Longitudinal Assessment of Right Ventricular Function in Hypoplastic Left Heart Syndrome.
Fractional area change
Hypoplastic left heart syndrome
Right ventricular function
Survival
Tricuspid annular systolic excursion
Journal
Pediatric cardiology
ISSN: 1432-1971
Titre abrégé: Pediatr Cardiol
Pays: United States
ID NLM: 8003849
Informations de publication
Date de publication:
Aug 2021
Aug 2021
Historique:
received:
23
12
2020
accepted:
21
04
2021
pubmed:
15
5
2021
medline:
27
7
2021
entrez:
14
5
2021
Statut:
ppublish
Résumé
Overall survival of patients with hypoplastic left heart syndrome (HLHS) has shown continued improvement. Right ventricular (RV) dysfunction, in the long term, adversely affects prognosis in these patients. This study examines changes in echocardiographic markers of RV function in a longitudinal cohort. We retrospectively reviewed patients with HLHS managed at our institution from 7/1994 to 1/2016. Follow-up included surgical and clinical data, and echocardiographic measures. Measures of RV function preceding and following all three stages of single ventricular palliation were collected. Freedom from transplant-free survival was assessed by Kaplan-Meier analysis. Multivariable associations with time to death or transplant were explored using the Cox proportional hazards model. A total of 120 patients with HLHS were identified. Norwood operation was performed in all patients. The probability of survival for the cohort was 71 ± 4.4%, 69 ± 4.5% and 66 ± 4.7% at 1, 2 and 5 years respectively after stage I Norwood operation. RV fractional area change (FAC), compared to post-Norwood was decreased at all subsequent stages with the greatest change noted post-superior cavo-pulmonary shunt from 40.7 ± 9.3% to 31.1 ± 8.3% (p < 0.001). Similarly, tricuspid valve annular systolic excursion (TAPSE) Z-score declined from -2.9 ± 1.3 to -9.7 ± 1.3 (p < 0.001) with a decrement at every stage of evaluation. In comparison to patients with post-Norwood RV FAC >35% and TAPSE Z-score > -5, patients with RV FAC ≤ 35% and TAPSE Z-score ≤ -5 had a significantly lower transplant-free survival (p < 0.0001). In patients with HLHS undergoing staged palliation, decrement in RV function manifests longitudinally. Post-Norwood RV FAC and TAPSE Z-score appear to be early markers of poor outcome in this population.
Identifiants
pubmed: 33987707
doi: 10.1007/s00246-021-02624-y
pii: 10.1007/s00246-021-02624-y
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1394-1404Informations de copyright
© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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