Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections

Thomas G. Day; Tomas Woodgate; Olatejumoye Knee; Vita Zidere; Trisha Vigneswaran; Marietta Charakida; Owen Miller; Gurleen Sharland; John Simpson

doi:10.1007/s00246-019-02176-2

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections

Thomas G. Day, Tomas Woodgate, Olatejumoye Knee, Vita Zidere, Trisha Vigneswaran, Marietta Charakida, Owen Miller, Gurleen Sharland, John Simpson^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › peer-review

5 Citations (Scopus)

Abstract

Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74–86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein’s anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein’s anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

Original language	English
Pages (from-to)	1509-1515
Number of pages	7
Journal	Pediatric Cardiology
Volume	40
Issue number	7
Early online date	25 Jul 2019
DOIs	https://doi.org/10.1007/s00246-019-02176-2
Publication status	Published - 1 Oct 2019

Keywords

Congenitally corrected transposition of the great arteries
Discordant atrioventricular and ventriculoarterial connections
Fetal cardiology

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10.1007/s00246-019-02176-2

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@article{cb0330ad531e4b719f32d5a92a5426c4,

title = "Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections",

abstract = "Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74–86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein{\textquoteright}s anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein{\textquoteright}s anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.",

keywords = "Congenitally corrected transposition of the great arteries, Discordant atrioventricular and ventriculoarterial connections, Fetal cardiology",

author = "Day, {Thomas G.} and Tomas Woodgate and Olatejumoye Knee and Vita Zidere and Trisha Vigneswaran and Marietta Charakida and Owen Miller and Gurleen Sharland and John Simpson",

year = "2019",

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doi = "10.1007/s00246-019-02176-2",

language = "English",

volume = "40",

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journal = "Pediatric Cardiology",

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T1 - Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections

AU - Day, Thomas G.

AU - Woodgate, Tomas

AU - Knee, Olatejumoye

AU - Zidere, Vita

AU - Vigneswaran, Trisha

AU - Charakida, Marietta

AU - Miller, Owen

AU - Sharland, Gurleen

AU - Simpson, John

PY - 2019/10/1

Y1 - 2019/10/1

N2 - Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74–86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein’s anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein’s anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

AB - Discordant atrioventricular and ventriculoarterial connection(s) (DAVVAC) are a rare group of congenital heart lesions. DAVVAC can be isolated or associated with a variety of other cardiac abnormalities. Previous studies examining the outcome of prenatally diagnosed DAVVAC have described only fetal and early postnatal outcome in small cohorts. We aimed to describe the medium-term outcome of these fetuses. Cases were identified by searching the fetal cardiac databases of two centers. Follow-up data were collected from the electronic patient records. We identified 98 fetuses with DAVVAC. 39 pregnancies were terminated and 51 resulted in a liveborn infant. Postnatal data were available for 43 patients. The median length of follow-up was 9.5 years (range 36 days to 22.7 years). The overall 5-year survival of the cohort was 80% (95% confidence interval 74–86%), no deaths were seen after this period. Associated cardiac lesions had a significant effect on both survival and surgery-free survival. Isolated DAVVAC and DAVVAC with pulmonary stenosis ± ventricular septal defect had a low mortality (89% and 100% 5-year survival, respectively). Poorer survival was seen in the group with Ebstein’s anomaly of the tricuspid valve, and other complex cardiac abnormalities. Antenatal tricuspid regurgitation had a significant negative impact on postnatal survival. In conclusion, the short- and medium-term outlook for fetuses with isolated DAVVAC, and those with DAVVAC and pulmonary stenosis are good. Antenatal risk factors for postnatal mortality include Ebstein’s anomaly of the tricuspid valve, especially if associated with tricuspid regurgitation, and the presence of complex associated lesions.

KW - Congenitally corrected transposition of the great arteries

KW - Discordant atrioventricular and ventriculoarterial connections

KW - Fetal cardiology

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U2 - 10.1007/s00246-019-02176-2

DO - 10.1007/s00246-019-02176-2

M3 - Article

AN - SCOPUS:85069645191

SN - 0172-0643

VL - 40

SP - 1509

EP - 1515

JO - Pediatric Cardiology

JF - Pediatric Cardiology

IS - 7

ER -

Postnatal Outcome Following Prenatal Diagnosis of Discordant Atrioventricular and Ventriculoarterial Connections

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