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


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.


Prenatal diagnosis Heterotaxy syndrome Atrioventricular block Bradycardia 


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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

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