parents, that they are doing their best.
(9) Offer reasonable hope: hope that their child might defy the odds and hope that her life, however long, will have an enriching and positive influence on all who love her.
(10) Support parents: ask them how you can help them.
We hope that families facing these decisions will find caregivers who are sensitive to their needs, willing to consider the options, and supportive.