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

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 studie...

Full description

Saved in:
Bibliographic Details
Published in:Pediatric cardiology 2014-08, Vol.35 (6), p.906-913
Main Authors: Escobar-Diaz, Maria C., Tworetzky, Wayne, Friedman, Kevin, Lafranchi, Terra, Fynn-Thompson, Francis, Alexander, Mark E., Mah, Douglas Y.
Format: Article
Language:English
Subjects:
Analysis
Atrioventricular Block - diagnosis
Atrioventricular Block - etiology
Atrioventricular Block - mortality
Atrioventricular Block - physiopathology
Atrioventricular Block - therapy
Boston - epidemiology
Bradycardia
Bradycardia - diagnosis
Bradycardia - etiology
Bradycardia - mortality
Bradycardia - physiopathology
Bradycardia - therapy
Cardiac Surgery
Cardiology
Echocardiography - methods
Edema - epidemiology
Female
Fetal Diseases - diagnosis
Fetal Diseases - mortality
Fetal Diseases - physiopathology
Fetal Mortality
Genetic disorders
Heterotaxy Syndrome - complications
Heterotaxy Syndrome - diagnosis
Heterotaxy Syndrome - mortality
Heterotaxy Syndrome - physiopathology
Humans
Infant
Infant Mortality
Infant, Premature
Infants
Male
Medical research
Medicine
Medicine & Public Health
Medicine, Experimental
Original Article
Pacemaker, Artificial - statistics & numerical data
Patient outcomes
Pregnancy
Pregnancy Outcome
Pregnant women
Prenatal Diagnosis - methods
Prenatal Diagnosis - statistics & numerical data
Prognosis
Risk Assessment
Spleen - abnormalities
Survival Rate
Vascular Surgery
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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
ISSN:0172-0643
1432-1971
DOI:10.1007/s00246-014-0874-x