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Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study
Journal of the American Heart Association, 2024-03, Vol.13 (6), p.e031722-e031722
[Peer Reviewed Journal]
ISSN: 2047-9980 ;EISSN: 2047-9980 ;DOI: 10.1161/JAHA.123.031722 ;PMID: 38497454
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Title:
Long-Term Survival After Single-Ventricle Palliation: A Swedish Nationwide Cohort Study
Author:
Dalén, Magnus
;
Odermarsky, Michal
;
Liuba, Petru
;
Johansson Ramgren, Jens
;
Synnergren, Mats
;
Sunnegårdh, Jan
Subjects:
Cardiac and Cardiovascular Systems
;
Child
;
Clinical Medicine
;
Cohort Studies
;
congenital heart disease
;
Fontan Procedure
;
functional single ventricle
;
Heart Defects, Congenital
;
Heart Ventricles - abnormalities
;
Heart Ventricles - diagnostic imaging
;
Heart Ventricles - surgery
;
Humans
;
Hypoplastic Left Heart Syndrome - surgery
;
Kardiologi
;
Klinisk medicin
;
Medical and Health Sciences
;
Medicin och hälsovetenskap
;
Pediatrics
;
Pediatrik
;
Retrospective Studies
;
survival
;
Sweden - epidemiology
;
Treatment Outcome
;
univentricular
;
Univentricular Heart
;
ventricular morphology
Is Part Of:
Journal of the American Heart Association, 2024-03, Vol.13 (6), p.e031722-e031722
Description:
Long-term survival after single-ventricle palliation and the effect of dominant ventricle morphology in large, unselected series of patients are scarcely reported. 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. 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. URL: https://www.clinicaltrials.gov; Unique identifier: NCT03356574.
Publisher:
England: Wiley
Language:
English
Identifier:
ISSN: 2047-9980
EISSN: 2047-9980
DOI: 10.1161/JAHA.123.031722
PMID: 38497454
Source:
GFMER Free Medical Journals
MEDLINE
PubMed Central
SWEPUB Freely available online
Wiley Blackwell Open Access Titles
ROAD: Directory of Open Access Scholarly Resources
DOAJ Directory of Open Access Journals
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