Long‐Term Survival After Single‐Ventricle Palliation : A Swedish Nationwide Cohort Study
Dalén, Magnus ; Odermarsky, Michal LU
; Liuba, Petru
LU
; Johansson Ramgren, Jens
LU
; Synnergren, Mats
and Sunnegårdh, Jan
(2024)
In Journal of the American Heart Association
13(6).
- Abstract
- Background
Long‐term survival after single‐ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported.
Methods and Results
This nationwide cohort study included all children undergoing operation with single‐ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for... (More) - Background
Long‐term survival after single‐ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported.
Methods and Results
This nationwide cohort study included all children undergoing operation with single‐ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for complete data on survival. Among 766 included patients, 333 patients (43.5%) were classified as having left or biventricular dominance, and 432 patients (56.4%) as having right ventricular (RV) dominance (of whom 231 patients had hypoplastic left heart syndrome). Follow‐up was 98.7% complete (10 patients emigrated). Mean follow‐up was 11.3 years (maximum, 26.7 years). Long‐term survival was significantly higher in patients with left ventricular compared with RV dominance (10‐year survival: 91.0% [95% CI, 87.3%–93.6%] versus 71.1% [95% CI, 66.4%–75.2%]). RV dominance had a significant impact on outcomes after first‐stage palliation but was also associated with impaired survival after completed total cavopulmonary connection. In total, 34 (4.4%) patients underwent heart transplantation. Of these 34 patients, 25 (73.5%) had predominant RV morphology.
Conclusions
This study provides clinically relevant knowledge about the long‐term prognosis in patients with different underlying cardiac anomalies undergoing single‐ventricle palliation. RV dominance had a significant impact on outcomes after initial surgical treatment but was also associated with impaired survival after completed Fontan circulation.
Registration
URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/98da781e-fecb-4e98-93a3-0ee83b73f129
- author
- Dalén, Magnus
; Odermarsky, Michal
LU
; Liuba, Petru
LU
; Johansson Ramgren, Jens
LU
; Synnergren, Mats
and Sunnegårdh, Jan
- organization
- publishing date
- 2024-03-18
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- congenital heart disease, functional single ventricle, survival, univentricular, ventricular morphology
- in
- Journal of the American Heart Association
- volume
- 13
- issue
- 6
- article number
- e031722
- pages
- 12 pages
- publisher
- Wiley-Blackwell
- external identifiers
-
- pmid:38497454
- scopus:85188479854
- ISSN
- 2047-9980
- DOI
- 10.1161/JAHA.123.031722
- language
- English
- LU publication?
- yes
- id
- 98da781e-fecb-4e98-93a3-0ee83b73f129
- date added to LUP
- 2024-03-18 14:23:33
- date last changed
- 2024-04-15 04:01:33
@article{98da781e-fecb-4e98-93a3-0ee83b73f129,
abstract = {{Background<br/><br/>Long‐term survival after single‐ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported.<br/>Methods and Results<br/><br/>This nationwide cohort study included all children undergoing operation with single‐ventricle palliation during their first year of life in Sweden between January 1994 and December 2019. Data were obtained from institutional records and assessment of underlying cardiac anomaly and dominant ventricular morphology was based on complete review of medical records, surgical reports, and echocardiographic examinations. Data on vital status and date of death were retrieved from the Swedish Cause of Death Register, allowing for complete data on survival. Among 766 included patients, 333 patients (43.5%) were classified as having left or biventricular dominance, and 432 patients (56.4%) as having right ventricular (RV) dominance (of whom 231 patients had hypoplastic left heart syndrome). Follow‐up was 98.7% complete (10 patients emigrated). Mean follow‐up was 11.3 years (maximum, 26.7 years). Long‐term survival was significantly higher in patients with left ventricular compared with RV dominance (10‐year survival: 91.0% [95% CI, 87.3%–93.6%] versus 71.1% [95% CI, 66.4%–75.2%]). RV dominance had a significant impact on outcomes after first‐stage palliation but was also associated with impaired survival after completed total cavopulmonary connection. In total, 34 (4.4%) patients underwent heart transplantation. Of these 34 patients, 25 (73.5%) had predominant RV morphology.<br/>Conclusions<br/><br/>This study provides clinically relevant knowledge about the long‐term prognosis in patients with different underlying cardiac anomalies undergoing single‐ventricle palliation. RV dominance had a significant impact on outcomes after initial surgical treatment but was also associated with impaired survival after completed Fontan circulation.<br/>Registration<br/><br/>URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574.}},
author = {{Dalén, Magnus and Odermarsky, Michal and Liuba, Petru and Johansson Ramgren, Jens and Synnergren, Mats and Sunnegårdh, Jan}},
issn = {{2047-9980}},
keywords = {{congenital heart disease; functional single ventricle; survival; univentricular; ventricular morphology}},
language = {{eng}},
month = {{03}},
number = {{6}},
publisher = {{Wiley-Blackwell}},
series = {{Journal of the American Heart Association}},
title = {{Long‐Term Survival After Single‐Ventricle Palliation : A Swedish Nationwide Cohort Study}},
url = {{http://dx.doi.org/10.1161/JAHA.123.031722}},
doi = {{10.1161/JAHA.123.031722}},
volume = {{13}},
year = {{2024}},
}