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Informed consent, bioethical equipoise, and hypoplastic left heart syndrome

  • Constantine Mavroudis (a1) (a2), Constantine D. Mavroudis (a3), Ruth M. Farrell (a2), Marshall L. Jacobs (a1), Jeffrey P. Jacobs (a4) and Eric D. Kodish (a2)...
Abstract
Abstract

In utero diagnosis of complex progressive cardiac disease such as hypoplastic left heart syndrome presents a novel opportunity for antepartum, intrapartum, and neonatal management. The clinical possibilities and potential for differing outcomes challenge the mother–foetus dyad with regard to informed consent. Previous studies reveal that rates of termination of pregnancy for foetuses with hypoplastic left heart syndrome vary widely in the United States and Europe, leading us to surmise that informed consent may be practised differently. The purpose of this paper is to review the ethical considerations and physician responsibilities of informed consent as they relate to prenatal and postnatal patients with hypoplastic left heart syndrome. Special consideration is paid to the informed consent process as practised by the obstetrician, perinatologist, paediatric cardiologist, and paediatric cardiac surgeon as it relates to termination of pregnancy, comfort care, and surgical palliation. We will argue that informed consent as it relates to hypoplastic left heart syndrome is far from standardised and that there exists a state of bioethical equipoise concerning the extent and limits of its application in the current clinical setting.

Copyright
Corresponding author
Correspondence to: Dr C. Mavroudis, MD, Department of Pediatric and Adult Congenital Heart Surgery, Cleveland Clinic Children's Hospital, 9500 Euclid Avenue/M41-02, Cleveland, Ohio 44195, United States of America. Tel: +1 216 636 5288; Fax: +1 216 636 5859; E-mail: mavrouc@ccf.org
References
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1.Rasiah SV, Publicover M, Ewer AK, Khan KS, Kilby MD, Zamora J. A systematic review of the accuracy of first-trimester ultrasound examination for detecting major congenital heart disease. Ultrasound Obstet Gynecol 2006; 28: 110116.
2.Harris LH. Rethinking maternal-fetal conflict: gender and equality in perinatal ethics. Obstet Gynecol 2000; 96: 786791.
3.Berg JW, Appelbaum PS, Lidz CW, Parker LS. Informed Consent: Legal Theory and Clinical Practice, 2nd edn. Oxford University Press, New York, 2001.
4. Salgo v. Leland Stanford Jr University Board of Trustees (154 Cal. App. 3d 560, 317 P.2d 170 [1957]).
5.Norwood WI Jr. Hypoplastic left heart syndrome. Ann Thorac Surg 1991; 52: 688695.
6.Bailey LL. Origins of neonatal heart transplantation: an historical perspective. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011; 14: 98100.
7.Mavroudis C, Harrison H, Klein JB, et al. Infant orthotopic cardiac transplantation. J Thorac Cardiovasc Surg 1988; 96: 912924.
8.Backer CL, Zales VR, Idriss FS, et al. Heart transplantation in neonates and in children. J Heart Lung Transplant 1992; 11: 311319.
9.McElhinney DB, Tworetzky W, Lock JE. Current status of fetal cardiac intervention. Circulation 2010; 121: 12561263.
10.McElhinney DB, Marshall AC, Wilkins-Haug LE, et al. Predictors of technical success and postnatal biventricular outcome after in utero aortic valvuloplasty for aortic stenosis with evolving hypoplastic left heart syndrome. Circulation 2009; 120: 14821490.
11.Gardiner HM. The case for fetal cardiac intervention. Heart 2009; 95: 16481652.
12.Simpson JM. Fetal cardiac interventions: worth it? Heart 2009; 95: 16531655.
13.Jone PN, Schowengerdt KO Jr. Prenatal diagnosis of congenital heart disease. Pediatr Clin North Am 2009; 56: 709715.
14.Manson NC, O'Neill O. Rethinking Informed Consent in Bioethics, 1st edn. Cambridge University Press, New York, 2007.
15.Aristotle. The Nicomachean Ethics, Book III (Translated by Sir David Ross). Oxford University Press, London, 1954.
16.Fontan F, Kirklin JW, Fernandez G, et al. Outcome after a “perfect” Fontan operation. Circulation 1990; 81: 15201536.
17.de Leval MR, Deanfield JE. Four decades of Fontan palliation. Nat Rev Cardiol 2010; 7: 520527.
18.Mavroudis C, Deal BJ, Backer CL, et al. J. Maxwell Chamberlain Memorial Paper for congenital heart surgery. 111 Fontan conversions with arrhythmia surgery: surgical lessons and outcomes. Ann Thorac Surg 2007; 84: 14571466.
19.Lyerly AD, Mitchell LM, Armstrong EM, et al. Risk and the pregnant body. Hastings Cent Rep 2009; 39: 3442.
20.Brown SD, Truog RD, Johnson JA, Ecker JL. Do differences in the American Academy of Pediatrics and the American College of Obstetricians and Gynecologists positions on the ethics of maternal–fetal interventions reflect subtly divergent professional sensitivities to pregnant women and fetuses? Pediatrics 2006; 117: 13821387.
21.Bove EL, Ohye RG, Devaney EJ. Hypoplastic left heart syndrome: conventional surgical management. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004; 7: 310.
22.Wernovsky G. The paradigm shift toward surgical intervention for neonates with hypoplastic left heart syndrome. Arch Pediatr Adolesc Med 2008; 162: 849854.
23.Hirsch J. Ethical considerations associated with caring for patients with hypoplastic left heart syndrome. Session one: Comfort care in the setting of improved techniques: Comfort care is no longer an option (Point counterpoint). Presented at the 11th Annual International Symposium on Congenital Heart Disease, St. Petersburg, Florida, February 10–13, 2011.
24.Ross LF, Frader J. Hypoplastic left heart syndrome: a paradigm case for examining conscientious objection in pediatric practice. J Pediatr 2009; 155: 1215.
25.Chervenak FA, McCullough LB. Ethics of fetal surgery. Clin Perinatol 2009; 36: 237246.
26.Mellander M. Perinatal management, counselling and outcome of fetuses with congenital heart disease. Semin Fetal Neonatal Med 2005; 10: 586593.
27.Jancelewicz T, Harrison MR. A history of fetal surgery. Clin Perinatol 2009; 36: 227236.
28.Patel A, Hickey E, Mavroudis C, et al. Impact of noncardiac congenital and genetic abnormalities on outcomes in hypoplastic left heart syndrome. Ann Thorac Surg 2010; 89: 18051814.
29.Alton GY, Rempel GR, Robertson CM, Newburn-Cook CV, Norris CM. Functional outcomes after neonatal open cardiac surgery: comparison of survivors of the Norwood staged procedure and the arterial switch operation. Cardiol Young 2010; 20: 668675.
30.Joesch JM, Smith KR. Children's health and their mothers’ risk of divorce or separation. Soc Biol 1997; 44: 159169.
31.Brosig CL, Mussatto KA, Kuhn EM, Tweddell JS. Psychosocial outcomes for preschool children and families after surgery for complex congenital heart disease. Pediatr Cardiol 2007; 28: 255262.
32.d'Udekem Y, Cheung MM, Setyapranata S, et al. How good is a Fontan operation? Quality of life and exercise capacity of Fontans without arrhythmias. Ann Thorac Surg 2009; 88: 19611969.
33.Groopman J. The Anatomy of Hope: How People Prevail in the Face of Illness. Random House Trade Paperbacks, New York, 2004.
34. Jacobs ML. Personal communication, January 2011.
35. Gifted RIT/NTID student gives hope, life, to others. Available at: http://deafroc.com/2011/01/04/gifted-ritntid-student-gives-hope-life-to-others/. Accessed April 28, 2011.
36.Opotowsky AR, Shellenberger D, Dharan V, et al. Successful pregnancies in two women with hypoplastic left heart syndrome. Congenit Heart Dis 2010; 5: 476481.
37.Lee ST, White AJ, Matsushita S, et al. Intramyocardial injection of autologous cardiospheres or cardiosphere-derived cells preserves function and minimizes adverse ventricular remodeling in pigs with heart failure post-myocardial infarction. J Am Coll Cardiol 2011; 57: 455465.
38.Lacour-Gayet FG, Lanning CJ, Stoica S, et al. An artificial right ventricle for failing Fontan: in vitro and computational study. Ann Thorac Surg 2009; 88: 170176.
39.Chinen J, Buckley RH. Transplantation immunology: solid organ and bone marrow. J Allergy Clin Immunol 2010; 125: S324S335.
40.Calne R. Xenografting – the future of transplantation, and always will be? Xenotransplantation 2005; 12: 56.
41.Elliott MJ. A European perspective on the management of hypoplastic left heart syndrome. Cardiol Young 2004; 14: 4146.
42.Prsa M, Holly CD, Carnevale FA, Justino H, Rohlicek CV. Attitudes and practices of cardiologists and surgeons who manage HLHS. Pediatrics 2010; 125: e625e630.
43.Khoshnood B, De Vigan C, Vodovar V, et al. Trends in prenatal diagnosis, pregnancy termination, and perinatal mortality of newborns with congenital heart disease in France, 1983–2000: a population-based evaluation. Pediatrics 2005; 115: 95101.
44.Plantinga RF, Frohn-Mulder IM, Takkenberg HJ, Bennink GB, Meijboom EJ, Bogers AJ. [Surgical treatment of hypoplastic left heart syndrome: limited success]. Ned Tijdschr Geneeskd 2001; 145: 8590.
45.Galindo A, Nieto O, Villagrá S, Grañeras A, Herraiz I, Mendoza A. Hypoplastic left heart syndrome diagnosed in fetal life: associated findings, pregnancy outcome and results of palliative surgery. Ultrasound Obstet Gynecol 2009; 33: 560566.
46.Tibballs J, Cantwell-Bartl A. Outcomes of management decisions by parents for their infants with hypoplastic left heart syndrome born with and without a prenatal diagnosis. J Paediatr Child Health 2008; 44: 321324.
47.Wilkinson DJ. Antenatal diagnosis of trisomy 18, harm and parental choice. J Med Ethics 2010; 36: 644645.
48.Parker MJ, Budd JL, Draper ES, Young ID. Trisomy 13 and trisomy 18 in a defined population: epidemiological, genetic and prenatal observations. Prenat Diagn 2003; 23: 856860.
49.Freedman B. Equipoise and the ethics of clinical research. N Engl J Med 1987; 317: 141145.
50.Ohye RG, Sleeper LA, Mahony L, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 2010; 362: 19801992.
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Cardiology in the Young
  • ISSN: 1047-9511
  • EISSN: 1467-1107
  • URL: /core/journals/cardiology-in-the-young
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