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Isolated congenital atrioventricular block diagnosed in utero: natural history and outcome.
J Matern Fetal Neonatal Med. 2008 Jul; 21(7):469-76.JM

Abstract

BACKGROUND

Isolated congenital atrioventricular block (CAVB) diagnosed in utero is associated with a high morbidity and mortality. Prognosis is especially poor when heart rate drops below 55 beats per minute (bpm) and when fetal hydrops develops. We describe the natural history and outcome of 24 infants with isolated CAVB diagnosed in utero, review the literature, and assess the risk factors that could predict outcome.

METHODS

This was a retrospective multicenter study of 24 patients with isolated CAVB diagnosed in utero.

RESULTS

CAVB was detected at a mean gestational age (GA) of 24.7 +/- 5.1 weeks. Ten fetuses initially presented with complete heart block. Low heart rate or incomplete heart block was the first documentation of bradyarrhythmia in the other 14 fetuses. In 11 of them, CAVB developed during pregnancy after a median time of 3 (range 1-16) weeks. Fetal hydrops developed in 10 of 24 (42%) fetuses at a mean GA of 27.6 +/- 5.1 weeks. Hydropic fetuses showed lower heart rates during pregnancy (47 +/- 10 bpm) than non-hydropic fetuses (57 +/- 10 bpm). There were three intrauterine deaths; all were hydropic and female. Nine viable females and 12 males were born at a mean GA of 37.1 +/- 6.1 weeks with an average birth weight of 3097 +/- 852 g. Fifteen CAVB patients required pacemaker (PM) intervention, 10 of them immediately after birth. Dilated cardiomyopathy (DCM) developed in three infants of whom two died of congestive heart failure, shortly after the diagnosis was made; one is still alive. Mortality before or after birth was 21%, and was associated with heart rates below 50 bpm and development of fetal hydrops. Poor outcome, defined as death, PM implantation, or development of DCM, occurred in 83% of cases and was associated with heart rates below 60 bpm during pregnancy.

CONCLUSIONS

Isolated CAVB diagnosed in utero is associated with high morbidity and mortality. Patients who develop fetal hydrops show lower heart rates during pregnancy than patients who do not. A fetal heart rate below 50 bpm and development of fetal hydrops is associated with increased mortality. Rates below 60 bpm are associated with PM requirement and/or DCM.

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Authors+Show Affiliations

Breur JM
Department of Obstetrics, University Medical Center, Utrecht, the Netherlands. hans_breur@hotmail.com
Kapusta L
No affiliation info available
Stoutenbeek P
No affiliation info available
Visser GH
No affiliation info available
van den Berg P
No affiliation info available
Meijboom EJ
No affiliation info available

MeSH

Atrioventricular BlockBradycardiaFemaleHumansHydrops FetalisKaplan-Meier EstimateMalePacemaker, ArtificialPregnancyPrenatal DiagnosisRetrospective Studies

Pub Type(s)

Journal Article
Multicenter Study

Language

eng

PubMed ID

18570127

Citation

Breur, Johannes M P J., et al. "Isolated Congenital Atrioventricular Block Diagnosed in Utero: Natural History and Outcome." The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, vol. 21, no. 7, 2008, pp. 469-76.
Breur JM, Kapusta L, Stoutenbeek P, et al. Isolated congenital atrioventricular block diagnosed in utero: natural history and outcome. J Matern Fetal Neonatal Med. 2008;21(7):469-76.
Breur, J. M., Kapusta, L., Stoutenbeek, P., Visser, G. H., van den Berg, P., & Meijboom, E. J. (2008). Isolated congenital atrioventricular block diagnosed in utero: natural history and outcome. The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 21(7), 469-76. https://doi.org/10.1080/14767050802052786
Breur JM, et al. Isolated Congenital Atrioventricular Block Diagnosed in Utero: Natural History and Outcome. J Matern Fetal Neonatal Med. 2008;21(7):469-76. PubMed PMID: 18570127.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Isolated congenital atrioventricular block diagnosed in utero: natural history and outcome. AU - Breur,Johannes M P J, AU - Kapusta,Livia, AU - Stoutenbeek,Philip, AU - Visser,Gerard H A, AU - van den Berg,Paul, AU - Meijboom,Erik-Jan, PY - 2008/6/24/pubmed PY - 2008/10/25/medline PY - 2008/6/24/entrez SP - 469 EP - 76 JF - The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians JO - J Matern Fetal Neonatal Med VL - 21 IS - 7 N2 - BACKGROUND: Isolated congenital atrioventricular block (CAVB) diagnosed in utero is associated with a high morbidity and mortality. Prognosis is especially poor when heart rate drops below 55 beats per minute (bpm) and when fetal hydrops develops. We describe the natural history and outcome of 24 infants with isolated CAVB diagnosed in utero, review the literature, and assess the risk factors that could predict outcome. METHODS: This was a retrospective multicenter study of 24 patients with isolated CAVB diagnosed in utero. RESULTS: CAVB was detected at a mean gestational age (GA) of 24.7 +/- 5.1 weeks. Ten fetuses initially presented with complete heart block. Low heart rate or incomplete heart block was the first documentation of bradyarrhythmia in the other 14 fetuses. In 11 of them, CAVB developed during pregnancy after a median time of 3 (range 1-16) weeks. Fetal hydrops developed in 10 of 24 (42%) fetuses at a mean GA of 27.6 +/- 5.1 weeks. Hydropic fetuses showed lower heart rates during pregnancy (47 +/- 10 bpm) than non-hydropic fetuses (57 +/- 10 bpm). There were three intrauterine deaths; all were hydropic and female. Nine viable females and 12 males were born at a mean GA of 37.1 +/- 6.1 weeks with an average birth weight of 3097 +/- 852 g. Fifteen CAVB patients required pacemaker (PM) intervention, 10 of them immediately after birth. Dilated cardiomyopathy (DCM) developed in three infants of whom two died of congestive heart failure, shortly after the diagnosis was made; one is still alive. Mortality before or after birth was 21%, and was associated with heart rates below 50 bpm and development of fetal hydrops. Poor outcome, defined as death, PM implantation, or development of DCM, occurred in 83% of cases and was associated with heart rates below 60 bpm during pregnancy. CONCLUSIONS: Isolated CAVB diagnosed in utero is associated with high morbidity and mortality. Patients who develop fetal hydrops show lower heart rates during pregnancy than patients who do not. A fetal heart rate below 50 bpm and development of fetal hydrops is associated with increased mortality. Rates below 60 bpm are associated with PM requirement and/or DCM. SN - 1476-4954 UR - https://www.unboundmedicine.com/medline/citation/18570127/Isolated_congenital_atrioventricular_block_diagnosed_in_utero:_natural_history_and_outcome_ L2 - https://www.tandfonline.com/doi/full/10.1080/14767050802052786 DB - PRIME DP - Unbound Medicine ER -