Pediatric Cardiology

, Volume 35, Issue 6, pp 906–913

Perinatal Outcome in Fetuses with Heterotaxy Syndrome and Atrioventricular Block or Bradycardia

  • Maria C. Escobar-Diaz
  • Wayne Tworetzky
  • Kevin Friedman
  • Terra Lafranchi
  • Francis Fynn-Thompson
  • Mark E. Alexander
  • Douglas Y. Mah
Original Article

Abstract

Congenital atrioventricular (AV) block is commonly associated with heterotaxy syndrome; together they have reportedly low survival rates (10–25 %). However, information about perinatal outcome and predictors of non-survival after prenatal diagnosis of this association is scarce. Therefore, we studied fetuses with heterotaxy syndrome and bradycardia or AV-block diagnosed between 1995 and 2011, and analyzed pre and post-natal variables. The primary outcome was death and the secondary outcome was pacemaker placement. Of the 154 fetuses with heterotaxy syndrome, 91 had polysplenia syndrome, 22/91(24 %) with bradycardia or AV-block. Thirteen (59 %) patients had sinus bradycardia at diagnosis, 8 (36 %) complete AV block, and 1 (5 %) second-degree AV-block. Three patients elected for termination of pregnancy (3/22, 14 %), 4 had spontaneous fetal demise (4/22, 18 %), and 15 (15/22, 68 %) were live-born. Of the fetuses with bradycardia/AV-block, 30 % presented with hydrops, 20 % had ventricular rates <55 beats/min, and 10 % had cardiac dysfunction. Excluding termination of pregnancy, 15/19 fetuses (79 %) survived to birth. Among the 15 live-born patients, 4 had bradycardia and 11 had AV-block. A further 3 patients died in infancy, all with AV-block who required pacemakers in the neonatal period. Thus, the 1-year survival rate, excluding termination of pregnancy, was 63 % (12/19). Of the remaining 12 patients, 9 required pacemaker. Predictors of perinatal death included hydrops (p < 0.0001), ventricular dysfunction (p = 0.002), prematurity (p = 0.04), and low ventricular rates (p = 0.04). In conclusion, we found a higher survival rate (63 %) than previously published in patients with heterotaxy syndrome and AV block or bradycardia diagnosed prenatally. Hydrops, cardiac dysfunction, prematurity and low ventricular rates were predictors of death.

Keywords

Prenatal diagnosis Heterotaxy syndrome Atrioventricular block Bradycardia 

References

  1. 1.
    Berg C, Geipel A, Smrcek J, Krapp M, Germer U, Kohl T et al (2003) Prenatal diagnosis of cardiosplenic syndromes: a 10-year experience. Ultrasound Obstet Gynecol 22(5):451–459CrossRefPubMedGoogle Scholar
  2. 2.
    Berg C, Geipel A, Kohl T, Breuer J, Germer U, Krapp M et al (2005) Atrioventricular block detected in fetal life: associated anomalies and potential prognostic markers. Ultrasound Obstet Gynecol 26(1):4–15CrossRefPubMedGoogle Scholar
  3. 3.
    Berg C, Geipel A, Kamil D, Knuppel M, Breuer J, Krapp M et al (2005) The syndrome of left isomerism: sonographic findings and outcome in prenatally diagnosed cases. J Ultrasound Med 24(7):921–931PubMedGoogle Scholar
  4. 4.
    Buyon JP, Hiebert R, Copel J, Craft J, Friedman D, Katholi M et al (1998) Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol 31(7):1658–1666CrossRefPubMedGoogle Scholar
  5. 5.
    Cohen MS, Schultz AH, Tian ZY, Donaghue DD, Weinberg PM, Gaynor JW et al (2006) Heterotaxy syndrome with functional single ventricle: does prenatal diagnosis improve survival? Ann Thorac Surg 82(5):1629–1636CrossRefPubMedGoogle Scholar
  6. 6.
    Dickinson DF, Wilkinson JL, Anderson KR, Smith A, Ho SY, Anderson RH (1979) The cardiac conduction system in situs ambiguus. Circulation 59(5):879–885CrossRefPubMedGoogle Scholar
  7. 7.
    Eronen M, Siren MK, Ekblad H, Tikanoja T, Julkunen H, Paavilainen T (2000) Short- and long-term outcome of children with congenital complete heart block diagnosed in utero or as a newborn. Pediatrics 106(1 Pt 1):86–91CrossRefPubMedGoogle Scholar
  8. 8.
    Eronen M, Heikkila P, Teramo K (2001) Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment, and outcome in six cases. Pediatr Cardiol 22(5):385–392CrossRefPubMedGoogle Scholar
  9. 9.
    Gembruch U, Hansmann M, Redel DA, Bald R, Knopfle G (1989) Fetal complete heart block: antenatal diagnosis, significance and management. Eur J Obstet Gynecol Reprod Biol 31(1):9–22CrossRefPubMedGoogle Scholar
  10. 10.
    Glatz AC, Gaynor JW, Rhodes LA, Rychik J, Tanel RE, Vetter VL et al (2008) Outcome of high-risk neonates with congenital complete heart block paced in the first 24 h after birth. J Thorac Cardiovasc Surg 136(3):767–773CrossRefPubMedGoogle Scholar
  11. 11.
    Gottschalk I, Berg C, Heller R (2012) Disorders of laterality and heterotaxy in the foetus. Z Geburtshilfe Neonatol 216(3):122–131CrossRefPubMedGoogle Scholar
  12. 12.
    Ho SY, Cook A, Anderson RH, Allan LD, Fagg N (1991) Isomerism of the atrial appendages in the fetus. Pediatr Pathol 11(4):589–608CrossRefPubMedGoogle Scholar
  13. 13.
    Ho SY, Fagg N, Anderson RH, Cook A, Allan L (1992) Disposition of the atrioventricular conduction tissues in the heart with isomerism of the atrial appendages: its relation to congenital complete heart block. J Am Coll Cardiol 20(4):904–910CrossRefPubMedGoogle Scholar
  14. 14.
    Jacobs JP, Anderson RH, Weinberg PM, Walters HL 3rd, Tchervenkov CI, Del Duca D et al (2007) The nomenclature, definition and classification of cardiac structures in the setting of heterotaxy. Cardiol Young 17(Suppl 2):1–28PubMedGoogle Scholar
  15. 15.
    Jaeggi ET, Hamilton RM, Silverman ED, Zamora SA, Hornberger LK (2002) Outcome of children with fetal, neonatal or childhood diagnosis of isolated congenital atrioventricular block. A single institution’s experience of 30 years. J Am Coll Cardiol 39(1):130–137CrossRefPubMedGoogle Scholar
  16. 16.
    Jaeggi ET, Hornberger LK, Smallhorn JF, Fouron JC (2005) Prenatal diagnosis of complete atrioventricular block associated with structural heart disease: combined experience of two tertiary care centers and review of the literature. Ultrasound Obstet Gynecol 26(1):16–21CrossRefPubMedGoogle Scholar
  17. 17.
    Lim JS, McCrindle BW, Smallhorn JF, Golding F, Caldarone CA, Taketazu M et al (2005) Clinical features, management, and outcome of children with fetal and postnatal diagnoses of isomerism syndromes. Circulation 112(16):2454–2461CrossRefPubMedGoogle Scholar
  18. 18.
    Lin AE, Ticho BS, Houde K, Westgate MN, Holmes LB (2000) Heterotaxy: associated conditions and hospital-based prevalence in newborns. Genet Med 2(3):157–172CrossRefPubMedGoogle Scholar
  19. 19.
    Lin JH, Chang CI, Wang JK, Wu MH, Shyu MK, Lee CN et al (2002) Intrauterine diagnosis of heterotaxy syndrome. Am Heart J 143(6):1002–1008CrossRefPubMedGoogle Scholar
  20. 20.
    Lopes LM, Tavares GM, Damiano AP, Lopes MA, Aiello VD, Schultz R et al (2008) Perinatal outcome of fetal atrioventricular block: one-hundred-sixteen cases from a single institution. Circulation 118(12):1268–1275CrossRefPubMedGoogle Scholar
  21. 21.
    Machado MV, Tynan MJ, Curry PV, Allan LD (1988) Fetal complete heart block. Br Heart J 60(6):512–515PubMedCentralCrossRefPubMedGoogle Scholar
  22. 22.
    Maeno Y, Himeno W, Saito A, Hiraishi S, Hirose O, Ikuma M et al (2005) Clinical course of fetal congenital atrioventricular block in the Japanese population: a multicentre experience. Heart 91(8):1075–1079PubMedCentralCrossRefPubMedGoogle Scholar
  23. 23.
    Michaelsson M, Engle MA (1972) Congenital complete heart block: an international study of the natural history. Cardiovasc Clin 4(3):85–101PubMedGoogle Scholar
  24. 24.
    Pepes S, Zidere V, Allan LD (2009) Prenatal diagnosis of left atrial isomerism. Heart 95(24):1974–1977CrossRefPubMedGoogle Scholar
  25. 25.
    Phoon CK, Villegas MD, Ursell PC, Silverman NH (1996) Left atrial isomerism detected in fetal life. Am J Cardiol 77(12):1083–1088CrossRefPubMedGoogle Scholar
  26. 26.
    Schmidt KG, Ulmer HE, Silverman NH, Kleinman CS, Copel JA (1991) Perinatal outcome of fetal complete atrioventricular block: a multicenter experience. J Am Coll Cardiol 17(6):1360–1366CrossRefPubMedGoogle Scholar
  27. 27.
    Swaminathan S, Parthiban A (2007) Progressive fetal atrioventricular block in heterotaxy syndrome. Cardiol Young 17(4):432–434CrossRefPubMedGoogle Scholar
  28. 28.
    Taketazu M, Lougheed J, Yoo SJ, Lim JS, Hornberger LK (2006) Spectrum of cardiovascular disease, accuracy of diagnosis, and outcome in fetal heterotaxy syndrome. Am J Cardiol 97(5):720–724CrossRefPubMedGoogle Scholar
  29. 29.
    Van Praagh S, Santini F, Sanders S (eds) (1992) Cardiac malpositions with special emphasis on visceral heterotaxy (asplenia and polysplenia syndromes). Nadas pediatric cardiology. Hanley & Belfus/Mosby, Philadelphia, pp 589–608Google Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Maria C. Escobar-Diaz
    • 1
    • 3
  • Wayne Tworetzky
    • 1
    • 3
  • Kevin Friedman
    • 1
    • 3
  • Terra Lafranchi
    • 1
    • 3
  • Francis Fynn-Thompson
    • 2
    • 4
  • Mark E. Alexander
    • 1
    • 3
  • Douglas Y. Mah
    • 1
    • 3
  1. 1.Department of CardiologyBoston Children’s HospitalBostonUSA
  2. 2.Department of Cardiac SurgeryBoston Children’s HospitalBostonUSA
  3. 3.Department of PediatricsHarvard Medical SchoolBostonUSA
  4. 4.Department of SurgeryHarvard Medical SchoolBostonUSA

Personalised recommendations