Parental Refusal of Surgery in an Infant With Tricuspid Atresia Alexander A. Kon, MD,a Angira Patel, MD, MPH, b Steven Leuthner, MD, MA,c John D. Lantos, MDd

We present a case of a fetal diagnosis of tricuspid atresia (TA). The abstract pregnant woman and her husband requested that the baby be treated with only palliative care. The cardiologist did not think it would be appropriate to withhold life-prolonging surgery once the infant was born. The neonatologist argued that outcomes for TA are similar to those for hypoplastic left heart syndrome, and the standard practice at the institution was to allow parents to choose surgery or end-of-life care for those infants. The team requested an ethics consultation to assist in determining whether forgoing life-prolonging interventions in this case would be ethically supportable. In this article, we ask a pediatric intensivist, a pediatric cardiologist, and a neonatologist to discuss the ethics of withholding life- sustaining treatment of a baby with TA. a Naval Medical Center San Diego and the University of California San Diego School of Medicine, San Diego, California; bLurie Children’s Hospital, Chicago, Illinois; cMedical College of Wisconsin, Milwaukee, Wisconsin; and Advances in fetal diagnosis now allow expert commentary on the ethics from dDepartment of Pediatrics, Children’s Mercy Kansas City, couples who are having a baby to an intensivist, a cardiologist, and a Kansas City, Missouri anticipate the decisions that will need neonatologist, all of whom are also All authors contributed to the design of this article to be made after birth. If the prenatal bioethicists. and the drafting and review of the manuscript, diagnosis is made early enough in and all authors approved the fi nal manuscript as pregnancy, then 1 option may be submitted. to terminate the pregnancy. When The views expressed in this article are those of the pregnancies are carried to term, then THE CASE authors and do not necessarily refl ect the offi cial policy or position of the Department of the Navy, the choices might be necessary about Department of Defense, or the US Government. whether to pursue life-sustaining A young married woman was DOI: 10.1542/peds.2016-1730 treatment or, instead, to provide only pregnant with her first child. During comfort-oriented palliative care. In routine prenatal care, an ultrasound Accepted for publication May 24, 2016 the abstract, the ethical principles that was performed and there was concern Address correspondence to John D. Lantos, MD, should guide such decisions are clear. that the child might have congenital Children’s Mercy Hospital, 2401 Gillham Rd, Kansas City, MO 64108. E-mail: [email protected] If the treatment is clearly beneficial, heart disease. A fetal echocardiogram then the baby’s right to treatment was performed at ~24 weeks’ PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275). should outweigh parents’ right to gestation that revealed tricuspid refuse. If, instead, the outcomes atresia (TA). The cardiologist met Copyright © 2016 by the American Academy of Pediatrics that are anticipated with treatment with the parents to discuss their son’s are ambiguous or uncertain, then condition, explaining the standard FINANCIAL DISCLOSURE: The authors have surgical approach and long-term indicated they have no fi nancial relationships the parents’ choices determine the relevant to this article to disclose. course of action. In practice, those prognosis. At home, the parents FUNDING: No external funding. principles are difficult to apply. researched the proposed surgery on Doctors may disagree about whether the Internet and learned that many POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential confl icts of a particular treatment of a particular parents decline this surgery. At their interest to disclose. condition is sufficiently successful so next appointment, the couple told the that parental refusal should not be cardiologist that they did not want permitted. Tricuspid atresia (TA) is 1 their baby to get the surgery and To cite: Kon AA, Patel A, Leuthner S, et al. Parental such condition. In this Ethics Rounds, instead would focus on making him Refusal of Surgery in an Infant With Tricuspid Atresia. Pediatrics. 2016;138(5):e20161730 we present a case of TA and seek comfortable.

Downloaded from www.aappublications.org/news by guest on September 28, 2021 PEDIATRICS Volume 138 , number 5 , November 2016 :e 20161730 ETHICS ROUNDS The perinatal team, including to overrule parents only when have feeding difficulties and high the cardiologist, neonatologist, parents make decisions that are caloric requirements for growth, obstetrician, and others, met to clearly contrary to the infant’s best so a gastrostomy tube is often discuss the case. The cardiologist interests. 1, 2 Merely disagreeing with needed to maintain adequate intake. voiced concerns about the father’s parents’ values and preferences is Potential complications of multiple decision, stating that she did not insufficient. This standard requires cardiac surgeries necessitating think it would be appropriate to that providers intervene only cardiopulmonary bypass include withhold life-prolonging surgery when parents make choices that heart failure, arrhythmias (which once the infant was born. The are inconsistent with decisions may necessitate pacemaker neonatologist argued that outcomes reasonable people would make. placement), and stroke with resultant for TA are similar to those for neurologic deficits. Furthermore, Providers lack the authority to hypoplastic left heart syndrome infants with SVs have a shorter than unilaterally overrule parents who (HLHS), and the standard practice at average life expectancy because of decline life-prolonging interventions the institution was to allow parents early heart and liver failure. Because (except in rare, emergent situations). to choose surgery or end-of-life infants with TA generally have an When providers believe that parents (EOL) care for those infants. Based adequately sized left ventricle, in are clearly acting contrary to the on the principle that equal patients contrast to infants with HLHS, there infant’s best interests, they may seek should be treated equally, he argued may be reason to believe that infants a court order to provide therapy that allowing the parents to decline with TA would be less likely to need that they believe is necessary and surgery would be appropriate. cardiac transplantation later in life appropriate. The decision to seek The team requested an ethics than infants with HLHS; however, a court order should not be made consultation to assist in determining there are insufficient data to draw lightly. In general, courts are more whether forgoing life-prolonging firm conclusions. willing to authorize treatment over interventions in this case would be parental objection if the treatment is As noted by the obstetrician, the ethically supportable. of short duration, there is consensus standard for infants with HLHS in the medical community regarding generally is to allow parents to Alex Kon, MD, Pediatric Intensivist, Comments the medically appropriate treatment, choose either life-prolonging the prognosis with treatment interventions or EOL care. In the When making life-and-death choices is favorable and there is a high case of HLHS, the option of EOL for an infant, parents and providers likelihood that the child would have care is generally considered must consider primarily the infant’s a relatively normal life, and without ethically permissible because best interest. 1 However, such treatment there is a high likelihood surgical outcomes are suboptimal decisions are highly value laden. that the child would die. With this (5-year survival is ~80%, 4 – 7 Different parents, and different understanding, we turn to the case at and there is significant risk of providers, may judge the same hand. neurodevelopmental and other situation very differently. To some, disorders among survivors8 – 15), Infants with single-ventricle (SV) the benefits of prolonging life, experts in the field are divided physiology such as TA and HLHS even for a short time in the face of between favoring life-prolonging whose parents choose life-prolonging significant morbidity, outweigh the interventions or EOL care, 16 –18 interventions follow a similar burdens of even significant suffering. and many believe that infants with surgical course. Both cardiac lesions For others, minimizing suffering is a HLHS endure significant suffering warrant surgical intervention more important goal than prolonging throughout their treatment course. within the first few days of life that life. In such cases, there is often no For these reasons, although there includes placement of a surgical single right answer. Furthermore, remains debate about appropriate shunt to supply pulmonary blood although the best interests of the care for infants with HLHS, 19 – 21 flow (note that experts continue to infant are central in decision-making, in general parents are given the develop new surgical and hybrid the interests of the parents, siblings, choice between life-prolonging approaches for initial management). and other may also be considered. 2, 3 interventions or EOL care. The initial surgery is usually followed The American Academy of by ≥2 additional cardiac surgeries, Surgical outcomes for infants with Pediatrics recognizes that most such generally including a bidirectional TA are similar when compared with decisions fall into a gray area in cavopulmonary anastomosis (Glenn outcomes for HLHS. Although it is which several goals of care may be procedure) by 6 months of life less well studied than HLHS, 5-year ethically permissible. The academy and a Fontan procedure before postoperative survival in TA is recommends that providers seek 5 years of age. Many affected infants ~80%, 22 – 25 and survivors are also

Downloaded from www.aappublications.org/news by guest on September 28, 2021 2 KON et al at risk for neurodevelopmental and for delayed surgical intervention. Angira Patel, MD, MPH, Pediatric other disorders.26 Therefore, when Therefore, left untreated the child Cardiologist, Comments the obstetrician argued that infants with TA will probably experience Congenital heart diseases consisting with TA should be treated similarly a slow decline in health during the of a functional SV (eg, HLHS, TA) to those with HLHS, his reasoning toddler and school-age years, leading require staged palliation concluding had some merit. to a long dying process. Because with a Fontan operation. Over the In this case, however, we must look of the length of the dying process, last 40 years, surgical technique not only at outcomes for infants who palliative interventions are less likely has evolved and significant strides undergo life-prolonging interventions to be effective, and there is a high risk have been made to reduce mortality. but also at outcomes for those whose that the child will suffer significantly. Historically, HLHS has been parents decline life-prolonging technically more challenging with intervention. In the case of HLHS, Because an infant with TA is likely higher mortality than other types of 95% of infants will die within the to suffer significantly if surgery is SVs, and EOL care has been accepted first month of life.27 Because of withheld, a decision to forgo life- an ethically permissible option. the rapid demise of these infants, prolonging interventions cannot Contemporary results, especially at providers can deliver high-quality be considered consistent with that high-volume technically excellent palliative care, and the pain and child’s best interest. Although the centers, show long-term survival suffering of infants during the dying obstetrician was correct in noting for HLHS approaching that of other process can be minimized. Therefore, that similar patients should be forms of SV such as TA. Data are EOL care is generally considered treated similarly, the difference difficult to extrapolate because of a reasonable alternative to life- in the natural course of TA and center-related and era effects, but prolonging interventions for infants HLHS leads to different ethically best estimates range from 80% to 85% for 10-year survival for both with HLHS. Data also suggest that permissible options for affected when given complete information, HLHS and TA. 7, 35 In actuality, all infants. Therefore, the parents parents are split regarding the functional SVs have a similar long- should be educated about the natural choices they make. 28 – 32 Furthermore, term burden of intensive surgical and course for their son if life-prolonging when experts are asked what they medical therapies. The difference interventions were not provided, and themselves would choose for their in mortality between HLHS and the providers should explain why own children, they too are split TA is negligible and no longer they believe that such a decision is in their decisions.16 – 18 Therefore, sufficient to treat the 2 diagnoses not appropriate. If the parents persist when parents of an infant with HLHS as different entities for an ethical believe that the potential burdens of in their refusal to give permission analysis. Specifically, life-prolonging interventions outweigh the potential for appropriate intervention, the treatment involves ≥2 surgeries benefits, they are generally allowed team should seek a court order in the first 3 years of life, cardiac to opt for EOL care. to authorize medically indicated catheterization and interventions, treatment. and lifelong need for monitoring and In contrast, infants with TA who treatment of complications including do not undergo life-prolonging In general, when we consider a premature death, ventricular interventions have a significantly child’s best interests, we tend to failure, thromboembolic disease, different prognosis. Many untreated focus solely on the potential benefits arrhythmia, liver disease, protein- infants with TA survive past the and burdens of the proposed losing enteropathy, and potential newborn period. Data suggest that intervention. This case illustrates the need for heart transplantation. These ~50% of these infants will die in the interventions are palliative and not first year of life, and the remaining importance of considering not only curative. However, the timing of children will survive for several the potential benefits and burdens of death without intervention for HLHS years (and potentially into young the proposed treatment but also the may be different than for TA; infants adulthood). 33, 34 The natural course potential benefits and burdens of the with HLHS generally die within 2 to of untreated TA is that children’s alternatives, including the option to 4 weeks without intervention, but pulmonary vascular resistance (PVR) forgo life-prolonging interventions. a small minority of infants with TA gradually increases over months to In some cases, such as this, the (depending on underlying anatomy) years. As the PVR increases, children alternatives are so clearly contrary can survive longer. 36 develop worsening hypoxemia. to the patient’s best interests that Because over time the increased a decision to forgo life-prolonging Given surgical and medical advances PVR becomes nonreversible, these interventions is not ethically leading to similar survival outcomes children are not good candidates supportable. for HLHS and TA with the same

Downloaded from www.aappublications.org/news by guest on September 28, 2021 PEDIATRICS Volume 138 , number 5 , November 2016 3 burden of long-term morbidities, the therapies or continued EOL care. The So let us assume the parents’ question now becomes, “Is it ethically subtleties of the diagnosis and the information is about SV issues and permissible to allow a family to forgo possibility that death may not come more likely HLHS, the diagnosis is life-prolonging interventions for a quickly necessitate a more nuanced correct, and there is no chromosomal child with any SV diagnosis? Do we discussion. abnormality or other syndrome. honor the choice of these parents The question then is whether HLHS I acknowledge the moral distress that is probably based on their and TA are equivalent medically of the cardiologist regarding what own family’s individual values and and therefore ethically. Some might is best for the child in the setting preferences?” I say yes. argue that the presentation of the of high probability for survival infant and the different forms of Parents are appropriately tasked (despite the intensive medical and TA are applicable. For instance, with the role of surrogate decision- surgical interventions and potential approximately one-third of TA cases makers for their children because morbidities). In this case, the have accompanying transposition they are in the best position to cardiologist may have to endure this of the great arteries that would consider the relative weight of distress yet stand with the family. necessitate a Norwood palliation risks and benefits of therapies. More importantly, the role of the in the newborn period, and two- Disagreements within the medical treating cardiologist continues thirds are not transposed and have community occur when there is a to remain crucial: to provide a pulmonary outflow track problem question of whether the parents are ongoing support, conveying known that would necessitate a Blalock– acting in the child’s best interest. information about how their child Taussig shunt or pulmonary band. For this child with TA, there are will die without treatment and what The fact remains that no matter both early and ongoing morbidities can be done to provide comfort to what the initial surgery would be, resulting in what parents perceive to optimize their time together. the eventual surgical goal is for be suffering and pain for their child. an SV Fontan procedure. So what In defining boundaries of what is Steven Leuthner, MD, Neonatologist, is the outcome difference for the medically and ethically acceptable, Comments Fontan physiology if a patient has we must weigh the morbidity and As the ethics consultant, I would a morphologic right rather than burden of invasive and intensive begin by clarifying 2 issues. The left ventricle, as well as long-term therapy against the potential first is the accuracy of the prenatal outcome of the Fontan procedure? benefits of those interventions. An diagnosis. One would not want to This gets at the justice issue the SV palliation by definition involves counsel and have the parents make neonatologist is suggesting, that if for lifelong invasive medical care this prenatal decision unless there is HLHS one would support providing that may not be the right choice a reasonable degree of accuracy in palliative care, then why not for TA? for some families. These parents, the diagnosis. Although there are a understanding both known and variety of anatomic situations with Here the data seem mixed. One unknown risks and benefits and TA, the published accuracy of the study suggests that 10-year survival incorporating their own values and diagnosis is excellent, at 97%. 24 Other is ≤85% of those born with a preferences, have decided that an SV independent predictors of poorer dominant left ventricle, as opposed palliation is not in the best interest of outcome, such as chromosomal or to only 65% for those born with a their child. There is no “right” answer syndromic findings, might be of value dominant right ventricle. 37 A much here. More leeway to parental in the prenatal counseling period as larger 40-year follow-up of the decision-making should be allowed well. Fontan operation, which includes when the burdens of expected The second issue to clarify would a large group of patients with TA, therapy are real and significant, be the Internet information the shows the 10-, 20-, and 30-year as they are in this case. Allowing parents are basing their decision on, freedom from death or cardiac the family to forgo life-prolonging for the single reason of making sure transplant being 73%, 59%, and therapies is ethically supportable. they are basing their decision on 40%, respectively. 35 They describe However, the parents of this infant appropriate data and similar cases. the many complications of the ought to be counseled specifically It would not be surprising if this Fontan, including premature death, on natural history of TA without Internet information was more about ventricular failure, thromboembolic intervention (which may be HLHS than TA. If there was some disease, arrhythmia, liver disease, different from HLHS). If the infant misunderstanding or inappropriate and protein-losing enteropathy. survives >6 months, a reevaluation comparisons were being made, It must be acknowledged that of options would be anticipated, simply reviewing this information this is a significant chronic illness including consideration of surgical might lead to consensus. with physical and neurocognitive

Downloaded from www.aappublications.org/news by guest on September 28, 2021 4 KON et al limitations, necessitating procedures parental choice is not only for the commentaries illustrate the ways in beyond the 3-stage repair if the initial neonatal surgery but for all which thoughtful people can look at child survives the surgeries. anticipated procedures. For the the same data or the same case and Acknowledging these issues, the SV path should this also include come up with different responses data suggest that at this time it is transplantation if the Fontan failed? about the appropriate course of reasonable to consider HLHS and TA Overriding parental authority action. That does not always happen. as equivalent SV lesions for ethical early also compromises issues Arguments by bioethicists have decision-making. of trust and long-term care for a changed the ways in which we medically complicated child. Ethical respond to a wide variety of cases. If the lesions are equivalent, then frameworks including the best We used to permit parents to refuse the ethical principle of justice, or interest standard, reasonable person life-saving surgery for babies with treating equal patients equally, does standard, or parental discretion all trisomy 21. We used to refuse to come into play. Interestingly, as support a family having the right to perform life-saving surgery on babies the survival of infants with HLHS make a reasonable decision, even with trisomy 18. The borderline undergoing the staged repair has if they are in the minority view. of viability has shifted slowly but improved, there continues to be They maintain that family values steadily and with it the threshold for debate about whether parents and family impact are reasonable mandating life-sustaining treatment should still be offered palliative to consider. These are high-risk of premature infants. When care. 19, 20 Essentially using the justice procedures with significant burdens disagreements persist, it suggests a argument, it is often suggested that for the infant and family, not only in lack of consensus in the professional with improved HLHS survival and the neonatal period but throughout community. In such cases, the proper outcomes there are other cardiac life. If we would not mandate things thing is to defer to parents. Careful cases with worse outcomes, yet all the way through, then EOL care at consideration of the arguments palliative care might not be offered any stage seems a permissible choice can help us counsel parents and in those. When thinking of justice for a family. ensure that their decisions are in this way, we should beware of informed decisions. In that sense, faulty reasoning, because 2 wrongs Remembering that this is a prenatal disagreements between bioethicists would not make a decision right. The case, it is important to appreciate are no different, and perhaps no ethical literature continues to show that a prenatal mandate to intervene more common, than disagreements although the outcomes for HLHS after birth could put the family between cardiologists, policymakers, have improved, and there might in a position to consider other or other experts. They signal the be a recommended medical plan, obstetrical options. These could limits of our collective ability to know they have not yet reached the level include termination of pregnancy, what is best and the intensity and for which palliative care is not an although this can only occur in a few integrity of our efforts to keep finding acceptable choice that parents should places nationally at this gestational out. be informed about. 21, 38, 39 In this case, age, or even arranging a delivery and at this institution, the standard plan to avoid the institution. This practice is to allow parents of infants would not serve the infant best, with HLHS to choose surgery or EOL because in the end there must be a ABBREVIATIONS care. The neonatologist is correct trusting relationship to help develop EOL: end of life to suggest that based on justice, a palliative care plan for this infant HLHS: hypoplastic left heart because the medical conditions who, depending on the outflow track syndrome are reasonably equivalent, if it is anatomy, could have different care PVR: pulmonary vascular reasonable to offer EOL care in cases needs and projections of neonatal resistance of HLHS, it is reasonable to offer it in death. SV: single ventricle cases of TA. TA: tricuspid atresia John D. Lantos, MD, Bioethicist, The underlying ethical question is Comments whether the survival and quality of life for these infants meet a Bioethics is often criticized for not REFERENCES threshold for which the medical team only having no right answers but for 1. American Academy of Pediatrics should consider intervening legally not even having a method of getting Committee on Bioethics. American to override parental authority. In to a right answer in difficult cases. Academy of Pediatrics Committee on the case of known complex staged Instead, the critics say, there are just Bioethics: guidelines on foregoing repair, ethical consistency would equally powerful arguments on both life-sustaining medical treatment. require that a decision to override sides. That is sometimes true. These Pediatrics. 1994;93(3):532–536

Downloaded from www.aappublications.org/news by guest on September 28, 2021 PEDIATRICS Volume 138 , number 5 , November 2016 5 2. Mercurio MR, Maxwell MA, Mears BJ, 12. Mahle WT, Visconti KJ, Freier MC, surgical strategy on survival of infants Ross LF, Silber TJ. American Academy et al. Relationship of surgical approach with tricuspid atresia. Ann Thorac of Pediatrics policy statements to neurodevelopmental outcomes Surg. 2015;100(4):1403–1409 on bioethics: summaries and in hypoplastic left heart syndrome. 23. Wilder TJ, Ziemer G, Hickey EJ, et al commentaries: part 2. Pediatr Rev. Pediatrics. 2006;117(1). Available at: Surgical management of competing 2008;29(3):e15–e22 www.pediatrics. org/ cgi/ content/ full/ pulmonary blood fl ow affects survival 117/ 1/ e90 3. Willems DL, Verhagen AA, van Wijlick before Fontan/Kreutzer completion E; Committee End-of-Life Decisions 13. Mahle WT, Wernovsky G. in patients with tricuspid atresia in Severely Ill Newborns of Royal Neurodevelopmental outcomes in type I. J Thorac Cardiovasc Surg. Dutch Medical Association. Infants’ hypoplastic left heart syndrome. Semin 2015;150(5):1222–1230.e7 best interests in end-of-life care for Thorac Cardiovasc Surg Pediatr Card newborns. Pediatrics. 2014;134(4). Surg Annu. 2004;7:39–47 24. Wald RM, Tham EB, McCrindle Available at: www.pediatrics. org/ cgi/ BW, et al. Outcome after prenatal 14. Knirsch W, Liamlahi R, Hug MI, et al. content/ full/ 134/ 4/ e1163 diagnosis of tricuspid atresia: a Mortality and neurodevelopmental multicenter experience. Am Heart J. 4. DiBardino DJ, Gomez-Arostegui J, outcome at 1 year of age comparing 2007;153(5):772–778 Kemp A, et al. Intermediate results hybrid and Norwood procedures. Eur J of hybrid versus primary Norwood Cardiothorac Surg. 2012;42(1):33–39 25. Sittiwangkul R, Azakie A, Van Arsdell operation. Ann Thorac Surg. GS, Williams WG, McCrindle BW. 15. Mahle WT, Clancy RR, Moss EM, 2015;99(6):2141–2147, discussion Outcomes of tricuspid atresia in Gerdes M, Jobes DR, Wernovsky 2147–2149 the Fontan era. Ann Thorac Surg. G. Neurodevelopmental outcome 2004;77(3):889–894 5. Alsoufi B, Wolf M, Botha P, et al. Late and lifestyle assessment in school- outcomes of infants supported by aged and adolescent children with 26. Mastalir ET, Kalil RA, Horowitz ES, extracorporeal membrane oxygenation hypoplastic left heart syndrome. et al. Late clinical outcomes of the following the Norwood operation. Pediatrics. 2000;105(5):1082–1089 Fontan operation in patients with World J Pediatr Congenit Heart Surg. tricuspid atresia. Arq Bras Cardiol. 16. Kon AA, Ackerson L, Lo B. Choices 2015;6(1):9–17 2002;79(1):56–60 physicians would make if they were 6. Carrillo SA, Mainwaring RD, Schaffer the parents of a child with hypoplastic 27. Barber G. Hypoplastic left heart JM, et al. Contemporaneous left heart syndrome. Am J Cardiol. syndrome. In: Garson A, Bricker J, comparison of the Yasui and 2003;91(12):1506–1509, A1509 McNamara D, eds. The Science and Norwood procedures at a single Practice of Pediatric Cardiology. 17. Prsa M, Holly CD, Carnevale FA, Justino institution. J Thorac Cardiovasc Surg. Philadelphia, PA: Lea & Febiger; H, Rohlicek CV. Attitudes and practices 2015;149(2):508–513 1990:1316–1331 of cardiologists and surgeons who 7. DiBardino DJ. Long-term progression manage HLHS. Pediatrics. 2010;125(3). 28. Chang RK, Chen AY, Klitzner TS. and survival following Norwood single Available at: www.pediatrics. org/ cgi/ Clinical management of infants with ventricle reconstruction. Curr Opin content/ full/ 125/ 3/ e625 hypoplastic left heart syndrome in the Cardiol. 2015;30(1):95–99 18. Kon AA, Prsa M, Rohlicek CV. Choices United States, 1988–1997. Pediatrics. 8. Hangge PT, Cnota JF, Woo JG, et al. doctors would make if their infant 2002;110(2 pt 1):292–298 Microcephaly is associated with had hypoplastic left heart syndrome: 29. Corrow C, Lapuk S, Mazzarella K, Sable early adverse neurologic outcomes comparison of survey data from A, Leopold H, Eisenfeld L. Hypoplastic in hypoplastic left heart syndrome. 1999 and 2007. Pediatr Cardiol. left heart syndrome: factors Pediatr Res. 2013;74(1):61–67 2013;34(2):348–353 infl uencing therapeutic choice. Conn 9. Sarajuuri A, Jokinen E, Puosi R, et al. 19. Kon AA. Healthcare providers must Med. 2001;65(4):195–203 Neurodevelopment in children with offer palliative treatment to parents hypoplastic left heart syndrome. 30. Osiovich H, Phillipos E, Byrne P, of neonates with hypoplastic left heart J Pediatr. 2010;157(3):414–420, Robertson M. Hypoplastic left heart syndrome. Arch Pediatr Adolesc Med. e411–414 syndrome: “to treat or not to treat.” 2008;162(9):844–848 J Perinatol. 2000;20(6):363–365 10. Tabbutt S, Nord AS, Jarvik GP, et al. 20. Wernovsky G. The paradigm shift Neurodevelopmental outcomes after 31. Vandvik IH, Førde R. Ethical issues in toward surgical intervention for staged palliation for hypoplastic parental decision-making. An interview neonates with hypoplastic left heart left heart syndrome. Pediatrics. study of mothers of children with syndrome. Arch Pediatr Adolesc Med. 2008;121(3):476–483 hypoplastic left heart syndrome. Acta 2008;162(9):849–854 Paediatr. 2000;89(9):1129–1133 11. Bordacova L, Docolomanska D, Masura J. Neuropsychological 21. Feudtner C. Ethics in the midst of 32. Munn MB, Brumfi eld CG, Lau Y, Colvin outcome in children with hypoplastic therapeutic evolution. Arch Pediatr EV. Prenatally diagnosed hypoplastic left heart syndrome. Bratisl Lek Listy Adolesc Med. 2008;162(9):854–857 left heart syndrome: outcomes after (Tlacene Vyd). 2007;108(4–5): 22. Alsoufi B, Schlosser B, Mori M, et al postnatal surgery. J Matern Fetal Med. 203–206 Infl uence of morphology and initial 1999;8(4):147–150

Downloaded from www.aappublications.org/news by guest on September 28, 2021 6 KON et al 33. Fesslova V, Hunter S, Stark J, Taylor JF. 35. Pundi KN, Johnson JN, Dearani JA, right ventricular dominance. J Am Coll Long-term clinical outcome of patients et al. 40-Year follow-up after the Cardiol. 2012;59(13):1178–1185 with tricuspid atresia. I. “Natural Fontan operation: long-term outcomes 38. Mercurio MR, Peterec SM, Weeks history.” J Cardiovasc Surg (Torino). of 1, 052 patients. J Am Coll Cardiol. B. Hypoplastic left heart syndrome, 1989;30(2):262–272 2015;66(15):1700–1710 extreme prematurity, comfort care 34. Schmaltz AA, Hinkeldey K, Hoffmeister 36. Hoffman JIE. The Natural and only, and the principle of justice. HE, Apitz J. [Prognosis of children with Unnatural History of Congenital Heart Pediatrics. 2008;122(1):186–189 congenital tricuspid and pulmonary Disease. Hoboken, NJ: Wiley-Blackwell; 39. Ross LF, Frader J. Hypoplastic left atresia 1967–83 in comparison with 2009 heart syndrome: a paradigm case for the natural course]. Monatsschr 37. d’Udekem Y, Xu MY, Galati JC, et al. examining conscientious objection Kinderheilkd. 1985;133(10): Predictors of survival after single- in pediatric practice. J Pediatr. 743–748 ventricle palliation: the impact of 2009;155(1):12–15

Downloaded from www.aappublications.org/news by guest on September 28, 2021 PEDIATRICS Volume 138 , number 5 , November 2016 7 Parental Refusal of Surgery in an Infant With Tricuspid Atresia Alexander A. Kon, Angira Patel, Steven Leuthner and John D. Lantos Pediatrics 2016;138; DOI: 10.1542/peds.2016-1730 originally published online October 7, 2016;

Updated Information & including high resolution figures, can be found at: Services http://pediatrics.aappublications.org/content/138/5/e20161730 References This article cites 37 articles, 10 of which you can access for free at: http://pediatrics.aappublications.org/content/138/5/e20161730#BIBL Subspecialty Collections This article, along with others on similar topics, appears in the following collection(s): Ethics/Bioethics http://www.aappublications.org/cgi/collection/ethics:bioethics_sub Fetus/Newborn Infant http://www.aappublications.org/cgi/collection/fetus:newborn_infant_ sub Birth Defects http://www.aappublications.org/cgi/collection/birth_defects_sub Permissions & Licensing Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: http://www.aappublications.org/site/misc/Permissions.xhtml Reprints Information about ordering reprints can be found online: http://www.aappublications.org/site/misc/reprints.xhtml

Downloaded from www.aappublications.org/news by guest on September 28, 2021 Parental Refusal of Surgery in an Infant With Tricuspid Atresia Alexander A. Kon, Angira Patel, Steven Leuthner and John D. Lantos Pediatrics 2016;138; DOI: 10.1542/peds.2016-1730 originally published online October 7, 2016;

The online version of this article, along with updated information and services, is located on the World Wide Web at: http://pediatrics.aappublications.org/content/138/5/e20161730

Pediatrics is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. Pediatrics is owned, published, and trademarked by the American Academy of Pediatrics, 345 Park Avenue, Itasca, Illinois, 60143. Copyright © 2016 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 1073-0397.

Downloaded from www.aappublications.org/news by guest on September 28, 2021