Pediatric Cardiology

, Volume 33, Issue 8, pp 1402–1410 | Cite as

Prenatal Diagnosis of Hypoplastic Left Heart Syndrome: Impact of Counseling Patterns on Parental Perceptions and Decisions Regarding Termination of Pregnancy

  • Debra Hilton-KammEmail author
  • Ruey-Kang Chang
  • Mark Sklansky
Original Article


An online survey for parents of children with congenital heart disease (CHD) was developed to study parents’ experiences at the time of diagnosis. The survey was distributed to online support groups. A total of 841 responses from parents of children with CHD were received during a 4-week period. The current study examined those respondents (211 [25 %]) who reported their child’s diagnosis as hypoplastic left heart syndrome (HLHS). Among these, 138 (65 %) reported receiving the diagnosis prenatally. 32 % of those receiving a prenatal diagnosis reported that after they declined to terminate the pregnancy, termination was mentioned again by their physicians. Parents who had termination mentioned again after their initial decline reported significantly lower optimism regarding their child’s life expectancy than those who did not have it mentioned again (66 vs. 94 %, p < 0.001); were more likely to interpret the term “rare” to mean “little or no chance of survival” (34 vs. 13 %, p = 0.01); and were more likely to change pediatric cardiologists (PCs) (43 vs. 12 %, p < 0.001). Similarly, 22 % of respondents receiving a prenatal diagnosis reported feeling pressure to terminate the pregnancy by the PC. Those who felt pressure to terminate reported lower optimism about their child’s life expectancy than respondents who did not feel pressure (48 vs. 88 %, p < 0.001) and were more likely to choose a new PC (48 vs. 17 %, p < 0.001). In our cohort of parents, when termination of pregnancy was mentioned after the parents declined it, or if the parents felt pressure to terminate, the parents perceived a lower chance of survival, felt less optimistic about their child’s life expectancy, and were more likely to choose another PC for long-term follow-up care. Our study could not determine whether repeated discussions of the possibility for termination of pregnancy independently impacts parental optimism regarding prognosis or whether those who counsel with repeated discussions of termination tend to have more guarded notions of the prognosis of children with HLHS. Further study is warranted to identify the implications of counseling patterns on parental perceptions and decisions regarding termination of pregnancy.


Hypoplastic left heart syndrome Termination Congenital heart disease Survey Parent Perception 



The authors thank Susan Sabanos, market researcher, for help in the survey design; Karin Yospe, cofounder of California Heart Connection, for distribution of the survey; and the many online support groups who distributed the survey to their members and provide much-needed support to families.

Conflict of interest



  1. 1.
    Allan LD, Huggon IC (2004) Counselling following a diagnosis of congenital heart disease. Prenat Diagn 24(13):1136–1142PubMedCrossRefGoogle Scholar
  2. 2.
    Allen RH, Benson CB, Haug LW (2005) Pregnancy outcome of fetuses with a diagnosis of hypoplastic left ventricle on prenatal sonography. J Ultrasound Med 24(9):1199–1203PubMedGoogle Scholar
  3. 3.
    Atz AM, Travison TG, Williams IA, Pearson GD, Laussen PC, Mahle WT et al (2010) Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: screening data from the Pediatric Heart Network Single Ventricle Reconstruction Trial. J Thorac Cardiovasc Surg 140(6):1245–1250PubMedCrossRefGoogle Scholar
  4. 4.
    Bailey LL (2004) Transplantation is the best treatment for hypoplastic left heart syndrome. Cardiol Young 14(Suppl 1):109–111PubMedGoogle Scholar
  5. 5.
    Bove EL (1998) Current status of staged reconstruction for hypoplastic left heart syndrome. Pediatr Cardiol 19:308–315PubMedCrossRefGoogle Scholar
  6. 6.
    Chang RK, Klitzner TS (2002) Can regionalization decrease the number of deaths for children who undergo cardiac surgery? A theoretical analysis. Pediatrics 109(2):173–181PubMedCrossRefGoogle Scholar
  7. 7.
    Checchia PA, McCollegan J, Daher N, Kolovos N, Levy F, Markovitz B (2005) The effect of surgical case volume on outcome after the Norwood procedure. J Thorac Cardiovasc Surg 129:754–759PubMedCrossRefGoogle Scholar
  8. 8.
    Chenni N, Lacroze V, Pouet C, Fraisse A, Kreitmann B, Gamerre M et al (2012) Fetal heart disease and interruption of pregnancy: factors influencing the parental decision-making process. Prenat Diagn 32(2):168–172PubMedCrossRefGoogle Scholar
  9. 9.
    Chrisant MR, Naftel DC, Drummond-Webb J, Chinnock R, Canter CE, Boucek MM et al (2005) Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study. J Heart Lung Transplant 24(5):576–582PubMedCrossRefGoogle Scholar
  10. 10.
    Feinstein JA, Benson DW, Dubin AM, Cohen MS, Maxey DM, Mahle WT et al (2012) Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 59(Suppl 1):S1–S42PubMedCrossRefGoogle Scholar
  11. 11.
    Gaynor JW, Bridges ND, Cohen MI, Mahle WT, Decampli WM, Steven JM et al (2002) Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? J Thorac Cardiovasc Surg 123(2):237–245PubMedCrossRefGoogle Scholar
  12. 12.
    Hirsch JC, Gurney JG, Donohue JE, Gebremariam A, Bove EL, Ohye RG (2008) Hospital mortality for Norwood and arterial switch operations as a function of institutional volume. Pediatr Cardiol 29(4):713–717PubMedCrossRefGoogle Scholar
  13. 13.
    Kon AA, Ackerson L, Lo B (2003) Choices physicians would make if they were the parents of a child with hypoplastic left heart syndrome. Am J Cardiol 91:1506–1509 A9PubMedCrossRefGoogle Scholar
  14. 14.
    Kon AA, Ackerson L, Lo B (2004) How pediatricians counsel parents when no “best-choice” management exists. Arch Pediatr Adolesc Med 158:436–441PubMedCrossRefGoogle Scholar
  15. 15.
    Mahle WT, Spray TL, Wernovsky G, Gaynor JW, Clark BJ (2000) Survival after reconstructive surgery for hypoplastic left heart syndrome: a 15-year experience from a single institution. Circulation 102(19 Suppl 3):III136–III141PubMedGoogle Scholar
  16. 16.
    Mahle WT, Clancy RR, McGaurn SP, Goin JE, Clark BJ (2001) Impact of prenatal diagnosis on survival and early neurologic morbidity in neonates with the hypoplastic left heart syndrome. Pediatrics 107(6):1277–1282PubMedCrossRefGoogle Scholar
  17. 17.
    Marino BS, Tomlinson RS, Drotar D, Claybon ES, Aguirre A, Ittenbach R et al (2009) Quality-of-life concerns differ among patients, parents, and medical providers in children and adolescents with congenital and acquired heart disease. Pediatrics 123(4):708–715CrossRefGoogle Scholar
  18. 18.
    McHugh KE, Hillman DG, Gurka MJ, Gutgesell HP (2010) Three-stage palliation of hypoplastic left heart syndrome in the University Health System Consortium. Congenit Heart Dis 5(1):8–15PubMedCrossRefGoogle Scholar
  19. 19.
    Menahem S, Grimwade J (2005) Pre-natal counselling—Helping couples make decisions following the diagnosis of severe heart disease. Early Hum Dev 81(7):601–607PubMedCrossRefGoogle Scholar
  20. 20.
    Mitchell ME, Ittenbach RF, Gaynor JW, Wernovsky G, Nicolson S, Spray TL (2006) Intermediate outcomes after the Fontan procedure in the current era. J Thorac Cardiovasc Surg 131(1):172–180PubMedCrossRefGoogle Scholar
  21. 21.
    Prsa M, Holly CD, Carnevale F, Justino H, Rohlicek C (2010) Attitudes and practices of cardiologists and surgeons who manage HLHS. Pediatrics 125(3):e625–e630PubMedCrossRefGoogle Scholar
  22. 22.
    Rempel GR, Cender LM, Lynam MJ, Sandor GG, Farquharson D (2004) Parents’ perspectives on decision making after antenatal diagnosis of congenital heart disease. J Obstet Gynecol Neonatal Nurs 33(1):64–70PubMedCrossRefGoogle Scholar
  23. 23.
    Renella P, Chang RK, Ferry DA, Bart RD, Sklansky MS (2007) Hypoplastic left heart syndrome: attitudes among pediatric residents and nurses towards fetal and neonatal management. Prenat Diagn 27(11):1045–1055PubMedCrossRefGoogle Scholar
  24. 24.
    Rychik J, Szwast A, Natarajan S, Quartermain M, Donaghue DD, Combs J et al (2010) Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience. Ultrasound Obstet Gynecol 36(4):465–470PubMedCrossRefGoogle Scholar
  25. 25.
    Sklansky M, Shaughnessy R, Lucas V, Kashani I, Rothman A (2000) A comparison of fetal echocardiography in university and health maintenance organization settings. Pediatr Cardiol 21(3):234–239PubMedCrossRefGoogle Scholar
  26. 26.
    Sklansky M, Tang A, Levy D, Grossfeld P, Kashani I, Shaughnessy R et al (2002) Maternal psychological impact of fetal echocardiography. J Am Soc Echocardiogr 15:159–166PubMedCrossRefGoogle Scholar
  27. 27.
    Tweddell JS, Hoffman GM, Mussatto KA, Fedderly RT, Berger S, Jaquiss RD et al (2002) Improved survival of patients undergoing palliation of hypoplastic left heart syndrome: lessons learned from 115 consecutive patients. Circulation 106:I82–I89PubMedGoogle Scholar
  28. 28.
    Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley FL, Silverman NH (2001) Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome. Circulation 103(9):1269–1273PubMedCrossRefGoogle Scholar
  29. 29.
    Yates AR, Hoffman TM, Boettner B, Feltes TF, Cua CL (2011) Initial counseling prior to palliation for hypoplastic left heart syndrome. Congenit Heart Dis 6(4):347–358PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Debra Hilton-Kamm
    • 1
    Email author
  • Ruey-Kang Chang
    • 2
  • Mark Sklansky
    • 3
  1. 1.California Heart ConnectionIrvineUSA
  2. 2.Harbor-UCLA Medical CenterTorranceUSA
  3. 3.David Geffen School of MedicineUniversity of California Los AngelesLos AngelesUSA

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