Standardized mortality ratio in adults with congenital heart disease
(2025) In European Heart Journal Open 5(6).- Abstract
Aims The prevalence of adults with congenital heart disease (ACHD) is rising due to improved paediatric care. In parallel, updated data on prognosis in adult life are needed. Objectives The aim was to calculate the standardized mortality ratio (SMR) and death rates in ACHD compared to the general population. Methods and results Data were obtained from the national register of congenital heart disease. The general Swedish population served as a reference. SMR was calculated as the ratio between observed and expected deaths. 9089 patients (median age 28 years, interquartile range [IQR] 20–45, 47% females) were followed for a median of 8 years (IQR 4–14). 525 deaths occurred during observation. The SMR increased by lesion complexity:... (More)
Aims The prevalence of adults with congenital heart disease (ACHD) is rising due to improved paediatric care. In parallel, updated data on prognosis in adult life are needed. Objectives The aim was to calculate the standardized mortality ratio (SMR) and death rates in ACHD compared to the general population. Methods and results Data were obtained from the national register of congenital heart disease. The general Swedish population served as a reference. SMR was calculated as the ratio between observed and expected deaths. 9089 patients (median age 28 years, interquartile range [IQR] 20–45, 47% females) were followed for a median of 8 years (IQR 4–14). 525 deaths occurred during observation. The SMR increased by lesion complexity: atrial septal defect [1.3 (95% CI: 1.1–1.5)]; ventricular septal defect [2.0 (1.4–2.7)]; congenital aortic valve disease [2.2 (1.6–2,9)]; Ebstein’s anomaly [3.2 (1.8–5.2)]; tetralogy of Fallot [3.8 (2.6–5.2)]; congenitally corrected transposition of the great arteries [5.6 (2.9–9.6)]; Eisenmenger syndrome [8.7 (5.5–13.1)]; transposition of the great arteries with a previous atrial redirection operation [12.3 (6.8–20.1)]; and Fontan physiology [22.5 (12.5–37.0)]. Calculations were also performed by severity (mild, moderate, and severe) and age by six age groups. SMR was generally higher in younger age, and the difference in mortality from the general population was estimated to be lower for older age groups. The mortality distribution and death rate per 1000 person-years have also been calculated for each lesion. Conclusion The mortality in ACHD remains increased compared to the general population and reflects the severity of the lesion. In higher ages, the observed mortality is more in line with the general population, probably because of survival of the least affected patients, and that few persons with severe lesions have reached advanced age.
(Less)
- author
- Hahlin, Elisabeth ; Christersson, Christina ; Sörensson, Peder ; Trzebiatowska-Krzynska, Aleksandra ; Mandalenakis, Zacharias ; Hlebowicz, Joanna LU ; Sandberg, Camilla LU ; Johansson, Bengt LU and Rinnström, Daniel
- organization
- publishing date
- 2025-11-01
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- Age and gender standardized mortality rate, Age-specific crude mortality rate, Mortality
- in
- European Heart Journal Open
- volume
- 5
- issue
- 6
- article number
- oeaf165
- publisher
- Oxford University Press
- external identifiers
-
- scopus:105025704797
- pmid:41451242
- ISSN
- 2752-4191
- DOI
- 10.1093/ehjopen/oeaf165
- language
- English
- LU publication?
- yes
- additional info
- Publisher Copyright: © The Author(s) 2025. Published by Oxford University Press on behalf of the European Society of Cardiology.
- id
- e750fad0-4f06-41b5-9e39-67a9cee06aca
- date added to LUP
- 2026-02-17 14:57:37
- date last changed
- 2026-09-03 19:17:51
@article{e750fad0-4f06-41b5-9e39-67a9cee06aca,
abstract = {{<p>Aims The prevalence of adults with congenital heart disease (ACHD) is rising due to improved paediatric care. In parallel, updated data on prognosis in adult life are needed. Objectives The aim was to calculate the standardized mortality ratio (SMR) and death rates in ACHD compared to the general population. Methods and results Data were obtained from the national register of congenital heart disease. The general Swedish population served as a reference. SMR was calculated as the ratio between observed and expected deaths. 9089 patients (median age 28 years, interquartile range [IQR] 20–45, 47% females) were followed for a median of 8 years (IQR 4–14). 525 deaths occurred during observation. The SMR increased by lesion complexity: atrial septal defect [1.3 (95% CI: 1.1–1.5)]; ventricular septal defect [2.0 (1.4–2.7)]; congenital aortic valve disease [2.2 (1.6–2,9)]; Ebstein’s anomaly [3.2 (1.8–5.2)]; tetralogy of Fallot [3.8 (2.6–5.2)]; congenitally corrected transposition of the great arteries [5.6 (2.9–9.6)]; Eisenmenger syndrome [8.7 (5.5–13.1)]; transposition of the great arteries with a previous atrial redirection operation [12.3 (6.8–20.1)]; and Fontan physiology [22.5 (12.5–37.0)]. Calculations were also performed by severity (mild, moderate, and severe) and age by six age groups. SMR was generally higher in younger age, and the difference in mortality from the general population was estimated to be lower for older age groups. The mortality distribution and death rate per 1000 person-years have also been calculated for each lesion. Conclusion The mortality in ACHD remains increased compared to the general population and reflects the severity of the lesion. In higher ages, the observed mortality is more in line with the general population, probably because of survival of the least affected patients, and that few persons with severe lesions have reached advanced age.</p>}},
author = {{Hahlin, Elisabeth and Christersson, Christina and Sörensson, Peder and Trzebiatowska-Krzynska, Aleksandra and Mandalenakis, Zacharias and Hlebowicz, Joanna and Sandberg, Camilla and Johansson, Bengt and Rinnström, Daniel}},
issn = {{2752-4191}},
keywords = {{Age and gender standardized mortality rate; Age-specific crude mortality rate; Mortality}},
language = {{eng}},
month = {{11}},
number = {{6}},
publisher = {{Oxford University Press}},
series = {{European Heart Journal Open}},
title = {{Standardized mortality ratio in adults with congenital heart disease}},
url = {{http://dx.doi.org/10.1093/ehjopen/oeaf165}},
doi = {{10.1093/ehjopen/oeaf165}},
volume = {{5}},
year = {{2025}},
}