Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study

Livia Kapusta; Rhona M. Eveleigh; Sumayah E. Poulino; Marry E. Rijlaarsdam; Gideon J. du Marchie Sarvaas; Jan L. Strengers; Tammo Delhaas; Chris L. de Korte; Ton Feuth; William A. Helbing

doi:10.1093/eurheartj/ehm398

Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study

Livia Kapusta^*
, Rhona M. Eveleigh
, Sumayah E. Poulino
, Marry E. Rijlaarsdam
, Gideon J. du Marchie Sarvaas
, Jan L. Strengers
, Tammo Delhaas
, Chris L. de Korte
, Ton Feuth
, William A. Helbing

^*Corresponding author for this work

Faculty of Medical Sciences/UMCG

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

33 Citations (Scopus)

Abstract

Aims The objective of this study is to establish factors associated with death after diagnosis of Ebstein's anomaly (EA) during childhood and adolescence.

Methods and results This study is a retrospective chart review. All paediatric patients were diagnosed with EA and followed in tertiary-care university hospitals between 1980 and 2005. Factors associated with death were obtained using the Cox regression and log-rank tests. Of the 93 patients with EA, 18 (19%) died and 75 (81%) survived. The median age at EA diagnosis and follow-up was 0 (range 0-162) and 86 months (range 0-216), respectively. After 35 months of diagnosis, the Kaplan-Meier survival probability remains stable at 80%. Young age at presentation (

Conclusion The overall survival of patients with EA during childhood and adolescence has dramatically improved when compared with earlier reports.

Original language	English
Pages (from-to)	2661-2666
Number of pages	6
Journal	European Heart Journal
Volume	28
Issue number	21
DOIs	https://doi.org/10.1093/eurheartj/ehm398
Publication status	Published - Nov-2007

Keywords

Ebstein
children
risk factors
death
survival
TRICUSPID-VALVE
NATURAL-HISTORY
CLINICAL PROFILE
SURVIVAL
ADULT
FETUS
NEONATE

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@article{cd7c8b02806f4302beb6b671e410d1cc,

title = "Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study",

abstract = "Aims The objective of this study is to establish factors associated with death after diagnosis of Ebstein's anomaly (EA) during childhood and adolescence.Methods and results This study is a retrospective chart review. All paediatric patients were diagnosed with EA and followed in tertiary-care university hospitals between 1980 and 2005. Factors associated with death were obtained using the Cox regression and log-rank tests. Of the 93 patients with EA, 18 (19\%) died and 75 (81\%) survived. The median age at EA diagnosis and follow-up was 0 (range 0-162) and 86 months (range 0-216), respectively. After 35 months of diagnosis, the Kaplan-Meier survival probability remains stable at 80\%. Young age at presentation (Conclusion The overall survival of patients with EA during childhood and adolescence has dramatically improved when compared with earlier reports.",

keywords = "Ebstein, children, risk factors, death, survival, TRICUSPID-VALVE, NATURAL-HISTORY, CLINICAL PROFILE, SURVIVAL, ADULT, FETUS, NEONATE",

author = "Livia Kapusta and Eveleigh, \{Rhona M.\} and Poulino, \{Sumayah E.\} and Rijlaarsdam, \{Marry E.\} and Sarvaas, \{Gideon J. du Marchie\} and Strengers, \{Jan L.\} and Tammo Delhaas and \{de Korte\}, \{Chris L.\} and Ton Feuth and Helbing, \{William A.\}",

year = "2007",

month = nov,

doi = "10.1093/eurheartj/ehm398",

language = "English",

volume = "28",

pages = "2661--2666",

journal = "European Heart Journal",

issn = "0195-668X",

publisher = "Oxford University Press",

number = "21",

}

TY - JOUR

T1 - Ebstein's anomaly

T2 - factors associated with death in childhood and adolescence: a multi-centre, long-term study

AU - Kapusta, Livia

AU - Eveleigh, Rhona M.

AU - Poulino, Sumayah E.

AU - Rijlaarsdam, Marry E.

AU - Sarvaas, Gideon J. du Marchie

AU - Strengers, Jan L.

AU - Delhaas, Tammo

AU - de Korte, Chris L.

AU - Feuth, Ton

AU - Helbing, William A.

PY - 2007/11

Y1 - 2007/11

N2 - Aims The objective of this study is to establish factors associated with death after diagnosis of Ebstein's anomaly (EA) during childhood and adolescence.Methods and results This study is a retrospective chart review. All paediatric patients were diagnosed with EA and followed in tertiary-care university hospitals between 1980 and 2005. Factors associated with death were obtained using the Cox regression and log-rank tests. Of the 93 patients with EA, 18 (19%) died and 75 (81%) survived. The median age at EA diagnosis and follow-up was 0 (range 0-162) and 86 months (range 0-216), respectively. After 35 months of diagnosis, the Kaplan-Meier survival probability remains stable at 80%. Young age at presentation (Conclusion The overall survival of patients with EA during childhood and adolescence has dramatically improved when compared with earlier reports.

AB - Aims The objective of this study is to establish factors associated with death after diagnosis of Ebstein's anomaly (EA) during childhood and adolescence.Methods and results This study is a retrospective chart review. All paediatric patients were diagnosed with EA and followed in tertiary-care university hospitals between 1980 and 2005. Factors associated with death were obtained using the Cox regression and log-rank tests. Of the 93 patients with EA, 18 (19%) died and 75 (81%) survived. The median age at EA diagnosis and follow-up was 0 (range 0-162) and 86 months (range 0-216), respectively. After 35 months of diagnosis, the Kaplan-Meier survival probability remains stable at 80%. Young age at presentation (Conclusion The overall survival of patients with EA during childhood and adolescence has dramatically improved when compared with earlier reports.

KW - Ebstein

KW - children

KW - risk factors

KW - death

KW - survival

KW - TRICUSPID-VALVE

KW - NATURAL-HISTORY

KW - CLINICAL PROFILE

KW - SURVIVAL

KW - ADULT

KW - FETUS

KW - NEONATE

U2 - 10.1093/eurheartj/ehm398

DO - 10.1093/eurheartj/ehm398

M3 - Article

SN - 0195-668X

VL - 28

SP - 2661

EP - 2666

JO - European Heart Journal

JF - European Heart Journal

IS - 21

ER -

Ebstein's anomaly: factors associated with death in childhood and adolescence: a multi-centre, long-term study

Abstract

Keywords

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