Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study

Background Norwood surgery provides a palliative surgical option for hypoplastic left heart syndrome and has been available in Sweden since 1993. The practice of prenatal ultrasound screening was gradually implemented in the same era, resulting in an increased prenatal detection rate. Our primary ai...

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Main Authors: Annika Öhman, Milad El‐Segaier, Gunnar Bergman, Katarina Hanséus, Torsten Malm, Boris Nilsson, Aldina Pivodic, Annika Rydberg, Sven‐Erik Sonesson, Mats Mellander
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
Subjects:
Online Access:https://www.ahajournals.org/doi/10.1161/JAHA.118.010893
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author Annika Öhman
Milad El‐Segaier
Gunnar Bergman
Katarina Hanséus
Torsten Malm
Boris Nilsson
Aldina Pivodic
Annika Rydberg
Sven‐Erik Sonesson
Mats Mellander
author_facet Annika Öhman
Milad El‐Segaier
Gunnar Bergman
Katarina Hanséus
Torsten Malm
Boris Nilsson
Aldina Pivodic
Annika Rydberg
Sven‐Erik Sonesson
Mats Mellander
author_sort Annika Öhman
collection DOAJ
description Background Norwood surgery provides a palliative surgical option for hypoplastic left heart syndrome and has been available in Sweden since 1993. The practice of prenatal ultrasound screening was gradually implemented in the same era, resulting in an increased prenatal detection rate. Our primary aims were to study changes in the incidence of live births, prenatal detection rate, and the termination of pregnancies over time. The secondary aims were to study the proportion of live‐borns undergoing surgery and to identify factors that influenced whether surgery was or was not performed. Methods and Results Neonates with hypoplastic left heart syndrome with aortic atresia born 1990‐2010 were identified through national databases, surgical files, and medical records. The fetal incidence was estimated from the period when prenatal screening was rudimentary. The study period was divided into the presurgical, early surgical, and late surgical periods. The incidence was calculated as the overall yearly incidence for each time period and sex separately. Factors influencing whether surgery was performed were analyzed using Cox‐logistic regression. The incidence at live birth decreased from 15.4 to 8.4 per 100 000. The prenatal detection rate increased from 27% to 63%, and terminations increased from 19% to 56%. The odds of having surgery was higher in the late period and higher in the group with prenatal diagnosis. Conclusions We observed a decrease in incidence of live‐borns with hypoplastic left heart syndrome aortic atresia. There was in increase in prenatal detection rate and an increase in termination of pregnancy. The proportion of live‐borns who underwent surgery increased between time periods.
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spelling doaj.art-d86f2cf3a8c74f08814c76c02e2d2cef2022-12-21T23:53:05ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802019-01-018210.1161/JAHA.118.010893Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort StudyAnnika Öhman0Milad El‐Segaier1Gunnar Bergman2Katarina Hanséus3Torsten Malm4Boris Nilsson5Aldina Pivodic6Annika Rydberg7Sven‐Erik Sonesson8Mats Mellander9Department of Paediatric Cardiology Queen Silvia Children′s Hospital Sahlgrenska University Hospital Gothenburg SwedenDepartment of Paediatric Cardiology Skåne University Hospital Lund SwedenDepartment of Pediatric Cardiology Karolinska University Hospital Stockholm SwedenDepartment of Paediatric Cardiology Skåne University Hospital Lund SwedenDepartment of Paediatric Cardiac Surgery Skåne University Hospital Lund SwedenDepartment of Paediatric Cardiology Queen Silvia Children′s Hospital Sahlgrenska University Hospital Gothenburg SwedenStatistiska konsultgruppen Gothenburg SwedenDepartment of Clinical Sciences, Paediatrics Umeå University Umeå SwedenDepartment of Women's and Children's Health Karolinska Institutet Stockholm SwedenDepartment of Paediatric Cardiology Queen Silvia Children′s Hospital Sahlgrenska University Hospital Gothenburg SwedenBackground Norwood surgery provides a palliative surgical option for hypoplastic left heart syndrome and has been available in Sweden since 1993. The practice of prenatal ultrasound screening was gradually implemented in the same era, resulting in an increased prenatal detection rate. Our primary aims were to study changes in the incidence of live births, prenatal detection rate, and the termination of pregnancies over time. The secondary aims were to study the proportion of live‐borns undergoing surgery and to identify factors that influenced whether surgery was or was not performed. Methods and Results Neonates with hypoplastic left heart syndrome with aortic atresia born 1990‐2010 were identified through national databases, surgical files, and medical records. The fetal incidence was estimated from the period when prenatal screening was rudimentary. The study period was divided into the presurgical, early surgical, and late surgical periods. The incidence was calculated as the overall yearly incidence for each time period and sex separately. Factors influencing whether surgery was performed were analyzed using Cox‐logistic regression. The incidence at live birth decreased from 15.4 to 8.4 per 100 000. The prenatal detection rate increased from 27% to 63%, and terminations increased from 19% to 56%. The odds of having surgery was higher in the late period and higher in the group with prenatal diagnosis. Conclusions We observed a decrease in incidence of live‐borns with hypoplastic left heart syndrome aortic atresia. There was in increase in prenatal detection rate and an increase in termination of pregnancy. The proportion of live‐borns who underwent surgery increased between time periods.https://www.ahajournals.org/doi/10.1161/JAHA.118.010893fetal cardiovascular abnomalityfetal echocardiographyhypoplastic left heart syndromeoutcome
spellingShingle Annika Öhman
Milad El‐Segaier
Gunnar Bergman
Katarina Hanséus
Torsten Malm
Boris Nilsson
Aldina Pivodic
Annika Rydberg
Sven‐Erik Sonesson
Mats Mellander
Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study
Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease
fetal cardiovascular abnomality
fetal echocardiography
hypoplastic left heart syndrome
outcome
title Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study
title_full Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study
title_fullStr Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study
title_full_unstemmed Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study
title_short Changing Epidemiology of Hypoplastic Left Heart Syndrome: Results of a National Swedish Cohort Study
title_sort changing epidemiology of hypoplastic left heart syndrome results of a national swedish cohort study
topic fetal cardiovascular abnomality
fetal echocardiography
hypoplastic left heart syndrome
outcome
url https://www.ahajournals.org/doi/10.1161/JAHA.118.010893
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