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Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood

OBJECTIVE: Left ventricular systolic dysfunction (LVSD) in adult survivors of tetralogy of Fallot (TOF) repair in childhood has been observed, although the relationship with long-term outcome remains inadequately described. METHODS: A cohort of 44 consecutive adult patients with TOF repair in childh...

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Autores principales: Anabtawi, Abdel, Mondragon, Judith, Dodendorf, Diane, Laskey, Warren K
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5708319/
https://www.ncbi.nlm.nih.gov/pubmed/29225904
http://dx.doi.org/10.1136/openhrt-2017-000690
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author Anabtawi, Abdel
Mondragon, Judith
Dodendorf, Diane
Laskey, Warren K
author_facet Anabtawi, Abdel
Mondragon, Judith
Dodendorf, Diane
Laskey, Warren K
author_sort Anabtawi, Abdel
collection PubMed
description OBJECTIVE: Left ventricular systolic dysfunction (LVSD) in adult survivors of tetralogy of Fallot (TOF) repair in childhood has been observed, although the relationship with long-term outcome remains inadequately described. METHODS: A cohort of 44 consecutive adult patients with TOF repair in childhood were followed prospectively from January 2001 through June 2016. LVSD was defined as an echocardiographically derived left ventricular (LV) ejection fraction <0.55. Clinical and demographic characteristics in patients with and without LVSD were compared. Event-free survival (all-cause death or hospitalisation) was estimated using the product limit method. RESULTS: The average time from childhood surgical repair to cohort inception was similar between groups (LVSD, 33.7±12.7 years; normal LV function, 36.1±14.9 years; P=0.62) as were their mean ages (LVSD, 36.5±14.5 years; normal LV function, 40.7±15.2 years; P=0.73). Patients with LVSD (n=13) had more prior surgeries, more frequent history of significant pulmonic regurgitation, right ventricular systolic dysfunction and more implantable cardiac devices. Over a total observation time of 15.5 years, patients with LVSD were at significantly higher risk of all-cause death or hospitalisation (P=0.006). Onset of LVSD frequently preceded an adverse outcome. CONCLUSIONS: In this cohort of adult patients with TOF repair in childhood followed for a total of 550 patient-years, the frequency of LVSD was 30%. LVSD was associated with lower event-free survival. The appearance of LVSD many years after TOF repair may herald the onset of an adverse outcome.
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spelling pubmed-57083192017-12-08 Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood Anabtawi, Abdel Mondragon, Judith Dodendorf, Diane Laskey, Warren K Open Heart Congenital Heart Disease OBJECTIVE: Left ventricular systolic dysfunction (LVSD) in adult survivors of tetralogy of Fallot (TOF) repair in childhood has been observed, although the relationship with long-term outcome remains inadequately described. METHODS: A cohort of 44 consecutive adult patients with TOF repair in childhood were followed prospectively from January 2001 through June 2016. LVSD was defined as an echocardiographically derived left ventricular (LV) ejection fraction <0.55. Clinical and demographic characteristics in patients with and without LVSD were compared. Event-free survival (all-cause death or hospitalisation) was estimated using the product limit method. RESULTS: The average time from childhood surgical repair to cohort inception was similar between groups (LVSD, 33.7±12.7 years; normal LV function, 36.1±14.9 years; P=0.62) as were their mean ages (LVSD, 36.5±14.5 years; normal LV function, 40.7±15.2 years; P=0.73). Patients with LVSD (n=13) had more prior surgeries, more frequent history of significant pulmonic regurgitation, right ventricular systolic dysfunction and more implantable cardiac devices. Over a total observation time of 15.5 years, patients with LVSD were at significantly higher risk of all-cause death or hospitalisation (P=0.006). Onset of LVSD frequently preceded an adverse outcome. CONCLUSIONS: In this cohort of adult patients with TOF repair in childhood followed for a total of 550 patient-years, the frequency of LVSD was 30%. LVSD was associated with lower event-free survival. The appearance of LVSD many years after TOF repair may herald the onset of an adverse outcome. BMJ Publishing Group 2017-11-28 /pmc/articles/PMC5708319/ /pubmed/29225904 http://dx.doi.org/10.1136/openhrt-2017-000690 Text en © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted. This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
spellingShingle Congenital Heart Disease
Anabtawi, Abdel
Mondragon, Judith
Dodendorf, Diane
Laskey, Warren K
Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood
title Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood
title_full Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood
title_fullStr Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood
title_full_unstemmed Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood
title_short Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood
title_sort late-stage left ventricular dysfunction in adult survivors of tetralogy of fallot repair in childhood
topic Congenital Heart Disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5708319/
https://www.ncbi.nlm.nih.gov/pubmed/29225904
http://dx.doi.org/10.1136/openhrt-2017-000690
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