Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in Denmark

Abstract Total anomalous pulmonary venous connection (TAPVC) is a rare and life-threatening condition, and long-term patient outcomes remain to be fully explored. This registry-based national cohort study including all Danish children born with TAPVC between 2000 and 2018 aimed to evaluate the survi...

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Main Authors: Maj Beldring Henningsen, Cathrine Bohnstedt, Therese Risom Vestergaard, Morten Holdgaard Smerup, Pi Vejsig Madsen, Lone Graff Stensballe
Format: Article
Language:English
Published: Nature Portfolio 2025-08-01
Series:Scientific Reports
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Online Access:https://doi.org/10.1038/s41598-025-15769-0
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author Maj Beldring Henningsen
Cathrine Bohnstedt
Therese Risom Vestergaard
Morten Holdgaard Smerup
Pi Vejsig Madsen
Lone Graff Stensballe
author_facet Maj Beldring Henningsen
Cathrine Bohnstedt
Therese Risom Vestergaard
Morten Holdgaard Smerup
Pi Vejsig Madsen
Lone Graff Stensballe
author_sort Maj Beldring Henningsen
collection DOAJ
description Abstract Total anomalous pulmonary venous connection (TAPVC) is a rare and life-threatening condition, and long-term patient outcomes remain to be fully explored. This registry-based national cohort study including all Danish children born with TAPVC between 2000 and 2018 aimed to evaluate the survival rates of Danish children receiving a TAPVC repair procedure. Additionally, the prevalence of early morbidity, severe chronic diseases, and mental and/or behavioral disorders in children diagnosed with TAPVC was investigated using Danish health registries. A total of 89 children with TAPVC were identified, of whom 65 underwent primary repair. Among those receiving surgery, the survival rate was 88% at 3 months, decreasing to 78% at 20 years. In the cohort of children with a TAPVC primary repair, 26% had one or more hospital visits with primary or secondary chronic somatic diagnoses, and 8% had one or more hospital visits with a mental and/or behavioral disorder. In comparison, 6% of all Danish children born during the same period had similar hospital visits for somatic diseases, and 3% for mental and/or behavioral disorders. The survival rate of our TAPVC cohort aligns with that reported in the literature. Furthermore, this study highlights the need for additional research into the physical and mental health of these children, as they require hospital care more frequently than their peers.
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spelling doaj-art-687473b9880b446fa3ec430e3363aa322025-08-24T11:23:24ZengNature PortfolioScientific Reports2045-23222025-08-011511810.1038/s41598-025-15769-0Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in DenmarkMaj Beldring Henningsen0Cathrine Bohnstedt1Therese Risom Vestergaard2Morten Holdgaard Smerup3Pi Vejsig Madsen4Lone Graff Stensballe5Mary Elizabeth’s Hospital, Copenhagen University Hospital RigshospitaletDepartment of Pediatrics and Adolescent Medicine, Copenhagen University Hospital RigshospitaletDepartment of Thoracic Anesthesiology, Copenhagen University Hospital RigshospitaletDepartment of Cardiothoracic Surgery, Copenhagen University Hospital RigshospitaletMary Elizabeth’s Hospital, Copenhagen University Hospital RigshospitaletMary Elizabeth’s Hospital, Copenhagen University Hospital RigshospitaletAbstract Total anomalous pulmonary venous connection (TAPVC) is a rare and life-threatening condition, and long-term patient outcomes remain to be fully explored. This registry-based national cohort study including all Danish children born with TAPVC between 2000 and 2018 aimed to evaluate the survival rates of Danish children receiving a TAPVC repair procedure. Additionally, the prevalence of early morbidity, severe chronic diseases, and mental and/or behavioral disorders in children diagnosed with TAPVC was investigated using Danish health registries. A total of 89 children with TAPVC were identified, of whom 65 underwent primary repair. Among those receiving surgery, the survival rate was 88% at 3 months, decreasing to 78% at 20 years. In the cohort of children with a TAPVC primary repair, 26% had one or more hospital visits with primary or secondary chronic somatic diagnoses, and 8% had one or more hospital visits with a mental and/or behavioral disorder. In comparison, 6% of all Danish children born during the same period had similar hospital visits for somatic diseases, and 3% for mental and/or behavioral disorders. The survival rate of our TAPVC cohort aligns with that reported in the literature. Furthermore, this study highlights the need for additional research into the physical and mental health of these children, as they require hospital care more frequently than their peers.https://doi.org/10.1038/s41598-025-15769-0Congenital heart diseaseRegistry-based cohort studyCardiac surgery outcomesPediatric epidemiology
spellingShingle Maj Beldring Henningsen
Cathrine Bohnstedt
Therese Risom Vestergaard
Morten Holdgaard Smerup
Pi Vejsig Madsen
Lone Graff Stensballe
Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in Denmark
Scientific Reports
Congenital heart disease
Registry-based cohort study
Cardiac surgery outcomes
Pediatric epidemiology
title Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in Denmark
title_full Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in Denmark
title_fullStr Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in Denmark
title_full_unstemmed Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in Denmark
title_short Nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in Denmark
title_sort nationwide registry study of long term survival and comorbidities in total anomalous pulmonary venous connection in denmark
topic Congenital heart disease
Registry-based cohort study
Cardiac surgery outcomes
Pediatric epidemiology
url https://doi.org/10.1038/s41598-025-15769-0
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