title: Perinatal outcome in fetuses with heterotaxy syndrome and atrioventricular block or bradycardia.
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.
authors: Department of Cardiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA, 02115, USA, mariaclaraescobar@yahoo.es.
authors: Maria C
authors: MC
authors: Escobar-Diaz
authors: Wayne
authors: W
authors: Tworetzky
authors: Kevin
authors: K
authors: Friedman
authors: Terra
authors: T
authors: Lafranchi
authors: Francis
authors: F
authors: Fynn-Thompson
authors: Mark E
authors: ME
authors: Alexander
authors: Douglas Y
authors: DY
authors: Mah
mesh_terms: D054537:Atrioventricular Block / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D001900:Boston / Q000453:epidemiology; D001919:Bradycardia / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D004452:Echocardiography / Q000379:methods; D004487:Edema / Q000453:epidemiology; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology; D046689:Fetal Mortality; D059446:Heterotaxy Syndrome / Q000150:complications / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology; D006801:Humans; D007223:Infant; D007226:Infant Mortality; D007234:Infant, Premature; D008297:Male; D010138:Pacemaker, Artificial / Q000706:statistics & numerical data*; D011247:Pregnancy; D011256:Pregnancy Outcome; D011296:Prenatal Diagnosis / Q000379:methods / Q000706:statistics & numerical data; D011379:Prognosis; D018570:Risk Assessment; D013154:Spleen / Q000002:abnormalities; D015996:Survival Rate