Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study

Lianne M Geerdink; Gideon J du Marchie Sarvaas; Irene M Kuipers; Willem A Helbing; Tammo Delhaas; Henriette ter Heide; Lieke Rozendaal; Chris L de Korte; Sandeep K Singh; Tjark Ebels; Mark G Hazekamp; Felix Haas; Ad J J C Bogers; Livia Kapusta

doi:10.1111/chd.12404

Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study

Lianne M Geerdink, Gideon J du Marchie Sarvaas, Irene M Kuipers, Willem A Helbing, Tammo Delhaas, Henriette ter Heide, Lieke Rozendaal, Chris L de Korte, Sandeep K Singh, Tjark Ebels, Mark G Hazekamp, Felix Haas, Ad J J C Bogers, Livia Kapusta

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

Abstract

OBJECTIVE: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study.

DESIGN: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.

RESULTS: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown cause. There were no late deaths.

CONCLUSIONS: Surgery in childhood represents the worse spectrum of disease, biventricular repair is often not applied. Patients often face revision surgery. Mortality is limited to the immediate postsurgical period.

Original language	English
Pages (from-to)	32-39
Number of pages	8
Journal	Congenital Heart Disease
Volume	12
Issue number	1
DOIs	https://doi.org/10.1111/chd.12404
Publication status	Published - Feb 2017

Keywords

Ebstein's anomaly
congenital
neonates
outcome
surgery

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10.1111/chd.12404

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Geerdink, L. M., du Marchie Sarvaas, G. J., Kuipers, I. M., Helbing, W. A., Delhaas, T., ter Heide, H., Rozendaal, L., de Korte, C. L., Singh, S. K., Ebels, T., Hazekamp, M. G., Haas, F., Bogers, A. J. J. C., & Kapusta, L. (2017). Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study. Congenital Heart Disease, 12(1), 32-39. https://doi.org/10.1111/chd.12404

@article{d02fc717a6b040ada6c913491bf3575e,

title = "Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study",

abstract = "OBJECTIVE: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study.DESIGN: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.RESULTS: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown cause. There were no late deaths.CONCLUSIONS: Surgery in childhood represents the worse spectrum of disease, biventricular repair is often not applied. Patients often face revision surgery. Mortality is limited to the immediate postsurgical period.",

keywords = "Ebstein's anomaly, congenital, neonates, outcome, surgery",

author = "Geerdink, {Lianne M} and {du Marchie Sarvaas}, {Gideon J} and Kuipers, {Irene M} and Helbing, {Willem A} and Tammo Delhaas and {ter Heide}, Henriette and Lieke Rozendaal and {de Korte}, {Chris L} and Singh, {Sandeep K} and Tjark Ebels and Hazekamp, {Mark G} and Felix Haas and Bogers, {Ad J J C} and Livia Kapusta",

note = "Publisher Copyright: {\textcopyright} 2016 Wiley Periodicals, Inc.",

year = "2017",

month = feb,

doi = "10.1111/chd.12404",

language = "English",

volume = "12",

pages = "32--39",

journal = "Congenital Heart Disease",

issn = "1747-079X",

publisher = "Wiley-Blackwell",

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Geerdink, LM, du Marchie Sarvaas, GJ, Kuipers, IM, Helbing, WA, Delhaas, T, ter Heide, H, Rozendaal, L, de Korte, CL, Singh, SK, Ebels, T, Hazekamp, MG, Haas, F, Bogers, AJJC & Kapusta, L 2017, 'Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study', Congenital Heart Disease, vol. 12, no. 1, pp. 32-39. https://doi.org/10.1111/chd.12404

TY - JOUR

T1 - Surgical outcome in pediatric patients with Ebstein's anomaly

T2 - A multicenter, long-term study

AU - Geerdink, Lianne M

AU - du Marchie Sarvaas, Gideon J

AU - Kuipers, Irene M

AU - Helbing, Willem A

AU - Delhaas, Tammo

AU - ter Heide, Henriette

AU - Rozendaal, Lieke

AU - de Korte, Chris L

AU - Singh, Sandeep K

AU - Ebels, Tjark

AU - Hazekamp, Mark G

AU - Haas, Felix

AU - Bogers, Ad J J C

AU - Kapusta, Livia

PY - 2017/2

Y1 - 2017/2

N2 - OBJECTIVE: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study.DESIGN: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.RESULTS: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown cause. There were no late deaths.CONCLUSIONS: Surgery in childhood represents the worse spectrum of disease, biventricular repair is often not applied. Patients often face revision surgery. Mortality is limited to the immediate postsurgical period.

AB - OBJECTIVE: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study.DESIGN: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed. Demographic variables, intraoperative procedures and postoperative outcomes were analyzed.RESULTS: Sixty-three patients underwent 109 operations. Median follow-up after diagnosis was 121 months (range 0-216 months). Twenty-nine (46%) patients required surgery in the first year of life, including 21 who required neonatal surgery. The intended treatment was biventricular (n = 37, 59%) and 1.5 ventricle (n = 5, 8%) repair or univentricular (n = 21, 33%) palliation. The one-, five-, and 10-year freedom from unplanned reoperation was 89%, 79%, and 75% respectively. There were nine (14%) in hospital deaths (within 30 d after surgery). Causes of death were low cardiac output syndrome, cardiac failure, hypoxemia, pulmonary hypertension or an unknown cause. There were no late deaths.CONCLUSIONS: Surgery in childhood represents the worse spectrum of disease, biventricular repair is often not applied. Patients often face revision surgery. Mortality is limited to the immediate postsurgical period.

KW - Ebstein's anomaly

KW - congenital

KW - neonates

KW - outcome

KW - surgery

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U2 - 10.1111/chd.12404

DO - 10.1111/chd.12404

M3 - Article

C2 - 27558473

SN - 1747-079X

VL - 12

SP - 32

EP - 39

JO - Congenital Heart Disease

JF - Congenital Heart Disease

IS - 1

ER -

Surgical outcome in pediatric patients with Ebstein's anomaly: A multicenter, long-term study

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Keywords

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