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Pediatric Cardiology

, Volume 40, Issue 1, pp 61–70 | Cite as

Prenatal Diagnosis of Single Ventricle Physiology Impacts on Cardiac Morbidity and Mortality

  • Roland W. WeberEmail author
  • Brian Stiasny
  • Beate Ruecker
  • Margrit Fasnacht
  • Anna Cavigelli-Brunner
  • Emanuela R. Valsangiacomo Buechel
Original Article

Abstract

We sought to evaluate the impact of prenatal diagnosis on morbidity and mortality in single ventricle (SV) lesions. All consecutive patients with pre- or postnatally diagnosed SV physiology admitted to our centre between January 2001 and June 2013 were reviewed. Primary endpoints included survival until 30 days after bidirectional cavopulmonary connection (BCPC) without transplant or BCPC takedown. Prenatal diagnosis was performed in 160 of 259 cases (62%). After excluding all cases with termination of pregnancy, intrauterine demise or treated with comfort care, a total of 180 neonates were admitted to our centre for treatment, including 87 with a prenatal and 93 with a postnatal diagnosis. Both groups showed similar distribution regarding diagnosis, dominant ventricle and risk factors such as restrictive foramen or some form of atrial isomerism. A larger proportion of postnatally diagnosed children presented at admission with elevated lactate > 10 mmol/l (p = 0.02), a higher dose of prostaglandin (p = 0.0013) and need for mechanical ventilation (p < 0.0001). Critical lesions such as hypoplastic left heart syndrome were an important determinant for morbidity and mortality. Thirty-days survival after BCPC was better in patients with prenatal diagnosis (p = 0.025). Prenatal diagnosis is associated with higher survival in neonates with SV physiology.

Keywords

Foetal echocardiography Single ventricle Prenatal diagnosis Outcome 

Notes

Acknowledgements

We would like to thank Dr. Janet F. Kelly from the department of intensive care at the University children’s hospital of Zurich for her constructive and critical review of this manuscript as a native speaker.

Compliance with Ethical Standards

Conflict of interest

None of the authors has a conflict of interest to disclose. There is no relationship with industry or funding sources. All authors were involved in the study design, manuscript preparation, data collection and data analysis.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Supplementary material

246_2018_1961_MOESM1_ESM.docx (22 kb)
Supplementary material 1 (DOCX 21 KB)

References

  1. 1.
    Allan LD, Sharland GK, Milburn A, Lockhart SM, Groves AM, Anderson RH, Cook AC, Fagg NL (1994) Prospective diagnosis of 1,006 consecutive cases of congenital heart disease in the fetus. J Am Coll Cardiol 23(6):1452–1458CrossRefGoogle Scholar
  2. 2.
    Copel JA, Tan AS, Kleinman CS (1997) Does a prenatal diagnosis of congenital heart disease alter short-term outcome? Ultrasound Obstet Gynecol 10(4):237–241CrossRefGoogle Scholar
  3. 3.
    Jaeggi ET, Sholler GF, Jones OD, Cooper SG (2001) Comparative analysis of pattern, management and outcome of pre- versus postnatally diagnosed major congenital heart disease: a population-based study. Ultrasound Obstet Gynecol 17(5):380–385CrossRefGoogle Scholar
  4. 4.
    Khoshnood B, De Vigan C, Vodovar V, Goujard J, Lhomme A, Bonnet D, Goffinet F (2005) Trends in prenatal diagnosis, pregnancy termination, and perinatal mortality of newborns with congenital heart disease in France, 1983–2000: a population-based evaluation. Pediatrics 115(1):95–101CrossRefGoogle Scholar
  5. 5.
    Friedberg MK, Silverman NH, Moon-Grady AJ, Tong E, Nourse J, Sorenson B, Lee J, Hornberger LK (2009) Prenatal detection of congenital heart disease. J Pediatr 155(1):26–31, 31.e1CrossRefGoogle Scholar
  6. 6.
    Holland BJ, Myers JA, Woods CR Jr (2015) Prenatal diagnosis of critical congenital heart disease reduces risk of death from cardiovascular compromise prior to planned neonatal cardiac surgery: a meta-analysis. Ultrasound Obstet Gynecol 45(6):631–638CrossRefGoogle Scholar
  7. 7.
    Morris SA, Ethen MK, Penny DJ, Canfield MA, Minard CG, Fixler DE, Nembhard WN (2014) Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome. Circulation 129(3):285–292CrossRefGoogle Scholar
  8. 8.
    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):1277–1282CrossRefGoogle Scholar
  9. 9.
    Tworetzky W, McElhinney DB, Reddy VM, Brook MM, Hanley FL, Silverman NH (2001) Improved surgical outcome after fetal diagnosis of hypo-plastic left heart syndrome. Circulation 103:1269–1273CrossRefGoogle Scholar
  10. 10.
    Oster ME, Kim CH, Kusano AS, Cragan JD, Dressler P, Hales AR, Mahle WT, Correa A (2014) A population-based study of the association of prenatal diagnosis with survival rate for infants with congenital heart defects. Am J Cardiol 113(6):1036–1040CrossRefGoogle Scholar
  11. 11.
    Sivarajan V, Penny DJ, Filan P, Brizard C, Shekerdemian LS (2009) Impact of antenatal diagnosis of hypoplastic left heart syndrome on the clinical presentation and surgical outcomes: the Australian experience. J Paediatr Child Health 45(3):112–117CrossRefGoogle Scholar
  12. 12.
    Beroukhim RS, Gauvreau K, Benavidez OJ, Baird CW, LaFranchi T, Tworetzky W (2015) Perinatal outcome after prenatal diagnosis of single-ventricle cardiac defects. Ultrasound Obstet Gynecol 45(6):657–663CrossRefGoogle Scholar
  13. 13.
    d’Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, Bullock A, Justo RN, Grigg LE, Sholler GF, Hope S, Radford DJ, Gentles TL, Celermajer DS, Winlaw DS (2014) Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation 130(11 Suppl 1):S32–S38CrossRefGoogle Scholar
  14. 14.
    Pruetz JD, Carroll C, Trento LU, Chang RK, Detterich J, Miller DA, Sklansky M (2014) Outcomes of critical congenital heart disease requiring emergent neonatal cardiac intervention. Prenat Diagn 34(12):1127–1132CrossRefGoogle Scholar
  15. 15.
    Wald RM, Tham EB, McCrindle BW, Goff DA, McAuliffe FM, Golding F, Jaeggi ET, Hornberger LK, Tworetzky W, Nield LE (2007) Outcome after prenatal diagnosis of tricuspid atresia: a multicenter experience. Am Heart J 153(5):772–778CrossRefGoogle Scholar
  16. 16.
    Cohen MS, Schultz AH, Tian ZY, Donaghue DD, Weinberg PM, Gaynor JW, Rychik J (2006) Heterotaxy syndrome with functional single ventricle: does prenatal diagnosis improve survival? Ann Thorac Surg 82(5):1629–1636CrossRefGoogle Scholar
  17. 17.
    Bull C, on behalf of the British Paediatric Cardiac Association (1999) Current and potential impact of fetal diagnosis on the prevalence and spectrum of serious congenital heart disease at term. Lancet 354:1242–1247CrossRefGoogle Scholar
  18. 18.
    Landis BJ, Levey A, Levasseur SM, Glickstein JS, Kleinman CS, Simpson LL, Williams IA (2013) Prenatal diagnosis of congenital heart disease and birth outcomes. Pediatr Cardiol 34(3):597–605CrossRefGoogle Scholar
  19. 19.
    Fountain-Dommer RR, Bradley SM, Atz AM, Stroud MR, Forbus GA, Shirali GS (2004) Outcome following, and impact of, prenatal identification of the candidates for the Norwood procedure. Cardiol Young 14(1):32–38CrossRefGoogle Scholar
  20. 20.
    Galindo A, Nieto O, Villagrá S, Grañeras A, Herraiz I, Mendoza A (2009) Hypoplastic left heart syndrome diagnosed in fetal life: associated findings, pregnancy outcome and results of palliative surgery. Ultrasound Obstet Gynecol 33(5):560–566CrossRefGoogle Scholar
  21. 21.
    Tibballs J, Cantwell-Bartl A (2008) 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 44(6):321–324CrossRefGoogle Scholar
  22. 22.
    Marek J, Tomek V, Skovránek J, Povysilová V, Samánek M (2011) Prenatal ultrasound screening of congenital heart disease in an unselected national population: a 21-year experience. Heart 97(2):124–130CrossRefGoogle Scholar
  23. 23.
    Kuelling B, Arlettaz Mieth R, Bauersfeld U, Balmer C (2009) Pulse oximetry screening for congenital heart defects in Switzerland: most but not all maternity units screen their neonates. Swiss Med Wkly 139(47–48):699–704.Google Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Pediatric Heart CentreUniversity Children’s HospitalZurichSwitzerland
  2. 2.Children’s HospitalZurichSwitzerland
  3. 3.Children’s Research CentreZurichSwitzerland

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